Tumors of the Hand

Tumors of the Hand

Edited by

Daniel V Egloff, MD
Centre Hospitalier Universitaire Vaudois
and Clinique Longeraie
Lausanne, Switzerland

Published in association with the

Federation of European Societies
for Surgery of the Hand

>±) Taylor & Francis

Taylor & Francis Group

L O N D O N A N D N E W YORK

A MARTIN DUNITZ BOOK

© 2004 Taylor & Francis, an imprint of the Taylor & Francis Group

First published in the United Kingdom in 2004

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CONTENTS

List of c o n t r i b u t o r s vii

Preface xi

1 B e n i g n a n d m a l i g n a n t t u m o r s of t h e h a n d a n d u p p e r l i m b :
generalities, classification a n d frequency 1
Vincent R Hentz

2 H a n d t u m o r s at l'Institut d e la M a i n 11
Caroline Leclercq, Elena Mateva, Carlos Rueda and the members of l'Institut
de la Main, Paris, France

3 I m a g i n g of t u m o r s a n d tumor-like l e s i o n s of t h e h a n d a n d f i n g e r s 15
Nicolas Theumann and Jean-Luc Drape

4 G i a n t cell t u m o r of t h e t e n d o n s h e a t h 27
Daniel V Egloffand De bora loanna

5 Hand chondromas 39
Caroline Leclercq

6 G l o m u s t u m o r of t h e h a n d 47
Nicolas Favarger

1 O s t e o i d o s t e o m a of t h e h a n d a n d w r i s t 55
Dominique Le Viet and loannis Tsionos

8 Schwannomas 75
Anne Gray and Chantai Bonnard

9 Neurofibroma 79
Alberto Lluch

10 Malignant peripheral nerve sheath tumors 93

Chantai Bonnard

11 Vascular t u m o r s : classification a n d surgery 99

Antoine Lavie, Régis Legre and Guy Magalon

12 R a d i o l o g i c t r e a t m e n t of v a s c u l a r ' t u m o r s ' 113

Philippe Petit, Jean M Bartoli, Karen Lambot, Guy Moulin and Philippe Devred
vi T U M O R S OF THE HAND

13 W h a t c a n t h e h a n d s u r g e o n do w h e n facing a patient w i t h a m e l a n o n y c h i a ? 121

Christian Dumontier and Philippe Abimelec

14 Malignant melanoma 133

Margita Flügel

15 M e l a n o m a s of t h e h a n d : s e n t i n e l n o d e b i o p s y 139
Vincent R Hentz

16 P r i n c i p l e s of b i o p s y a n d s u r g i c a l m a n a g e m e n t of soft t i s s u e t u m o r s 147
Elyazid Mouhsine, Raffaele Garofalo and Pierre F Leyvraz

17 M a l i g n a n t soft t i s s u e t u m o r s of t h e h a n d : h i s t o l o g i c a l d i a g n o s i s a n d c l a s s i f i c a t i o n 155
Louis Guillou

18 S o f t t i s s u e s a r c o m a of t h e h a n d a n d w r i s t 167
Massimo Ceruso, Domenico Campanacci, Patrizio Caldora and Prospero Bigazzi

19 O b j e c t i v e s a n d r e s u l t s of t h e C o o p e r a t i v e O s t e o s a r c o m a S t u d y G r o u p 175
Stefan S Bielack and Silke Flege

20 Chondrosarcoma 183
Caroline Leclercq

21 M e t a s t a t i c t u m o r s to t h e h a n d a n d w r i s t 187
Vincent R Hentz

Index 193
L I S T OF C O N T R I B U T O R S

Philippe Abimelec Patrizio C a l d o r a

Dermatology Il D i v i s i o n e O r t o p e d i a
Hôpital Saint Louis Centro D'Oncologia Ricostruttiva
R u e Claude Vellefaux Azienda Ospedaliera -Universitaria
75010 P a r i s Ca reggi
France 50139 Firenze
Italy
Jean M Bartoli
Radiology Department Domenico Campanacci
HôpitaiTimone - Adultes Il D i v i s i o n e O r t o p e d i a
Marseille Centro D'Oncologia Ricostruttiva
France
Azienda Ospedaliera-Universitaria
Careggi
Stefan S Bielack 50139 Firenze
Universitätsklinikum Münster Italy
Klinik u n d Poliklinik fur Kinder- u n d
Jugendmedizin
Massimo Caruso
Pädiatrische Hämatologie und
C h i r u r g i a della M a n o e Microchirurgia
Onkologie
Ricostruttiva
Albert-Scheweitzer Str. 33
Azienda Ospedaliera-Universitaria
48129 M ü n s t e r
Careggi
Germany
50139 Firenze
Italy
Prospero Bigazzi
C h i r u r g i a della M a n o e Microchirurgia
Ricostruttiva Philippe Devred
Azienda Ospedaliera-Universitaria Radiology Department
Ca r e g g i HôpitaiTimone - Enfants
50139 Firenze Marseille
Italy France

Chantal Bonnard Jean-Luc Drape

Clinque de Longeraie Service de Radiology
A v . d e la G a r e 9 Hôpital Cochin
1003 L a u s a n n e Paris
Switzerland France
 T U M O R S OF THE HAND

Christian Dumontier Vincent R Hentz

Institut d e la M a i n Robert A. Chase Hand S u r g e r y Center
6 Square Jouvenet Stanford University
75010 P a r i s 900 W e l c h R o a d
France P a l o A l t o , C A 94304
USA
Daniel V Egloff
Service Universitaire d e Chirurgie Debora loanna
P l a s t i q u e et R e c o n s t r u c t i v e Service Universitaire de Chirurgie
C h i r u r g i e d e la M a i n P l a s t i q u e et R e c o n s t r u c t i v e
A v e n u e d e la G a r e 9 C h i r u r g i e d e la M a i n
1003 L a u s a n n e Clinique de Longeraie
Switzerland A v e n u e d e la G a r e 9
1003 L a u s a n n e
Nicolas Favarger Switzerland
C l i n i q u e C h i r u r g i c a l e et P e r m a n e n c e d e
Longeraie
Karen Lambot
A v e n u e d e la G a r e 9 Radiology Department

1003 L a u s a n n e Hôpital T i m o n e - E n f a n t s

Switzerland Marseille
France
Silke Flege
Universitätsklinikum Münster
Antoine Lavie
S e r v i c e d e C h i r u r g i e d e la M a i n
Klinik u n d Poliklink für Kinder- u n d
C h i r u r g i e P l a s t i q u e et R é p a r a t r i c d e s M e m b r e s
Jugendamedizin
H ô p i t a l d e la C o n c e p t i o n
Pädiatrische Hämatologie und
145 B l d B a i l l e
Onkologie
13005 M a r s e i l l e
A l b e r t - S c h w e i t z e r S t r . 33
France
48129 M ü n s t e r
Germany Caroline Leclercq
Institut d e la M a i n
Margita Flügel 6 Square Jouvenet
Unfallklinik Friederikenstrifts
75016 P a r i s
M a r i e n s t r a s s e 37
France
30171 H a n n o v e r
Germany Régis Legre
S e r v i c e d e C h i r u r g i e d e la M a i n
Raffaele Garofalo C h i r u r g i e P l a s t i q u e et R é p a r a t r i c d e s M e m b r e s
Orthopedics and Traumatology H ô p i t a l d e la C o n c e p t i o n
Centre Hospitalier Universitaire Vaudois 145 B l d B a i l l e
1011 L a u s a n n e 13005 M a r s e i l l e
Switzerland France

Anne Gray Dominique Le Viet

Clinique de Longeraie Institut d e la M a i n
A v . d e la G a r e 9 6, S q u a r e J o u v e n e t
1003 L a u s a n n e 75016 P a r i s
Switzerland France

Louis Guillou Pierre F Leyvraz

Institut U n i v e r s i t a i r e d e P a t h o l o g i e Orthopedics and Traumatology
R u e d u B u g n o n 25 Centre Hospitalier Universitaire V a u d o i s
1011 L a u s a n n e 1011 L a u s a n n e
Switzerland Switzerland
 LIST OF CONTRIBUTORS ix

Alberto Lluch Philippe Petit

Institut K a p l a n for S u r g e r y of t h e H a n d Radiology Department
and U p p e r Extremity H ô p i t a l T i m o n e - Enfants a n d Hôpital
Paseo Bonanova 9 Timone - Adultes
08022 B a r c e l o n a Marseille
Spain France

Guy Magalon Carlos Rueda

S e r v i c e d e C h i r u r g i e d e la M a i n Institut d e la M a i n
C h i r u r g i e P l a s t i q u e et R é p a r a t r i c d e s M e m b r e s 6, S q u a r e J o u v e n e t
H ô p i t a l d e la C o n c e p t i o n 75016 P a r i s
145 B i d B a i l l e France
13005 M a r s e i l l e
France Nicolas Theumann
S e r v i c e d e R a d i o d i a g n o s t i c et d e
Elena Mateva Radiologie Interventionnelle
Institut d e la M a i n Centre Hospitalier Universitaire V a u d o i s
6, S q u a r e J o u v e n e t 1011 L a u s a n n e
75016 P a r i s Switzerland
France
loannis Tsionos
Elyazid Mouhsine Institut d e la M a i n
Orthopedics andTraumatology 6, S q u a r e J o u v e n e t
Centre Hospitalier Universitaire Vaudois 75016 P a r i s
1011 L a u s a n n e France
Switzerland

Guy Moulin
Radiology Department
Hôpital T i m o n e - Adultes
Marseille
France
 PREFACE

R e c e n t d e c a d e s h a v e s e e n a d r a s t i c e v o l u t i o n in not o n l y s e r v e t h r o u g h o u t t h e d i s e a s e c o u r s e to
t h e r e c o g n i t i o n a n d t r e a t m e n t o f t u m o r s of t h e help in diagnosis, but also ensure follow-up
hand, wrist, and, one should add, peripheral detection of recurrences, and of extensions.
n e r v e s . T h i s is o w i n g t o s e v e r a l r e a s o n s . T h e m a i n I m m u n o h i s t o c h e m i s t r y a n d , n o w , m o l e c u l a r bio-
o n e is p r o b a b l y r e l a t e d to t h e h a n d l i n g of m a l i g - l o g y a r e a l s o v e r y helpful in a l l o w i n g a more
n a n t t u m o r s , w h i c h is p e r f o r m e d m o r e a n d m o r e a c c u r a t e h i s t o l o g i c a l d i a g n o s i s , w i t h its t h e r a p e u -
o f t e n in m e d i c a l c e n t e r s t h a t c a n c o l l e c t a suffi- tic a n d p r o g n o s t i c v a l u e , to b e m a d e .
c i e n t n u m b e r of c a s e s a n d c a n a s s e m b l e a t e a m T h i s b o o k c o v e r s a l m o s t t h e e n t i r e d o m a i n of
including: a hand surgeon, w h o should also be a hand t u m o r s . S o m e subjects h a v e been deliber-
microsurgeon; a pathologist s p e c i a l i z e d in this ately omitted. For instance the ganglion, despite
field; a n oncologist; a n d radiologists, o n e familiar b e i n g t h e m o s t f r e q u e n t m a s s in t h e h a n d , i s
with imaging, and another with radiotherapy.This not a t u m o r a n d its t r e a t m e n t is to b e f o u n d in
e n a b l e s t h e tactical t h e r a p e u t i c a p p r o a c h to b e all t e x t b o o k s . Skin tumors, except melanoma
d e c i d e d b e f o r e h a n d , a n d t h e appropriate adjust- which requires special considerations, are also
m e n t s to b e m a d e d u r i n g t h e c o u r s e of t r e a t m e n t . not d i s c u s s e d .
The second reason is the critical mass I w i s h t o t h a n k all t h e a u t h o r s w h o not o n l y
a c h i e v e d e i t h e r t h r o u g h t h e literature o r t h e s h a r - c o n s e n t e d to g i v e l e c t u r e s in B u d a p e s t but w h o
ing of c a s e s in a c o u n t r y or a m o n g s e v e r a l insti- also h a v e m a d e a substantial effort to deliver
t u t i o n s . T h e ' s e r i e s of t h e Institut d e la M a i n ' , t h e their m a n u s c r i p t s o n t i m e a n d t h u s e n a b l e p u b l i -
'COSS study', and the 'French Federation of c a t i o n of t h i s b o o k . T h i s d i l i g e n c e a n d c o n t r i b u -
C a n c e r C e n t e r s ' a r e e x a m p l e s of s u c h s t u d i e s . t i o n s b y m o r e t h a n 30 h i g h l y s p e c i a l i z e d a u t h o r s
T h e y p r o v i d e d a t a of statistical v a l u e . m a k e t h i s b o o k t h e v e r y real s t a t e of t h e art.
L a s t but not least, n e w d i a g n o s t i c t o o l s , e s p e -
cially m a g n e t i c r e s o n a n c e imaging ( M R I ) , w h i c h Daniel V Egloff
 1
Benign and malignant tumors of the
hand and upper limb: generalities,
classification and frequency
Vincent R Hentz

Principles definitely a n e p i d e r m a l i n c l u s i o n c y s t . S i m i l a r l y , a
firm s m o o t h m a s s that spontaneously d e v e l o p s
S o f t t i s s u e t u m o r s of t h e h a n d a n d u p p e r l i m b a r e in a r e a s w h e r e g a n g l i a a r e f r e q u e n t , s u c h a s t h e
frequently encountered by physicians and since d o r s u m of t h e w r i s t , is a l m o s t c e r t a i n l y a g a n -
benign lesions are far m o r e c o m m o n , serious glion. A s i m p l e ultrasound study will distinguish
t u m o r s a r e f r e q u e n t l y not c o n s i d e r e d in t h e dif- solid f r o m cystic t u m o r s . Aspiration of a s u s -
f e r e n t i a l d i a g n o s i s of t h e s e l u m p s . T h i s o v e r s i g h t p e c t e d g a n g l i o n w i l l e s t a b l i s h t h e d i a g n o s i s of a
l e a d s t o i n c o r r e c t t r e a t m e n t . In a d d i t i o n , t h e r e is a g a n g l i o n . A d o r s a l m a s s t h a t m o v e s to a n d fro
n a t u r a l t e m p t a t i o n t o u n d e r treat t h e s e t u m o r s in w i t h t h e e x t e n s o r t e n d o n s is a l m o s t s u r e l y a
a n attempt to minimize morbidity a n d avoid c o m - tenosynovial tumor. However, a symptomless
p l e x r e c o n s t r u c t i o n . S u r g e o n s t r e a t i n g l a r g e r soft m a s s t h a t d e v e l o p s w i t h o u t c a u s e in a r e a s o t h e r
t i s s u e o r b o n e t u m o r s of t h e u p p e r extremity than those typical for t h e m o s t c o m m o n t u m o r s
m a y b e r e q u i r e d to p l a y t w o o p p o s i n g r o l e s in is cause for alarm. Symptomatic tumors, or
m a n a g i n g t h e s e t u m o r s . T h e initial r o l e is t h a t of t u m o r s that g r o w m u s t b e d i a g n o s e d , a n d t h e n
a n a b l a t i v e s u r g e o n , w h o m u s t b e a g g r e s s i v e in if n e c e s s a r y , c l a s s i f i e d .
removing the t u m o r and be comfortable resecting B o n e t u m o r s of t h e h a n d a n d w r i s t a r e f a r less
n o r m a l t i s s u e s to a c h i e v e a t u m o r - f r e e margin. c o m m o n t h a n soft t i s s u e t u m o r s a n d , in f a c t , a r e
T h e s e c o n d a r y r o l e is t h a t of a r e c o n s t r u c t i v e r e l a t i v e l y r a r e n e o p l a s m s t h a t a c c o u n t for only
surgeon, w h o must consider whether complex 4 p e r c e n t of all t h e b o n e t u m o r s f o u n d in t h e b o d y
r e c o n s t r u c t i o n s w i l l return t h e u p p e r l i m b to a n ( C a m p b e l l et al 1995). H o w e v e r , their i m p o r t a n c e
aesthetically acceptable a n d functionally useful c a n n o t b e u n d e r e s t i m a t e d d u e to their i n s i d i o u s
limb. growth and malignant potential. J a f f e in 1958
A s a rule, e v e r y m a s s should be v i e w e d a s r e c o m m e n d e d a t r i a n g l e of e x p e r t i s e w h e r e t h e
p o t e n t i a l l y t r o u b l e s o m e a n d d e s e r v i n g of s p e c i a l s u r g e o n , radiologist, a n d p a t h o l o g i s t s h a r e p o i n t s
a t t e n t i o n . F o r t u n a t e l y , t u m o r s of t h e h a n d a n d of v i e w to a r r i v e at a d i a g n o s i s ; this c o n s e n s u s
u p p e r l i m b a r e r e c o g n i z e d e a r l i e r t h a n t u m o r s in m o d e l c o n t i n u e s to b e helpful t o d a y ( J a f f e 1958).
m a n y other anatomic locations because they are The evaluation, diagnosis, classification, and
n o t i c e a b l e . A s s u c h , t h e m o s t c o m m o n soft t i s s u e c h o i c e of t r e a t m e n t for t h e s e t u m o r s is c h a l l e n g -
tumors c a n be relatively easily diagnosed by ing b e c a u s e of t h e w i d e r a n g e of clinical b e h a v i o r
several simple steps. For e x a m p l e , a slowly e x h i b i t e d b y both b e n i g n a n d m a l i g n a n t t u m o r s .
e n l a r g i n g m a s s o n t h e p a l m o r p a l m a r a s p e c t of To a v o i d pitfalls a n d e r r o r s of o m i s s i o n , a s y s -
the digit, following local trauma is almost t e m a t i c s e q u e n c e of e v a l u a t i o n a n d t r e a t m e n t is
2 T U M O R S OF THE HAND

key, b e g i n n i n g with the history of t h e lesion. d e p e n d i n g o n clinical s u s p i c i o n . F o r e x a m p l e , a n

T h e initial p a t i e n t interview should specifically infectious p r o c e s s c o u l d b e i n c l u d e d in t h e differ-
a d d r e s s k e y f a c t o r s t h a t w i l l h e l p to e s t a b l i s h t h e ential d i a g n o s i s for bony m a s s e s ; therefore, a
likely d i a g n o s i s , a n d t h e b i o l o g i c a l p o t e n t i a l of n o r m a l w h i t e b l o o d cell c o u n t a n d s e d i m e n t a t i o n
the tumor. K e y information includes the duration rate m a y h e l p to rule o u t a n i n f e c t i o u s etiology.
of t u m o r g r o w t h , c o u r s e of t h e s y m p t o m s , a n d
any other possible bone or joint pathology.
Careful history taking will demonstrate any
history of t r a u m a o r e x p o s u r e t o h a r m f u l sub- Imaging
stances, or indicate a family history that s h o u l d
alert u s t o f a m i l i a l t u m o r s s u c h a s L i - F r a u m e n i F o r soft t i s s u e t u m o r s , s t a n d a r d X - r a y s a r e first

syndrome (Li and Fraumeni 1969), familial r e q u i r e d . W h i l e t h e s e w i l l often o n l y s h o w a soft

m e l a n o m a (Clark et al 1978) ( s o - c a l l e d B - K m o l e tissue s h a d o w , additional important information
syndrome), and certain fibrous tumors. How m a y b e o b t a i n e d . F o r e x a m p l e , t i s s u e of origin;

r e c e n t l y t h e l e s i o n h a s b e e n n o t i c e d , w h e t h e r it is t h a t is, b o n e v e r s u s soft t i s s u e , m a y b e d i s c e r n e d

g r o w i n g a n d h o w quickly, w h e t h e r t h e r e is p a i n f r o m this, or w i t h e v e n greater likelihood f r o m the

a s s o c i a t e d w i t h t h e l e s i o n , a n d if t h e r e is a h i s t o r y follow-up magnetic resonance imaging (MRI)
of t o b a c c o u s e a n d e x p o s u r e t o h u m a n p a p i l l o m a s t u d y . C a l c i f i e d p h l e b o l i t h s in a soft t i s s u e m a s s
v i r u s s h o u l d all b e d e t e r m i n e d . m a y b e c o n s i s t e n t w i t h a h e m a n g i o m a , w h i l e soft
t i s s u e c a l c i f i c a t i o n in o t h e r m a s s e s m a y r a i s e t h e
question of, for example, a synovial sarcoma.
C o r t i c a l e r o s i o n a s s o c i a t e d w i t h a n o v e r l y i n g soft
Physical examination t i s s u e m a s s r a i s e s t h e q u e s t i o n of malignancy,
a l t h o u g h cortical e r o s i o n s c a n b e f o u n d r e l a t e d t o
T h e r e s u l t s of t h e p h y s i c a l e x a m i n a t i o n f r e q u e n t l y a b e n i g n p r o c e s s (for e x a m p l e , n o d u l a r fasciitis).
determine whether subsequent steps are simple A k e y p o i n t is o b t a i n i n g t h e M R I , or, if n e c e s s a r y ,
or c o m p l e x t e s t i n g is n e e d e d . T h e t u m o r l o c a t i o n , magnetic resonance angiography ( M R A ) study
size, c o n s i s t e n c y , fixation to s u r r o u n d i n g t i s s u e s , prior to b i o p s y , lest t h e p o s t b i o p s y e d e m a a n d / o r
a n d m o v e m e n t in c o n j u n c t i o n w i t h o t h e r g l i d i n g h e m o r r h a g e distort t h e s t u d y . T h i s c o u l d c o s t t h e
structures are essential observations. Both upper patient a n d p h y s i c i a n s t h e o p p o r t u n i t y to o b t a i n
extremities should be examined for possible a n u n d e r s t a n d i n g of t h e e x t e n t of t h e t u m o r a n d
multifocal p r o c e s s e s a n d for metastasis to all its r e l a t i o n s h i p t o i m p o r t a n t surrounding struc-
r e g i o n a l d r a i n i n g l y m p h n o d e s . T h e initial e x a m - tures (Moser a n d P a r r i s h 2001). W h i l e M R I is
ination m a y c o n f i r m t h e clinical s u s p i c i o n , b a s e d p r e f e r r e d f o r soft t i s s u e t u m o r evaluation pre-
o n history, t h a t t h e t u m o r i s , a s s u s p e c t e d , o n e of o p e r a t i v e l y , o n e m u s t k e e p in m i n d t h a t t h e l u n g s
the c o m m o n benign processes. This suspicion a r e t h e m o s t c o m m o n first o r g a n s i n v o l v e d b y
m a y be reinforced or confirmed by simple testing. m e t a s t a s e s f r o m soft t i s s u e s a r c o m a s a n d , t h e r e -
A s p i r a t i o n of c l e a r g e l a t i n o u s fluid f r o m a d o r s a l fore, computed tomography (CT) evaluation of
w r i s t m a s s is c o n f i r m a t o r y , a s is a p l a i n radio- t h e l u n g s is a l s o v a l u a b l e in t h e w o r k - u p of t h e s e
graph that demonstrates the characteristic patients.
s h a d o w s of a n e n c h o n d r o m a . N o f u r t h e r o r m o r e I m a g i n g is e v e n m o r e critically important in
extensive studies are necessary and progression t h e e v a l u a t i o n of a p a t i e n t w i t h a p o s s i b l e b o n e
to t h e s t e p s of d e c i s i o n - m a k i n g r e g a r d i n g man- tumor. Radiographic studies can demonstrate dis-
a g e m e n t c a n b e s a f e l y u n d e r t a k e n . T h i s is t h e tinctive patterns specific for certain b o n e t u m o r s .
most c o m m o n scenario. H o w e v e r , physical e x a m - For example, an enchondroma classically on
ination m a y r e v e a l a s m a l l a n d difficult-to-palpate X - r a y is a w e l l - d e f i n e d i n t r a m e d u l l a r y l e s i o n w i t h
l e s i o n t h a t r e q u i r e s j u d g m e n t a s t o w h e t h e r o r not c a l c i f i e d s p i c u l e s a n d t h e s u r r o u n d i n g c o r t e x is
to proceed with m o r e sophisticated diagnostic t h i n n e d . In a d d i t i o n , imaging provides staging
studies. Larger lesions certainly require careful i n f o r m a t i o n r e g a r d i n g p o s s i b l e i n v a s i o n of s u r -
evaluation, including sophisticated imaging rounding structures. T h e information gathered
s t u d i e s b e f o r e e n o u g h i n f o r m a t i o n is o b t a i n e d to f r o m a p l a i n f i l m of a b o n e t u m o r s h o u l d i n c l u d e :
p r o c e e d to a biopsy. Initial l a b o r a t o r y t e s t s v a r y the location of the tumor, p r e s e n c e of bone
 T U M O R S O F T H E HAND AND U P P E R LIMB 3

d e s t r u c t i o n , p e r i o s t e a l r e s p o n s e , t y p e of tumor differentiating certain p r o c e s s e s , for example,

m a t r i x , a n d t h e n a t u r e of soft t i s s u e i n v o l v e m e n t synovial chondromatosis from chondrosarcoma.
(Greenspan and Remagen 1998). If additional
information is still r e q u i r e d after plain X-rays, Scintigraphy
other imaging studies can provide m o r e tumor
R a d i o n u c l i d e s c a n n i n g is insufficiently sensitive
d e f i n i t i o n . W h i l e C T is better for e v a l u a t i n g b o n y
to a s s i s t in d e l i n e a t i n g o n e t u m o r f r o m a n o t h e r . It
d e s t r u c t i o n , M R I is p r e f e r r e d f o r d e t e c t i n g m a r -
is p r o b a b l y m o s t helpful in i d e n t i f y i n g additional
r o w i n v o l v e m e n t a n d soft t i s s u e e x t e n s i o n . M R I
lesions such as unsuspected bone metastases.
is t h e b e s t c h o i c e for s t a g i n g of a b o n e t u m o r
Therefore, this study m a y h a v e a n important role
b e c a u s e o f its ability t o d e t e c t soft t i s s u e i n v a s i o n
in d e t e r m i n i n g t h e d i r e c t i o n of t r e a t m e n t .
and delineate the tumor's relationship with
neurovascular a n d joint structures (Greenspan
and Remagen 1998, G a r c i a a n d B i a n c h i 2001).
Angiography
B e c a u s e a b i o p s y often t r a u m a t i z e s t h e t i s s u e Traditional a n g i o g r a p h y has g i v e n w a y to M R A a s
p l a n e a n d c a n distort i m a g i n g definition, it is t h e p r e f e r r e d m e t h o d to r e l a t e t u m o r s to a d j a c e n t
important that all radiographic tests be per- important vascular structures.
f o r m e d prior to a n y biopsy p r o c e d u r e s .
T h e a p p r o a c h to t h e a d d i t i o n a l e v a l u a t i o n of Magnetic resonance imaging
the patient, after the initial examination is
M R I h a s b e c o m e t h e m o s t u s e f u l t o o l in s t a g i n g
completed, is a s i m p o r t a n t as the pathologic
of soft t i s s u e a n d b o n e t u m o r s . T h e t e c h n o l o g y
diagnosis that will be determined by biopsy.
h a s i n c r e a s e d e x p o n e n t i a l l y in t e r m s of s p e c i f i c
R e c o m m e n d a t i o n s f o r m a n a g e m e n t rest not o n l y
identification of certain t u m o r s , for example,
o n l o o k i n g a t t i s s u e , but a l s o o n u n d e r s t a n d i n g
schwannomas.
t h e c l i n i c a l , a s w e l l a s r a d i o g r a p h i c , e v a l u a t i o n of
t h e patient. T h e r e f o r e , it is i m p o r t a n t to work
closely with the radiologist, a s w e l l a s the patho- Ultrasonography
logist. T h e i n f o r m a t i o n g a t h e r e d m a k e s it p o s s i b l e Sonography, w h i l e providing far less a n a t o m i c
t o s t a g e t h e t u m o r a n d t h e r e b y g i v e s i n s i g h t into detail than M R I or C T studies, retains a n impor-
t h e b e h a v i o r of t h e n e o p l a s m , a s w e l l a s s u r g i c a l t a n t r o l e in d e l i n e a t i n g c y s t i c f r o m s o l i d t u m o r s ,
m a n a g e m e n t ( H e a r e et al 1989). a n d is far less e x p e n s i v e .

Plain radiographs
O n l y f i l m of h i g h r e s o l u t i o n a n d detail w i l l p r o v i d e Biopsy
u s e f u l i n f o r m a t i o n . C a r e f u l t e c h n i q u e is v i t a l . P l a i n
f i l m s r e m a i n t h e b e n c h m a r k s t u d y in d e t e r m i n i n g T h e r e a r e n o totally p r e c i s e p h y s i c a l f i n d i n g s t h a t
t h e p r e s e n c e a n d l o c a t i o n of b o n e t u m o r s . can distinguish benign from malignant tumors.
T h e o n l y d e f i n i t i v e t e s t to d e t e r m i n e w h e t h e r a
Computed tomography soft t i s s u e o r b o n e t u m o r of t h e h a n d is b e n i g n o r
malignant is pathologic examination of the
H i g h r e s o l u t i o n C T c a n offer i n f o r m a t i o n r e g a r d -
tumor, obtained by biopsy, either incisional or
i n g t h e p r e s e n c e of s m a l l e r b o n e t u m o r s ,
e x c i s i o n a l . B i o p s y is t h e final s t a g e of t h e e v a l u a -
d e m o n s t r a t i n g soft t i s s u e e x t e n s i o n a n d c a l c i f i -
t i o n p r o c e s s . T h e b i o p s y n e e d s to b e c a r e f u l l y
cations within the tumor. C T provides clear dis-
p l a n n e d s o t h a t t h e o p t i o n of p e r f o r m i n g a l i m b
crimination b e t w e e n m e d u l l a r y c a n a l , cortex,
s a l v a g e p r o c e d u r e is p r e s e r v e d . P r e f e r a b l y , t h e
a n d a d j a c e n t soft t i s s u e s . T h e r e f o r e , it is v e r y
person carrying out the definitive surgical proce-
h e l p f u l in t h e s t a g i n g o f t u m o r s in c e r t a i n
dure should also perform the biopsy a n d , there-
anatomic locations.
f o r e , u n d e r s t a n d t h e t i s s u e s that m a y h a v e b e e n
contaminated by the biopsy instruments and h o w
Arthrography best to p l a c e t h e b i o p s y i n c i s i o n .
A r t h r o g r a p h y itself h a s little role. H o w e v e r , w h e n F o r soft t i s s u e t u m o r s , m a n y p r e f e r t o p e r f o r m
c o m b i n e d w i t h C T ( C T a r t h r o g r a p h y ) it c a n a i d in a n i n c i s i o n a l b i o p s y o n larger l e s i o n s , t h e r e b y
4 T U M O R S OF THE HAND

preventing the scattering of tumor fragments ( H a u et al 2002). C l o s e d b i o p s y is a l s o l e s s c o s t l y ,

associated with the 'shelling-out' t y p e of e x c i - m i n i m i z e s soft t i s s u e t r a u m a a n d h e m a t o m a for-
sional biopsy. S m a l l lesions can be excised for m a t i o n , a n d l e a v e s a s m a l l e r b i o p s y t r a c t f o r later
biopsy. However, the actual planning of the e x c i s i o n ( G h e l m a n 1998, S m i t h 1990). T h e m a j o r
b i o p s y is c r u c i a l t o a v o i d c o n t a m i n a t i o n of a d j a - d r a w b a c k of c l o s e d b i o p s y is p o t e n t i a l s a m p l i n g
cent compartments and interference with the error, resulting in an inadequate sample, or
ultimate limb salvage t y p e procedure. O n c e the m i s d i a g n o s i s of t h e t u m o r ( S i m o n 1982).
b i o p s y report is a v a i l a b l e , t h i s is c o m b i n e d w i t h The u s e of a n u p p e r a r m t o u r n i q u e t is a n
the other information described above, and a important consideration w h e n operating on a
pathologic stage c a n be assigned. This should n e o p l a s m of t h e u p p e r e x t r e m i t y . T h e r e i s a t h e o -
help determine prognosis, n e e d for adjuvant retical c o n c e r n for i n c r e a s e d risk of m e t a s t a s e s
t h e r a p y , a n d is a l s o i m p o r t a n t for t h e s t u d y of with tourniquet a p p l i c a t i o n . T h e belief is that
tumors and reporting of results. For those t u m o r c e l l s d i s l o d g e d into t h e b l o o d s t r e a m r e a c h
patients with biopsy-proven malignant tumors, a critical m a s s distal to t h e t o u r n i q u e t a n d s h o w e r
p r e s e n t a t i o n of t h e p a t i e n t t o c o l l e a g u e s in o n c o l - t h e l u n g s w h e n t h e cuff is d e f l a t e d . O n t h e o t h e r
ogy a n d radiation therapy should also be an early hand, increased bleeding at t h e surgical site
c o n s i d e r a t i o n in t h e p r e p a r a t i o n o f t h e p a t i e n t for may result in g r e a t e r l o c a l c o n t a m i n a t i o n and
definitive treatment. h i g h e r r e c u r r e n c e r a t e s . O u r c u r r e n t p r a c t i c e is t o
F o r m a n y b o n e t u m o r s of t h e h a n d , t h e initial u s e a t o u r n i q u e t but e x s a n g u i n a t i o n of t h e a r m is
o p e r a t i o n is d e s i g n e d both t o d e f i n i t i v e l y r e m o v e d o n e t h r o u g h e l e v a t i o n ; a n E s m a r c h b a n d a g e is
t h e t u m o r a n d p r o v i d e t h e p a t h o l o g i s t w i t h suffi- not u s e d .
cient tissue to confirm the strong pre-surgical For a n o p e n biopsy, the incision s h o u l d be
clinical s u s p i c i o n . W h e n t h e history, e x a m i n a t i o n , oriented along the longitudinal a x i s of t h e l i m b
a n d i m a g i n g s t u d i e s d o not c o r r e l a t e t o t a l l y w i t h (Enneking 1983). T h i s makes subsequent re-
o n e a n o t h e r in p r e d i c t i n g t h a t t h e t u m o r is o n e of e x c i s i o n of t h e s c a r e a s i e r , a n d w i l l c a u s e less
the c o m m o n benign processes, then an incisional disruption of t h e axial l y m p h a t i c d r a i n a g e . T h e
b i o p s y s h o u l d b e p e r f o r m e d . B i o p s y of s u s p i - biopsy should be performed sharply, using the
c i o u s b o n y h a n d t u m o r s is d e c e p t i v e l y c h a l l e n g - m o s t d i r e c t a p p r o a c h ; m u s c l e s c a n b e split t o
i n g . T h e p a t h o l o g i s t s h o u l d b e c o n s u l t e d prior t o a c c e s s t h e t u m o r but f l a p s s h o u l d not b e d e v e l -
a b o n e b i o p s y to p r o v i d e i n f o r m a t i o n o n t h e n e e d o p e d to avoid t u m o r spillage. For intramedullary
for s p e c i a l t u m o r p r e p a r a t i o n s a n d t h e quantity tumors, a small oval bone w i n d o w gives access
o f s p e c i m e n n e c e s s a r y for a n a l y s i s ( H e a r e et a l to the t u m o r . T h e resulting b o n y defect s h o u l d be
1989). W h e n p l a n n i n g a b i o p s y , t h e s u r g e o n m u s t sealed with methyl methacrylate to minimize the
a n t i c i p a t e t h a t t h e b i o p s y t r a c t w i l l n e e d to b e d e v e l o p m e n t of a h e m a t o m a ( H e a r e et al 1989).
excised during definitive tumor resection. H e m a t o m a c a n s p r e a d into t h e s u r r o u n d i n g soft
Improper biopsy design c a n c o m p r o m i s e future tissue planes and contaminate a greater area with
l i m b s p a r i n g o p t i o n s a n d result in u n n e c e s s a r y tumor cells; meticulous hemostasis should be
a m p u t a t i o n s . To e n s u r e p r o p e r b i o p s y site p l a c e - maintained throughout the case.
ment, the biopsy a n d the definitive resection To a v o i d s a m p l i n g error, t h e s p e c i m e n m u s t
should be performed by the s a m e surgeon (Heare b e o b t a i n e d f r o m t h e e d g e of t h e t u m o r , a s t h e
et al 1989). A s s u r p r i s e s in m e d i c i n e d o o c c u r , it is c e n t r a l r e g i o n often c o n t a i n s n e c r o t i c t i s s u e . T h e
best t o t r e a t e v e r y b i o p s y w i t h t h e a s s u m p t i o n s p e c i m e n s h o u l d b e h a n d l e d g e n t l y in o r d e r to
t h a t t h e t u m o r is a g g r e s s i v e . a v o i d c r u s h artifact t h a t c o u l d i m p e d e d i a g n o s i s .
The optimal biopsy modality for diagnosis It is p o s s i b l e to a s s u r e a n a d e q u a t e s a m p l e u s i n g
of bone tumors remains controversial. Open i n t r a o p e r a t i v e frozen s e c t i o n s , w i t h t h e s o l e pur-
b i o p s y in t h e o p e r a t i n g r o o m r e m a i n s t h e s t a n - p o s e of a s s e s s i n g if v i a b l e t u m o r c e l l s a r e p r e s e n t
dard for obtaining g e n e r o u s a m o u n t s of tissue within the s a m p l e . B e c a u s e infection m a y m a s -
for pathology examination. Closed biopsy has querade as b o n e tumor, a s a m p l e should be sent
s e v e r a l distinct a d v a n t a g e s a n d the core tech- for culture if t h e r e is a r e m o t e c h a n c e of an
nique can provide accurate diagnostic information infectious etiology. A s e p a r a t e instrument set-up
( M o o r e et al 1979), b e p e r f o r m e d w i t h o u t a n inci- s h o u l d b e u s e d if a n a d d i t i o n a l p r o c e d u r e is per-
sion, and be d o n e under radiographic guidance f o r m e d at a d i s t a n t s i t e . T h e r e h a v e b e e n s e v e r a l
 T U M O R S OF THE HAND AND U P P E R LIMB 5

r e p o r t s of t u m o r s being transplanted to other L o c a t i o n of t u m o r (T) d e s c r i b e s t h e extent of t h e

surgical sites by contaminated instruments (Brien disease a n d T 0 represents an intra-capsular posi-
et al 1997). tion, T 1 a n e x t r a c a p s u l a r but still intracompart-
C h r o m o s o m e a b e r r a t i o n s h a v e b e e n f o u n d in mental tumor l o c a t i o n , a n d , finally, a T 2 lesion,
many benign and malignant tumors (Fletcher w h i c h has extended outside both the capsule a n d
2001). By employing cytogenetic techniques the compartment. T h e compartments represent
t h e s e c h r o m o s o m e a b n o r m a l i t i e s c a n b e u s e f u l in t h e n a t u r a l t i s s u e b a r r i e r s to t u m o r spread. A
d i a g n o s i n g soft t i s s u e t u m o r s . compartment can be defined as intraosseus,
intra-articular, or intrafascial. T h e s u r g e o n must
u n d e r s t a n d t h e a n a t o m y of t h e c o m p a r t m e n t s to
d e l i n e a t e t h e extent of t h e r e s e c t i o n . T h e intra-
Classification - staging f a s c i a l c o m p a r t m e n t s of t h e u p p e r e x t r e m i t y a r e
ray of h a n d , v o l a r f o r e a r m , a n d d o r s a l f o r e a r m
Current concepts concerning the growth and ( E n n e k i n g 1986). T h e c o m p a r t m e n t s of t h e p a l m
b e h a v i o r of a d u l t m a l i g n a n t t u m o r s , a s e x p r e s s e d are divided by fibrous septae and not the
by the A m e r i c a n J o i n t Committee on Cancer and metacarpals. Finally, there are no boundaries
also as understood generally by surgeons and separating the w e b spaces, and thus tumor can
other physicians working in t h i s a r e a , s u g g e s t easily s p r e a d to adjacent proximal phalanges
t h a t t u m o r s g r o w in a c o m p l e x w a y , b a s e d o n ( P a t e l et al 1987).
their o w n genetics, a n d influenced by interactions The nomenclature for the musculoskeletal
with host r e s p o n s e s . If u n c o n t r o l l e d at some staging s y s t e m represents benign t u m o r s with
point in the process, the tumor will spread the A r a b i c n u m b e r s 1 , 2, a n d 3, a n d malignant
t h r o u g h t h e host. T h i s h a s led to t h e A m e r i c a n tumors by the R o m a n numerals I, II, a n d III.
Joint Committee on Cancer Classification of Malignant t u m o r s are further subdivided by the
Tumors (American Joint Committee on Cancer letters A a n d B , w h e r e A represents an intra-
1997) b a s e d o n , a n a t o m i c site a n d histology...'. c o m p a r t m e n t a l lesion a n d B represents a lesion
In devising their staging, they consider that that h a s s p r e a d e x t r a c o m p a r t m e n t a l l y ( E n n e k i n g
similar patterns of growth and extension will et al 1980). F o r e x a m p l e , a n e n c h o n d r o m a c o n -
o c c u r for t u m o r s t h a t a r e in t h e s a m e a n a t o m i c tained within the middle phalanx will be stage 1
l o c a t i o n a n d h a v e t h e s a m e histology. O n c e t h e G 0 T 0 M 0 and an aggressive osteosarcoma con-
anatomic extent of the tumor, its histologic tained within the radius would be s t a g e 11A
g r a d i n g , a n d w h e t h e r o r not t h e l e s i o n h a s m e t a s - G T,M .
2 0

t a s i z e d a r e u n d e r s t o o d , it c a n t h e n b e fit into a
staging s y s t e m .
E n n e k i n g et al (1980) p r o p o s e d a surgically
appropriate s y s t e m the important considerations Surgical treatment
of w h i c h i n c l u d e t h e g r a d e of t h e t u m o r ; that i s ,
w h e t h e r it is l o w - o r h i g h - g r a d e , t h e r e b y s i m p l i f y - T h e local g r o w t h of m a l i g n a n t soft t i s s u e t u m o r s
i n g t h i s s c h e m e into o n e o f t w o g r a d e s , a n d a l s o is in a c e n t r i f u g a l f a s h i o n t h a t c o m p r e s s e s s u r -
w h e t h e r or not t h e l e s i o n w a s m a i n t a i n e d w i t h i n rounding structures producing a pseudocapsule,
a s p e c i f i c a n a t o m i c c o m p a r t m e n t . T h i s t h e y felt w h i c h is not a barrier to e x t e n s i o n of t h e m a l i g -
w a s m o r e important t h a n size. T h i s t h r e e - s t a g e n a n c y . T h i s a r e a of c o m p r e s s e d t i s s u e is m e t b y
s y s t e m is a p p l i c a b l e to b o t h b o n e a n d soft t i s s u e t h e host's r e s p o n s e p r o d u c i n g a n a r e a of e d e m a
sarcomas. and neovascularity termed the 'reactive zone'
T h e r e a r e t h r e e criteria u s e d for s t a g i n g of a t h r o u g h w h i c h e x t e n d ' f i n g e r s of n e o p l a s m g i v i n g
musculoskeletal tumor: histological grade (G), rise to satellite c o l o n i e s ' of t u m o r ( E n n e k i n g et al
a n a t o m i c location (T), a n d the p r e s e n c e of m e t a s - 1981). Surgical dissection within the tumor
tases ( M ) . T h e histological g r a d e is s u b d i v i d e d (intralesional) or marginal dissection through the
into t h r e e a d d i t i o n a l categories based on the ' r e a c t i v e z o n e ' risks l e a v i n g c o l o n i e s of tumor
c e l l u l a r a p p e a r a n c e of t h e s p e c i m e n , s u c h t h a t c e l l s s c a t t e r e d in t h e w o u n d .
G 0 r e p r e s e n t s a b e n i g n p r o c e s s , G , is l o w - g r a d e Good barriers to the early extension of
malignant, and G 2 is high-grade malignant. soft t i s s u e m a l i g n a n c i e s i n c l u d e f a s c i a , poorly
6 T U M O R S OF THE HAND

vascularized ligamentous structures and bone. intraoperatively, which may require a more
However, those soft t i s s u e sarcomas located radical resection. A m p u t a t i o n s h o u l d be o n e v e r y
in extracompartmental locations spread more c o n s e n t f o r m e v e n if a l e s s e x t e n s i v e p r o c e d u r e is
r e a d i l y in t h e l o o s e t i s s u e s t h a n d o t h o s e t u m o r s planned.
within the compartments. T h e surgical margin is t h e a m o u n t of sur-
rounding tissue excised with the tumor. Four
standard d e f i n i t i o n s of surgical margins have
b e e n a d o p t e d to facilitate c o m m u n i c a t i o n among
Considerations for soft tissue tumors h e a l t h - c a r e p r o v i d e r s ( E n n e k i n g 1986).The c o r r e c t
s u r g i c a l m a r g i n f o r a s p e c i f i c n e o p l a s m is d i c -
T h e surgical treatment for benign t u m o r s r a n g e s
tated by the tumor's a g g r e s s i v e potential. A s a
f r o m o b s e r v a t i o n for t h o s e t h a t a r e c l e a r l y n o n -
general rule, the w i d e r the m a r g i n , the l o w e r the
a g g r e s s i v e , to marginal excisions, to occasional
r e c u r r e n c e rate.
w i d e e x c i s i o n s w i t h c a r e to a v o i d i n j u r i n g sur-
I n t r a c a p s u l a r o r i n t r a l e s i o n a l r e s e c t i o n is often
rounding important structures. T h e specifics of
used for the treatment of low-grade benign
t h e m a n a g e m e n t of soft t i s s u e t u m o r s w i l l b e d i s -
t u m o r s , s u c h a s e n c h o n d r o m a s . T h i s is a d e b u l k -
c u s s e d in detail in t h e f o l l o w i n g c h a p t e r s of t h i s
ing procedure performed within the pseudo-
book. H o w e v e r , in general, certain principles
capsule that surrounds the lesion. Intracapsular
a p p l y . B e c a u s e of c o n c e r n r e g a r d i n g dislodging
r e s e c t i o n is often a c h i e v e d b y c u r e t t a g e of the
a n d / o r s p r e a d i n g t u m o r c e l l s , e l e v a t i o n of the
t u m o r c a v i t y . C u r e t t a g e is a v i a b l e o p t i o n w h e n
h a n d for g r a v i t y d r a i n a g e , f o l l o w e d b y inflation of
g r e a t e r t h a n 50 p e r c e n t of t h e c i r c u m f e r e n c e of
a p r o x i m a l t o u r n i q u e t is p r e f e r r e d to e x s a n g u i n a -
cortical bone remains (Vander Griend and
t i o n . H a v i n g b e e n c a r e f u l to c a r r y o u t t h e b i o p s y
F u n d e r b u r k 1993, S h e t h et al 1995).
in s u c h a w a y a s t o a v o i d c o n t a m i n a t i n g a d j a c e n t
c o m p a r t m e n t s , a s w e l l a s avoiding dissection of Marginal excision is appropriate for more
tumor contaminated blood (hematoma) consider- aggressive t u m o r s , such as giant cell t u m o r s a n d

ation of t e c h n i q u e during tumor ablation and a n e u r y s m a l b o n e c y s t s . T h i s e x c i s i o n is p l a c e d

r e c o n s t r u c t i o n is i m p o r t a n t ( F l e e g l e r 1994). After extracapsularly within the reactive zone. A mar-

a b l a t i o n of t h e t u m o r , f r e q u e n t l y by w i d e exci- g i n a l e x c i s i o n c a n b e a c h i e v e d s h a r p l y , s u c h a s in
sion, surgical instruments and other objects can an e n bloc excisional biopsy. M a r g i n a l excision
s e e d t h e w o u n d w i t h t u m o r c e l l s . M e a s u r e s to c a n a l s o b e o b t a i n e d b y c u r e t t a g e of t h e t u m o r ,
e m p l o y t h a t m a y p r e v e n t t h i s i n c l u d e c h a n g e of followed b y local a d j u v a n t t r e a t m e n t . Adjuvant
instruments, gloves, gowns, and drapes, and t h e r a p y t r e a t s t h e t u m o r c a v i t y after curettage
irrigation of t h e w o u n d . P e r h a p s in t h e f u t u r e , u s e thereby extending the zone of resection and

of s p e c i f i c t u m o r i m p l a n t a t i o n inhibitors will be r e m o v i n g a n y r e s i d u a l t u m o r c e l l s . A v a r i e t y of
a v a i l a b l e ( W h a l e n a n d I n g b e r d 1989). local a d j u n c t i v e t h e r a p i e s h a v e b e e n d e s c r i b e d
including high-speed mechanical burring, liquid
nitrogen, a n d phenol ( M a r c o v e and Miller 1969,
Averill et al 1980, Eckardt and Grogan 1986,
Surgical management of bone tumors O'Donnell et al 1994, A t h a n a s i a n et al 1997,
Biscaglia et al 2000, Wittig et al 2001). T h e
Once a bone tumor has been diagnosed and m e c h a n i c a l a c t i o n of a h i g h - s p e e d burr e x t e n d s
s t a g e d , r e s e c t i o n c a n b e p l a n n e d . It is i m p o r t a n t t h e z o n e of r e s e c t i o n into t h e s u r r o u n d i n g cortex.
that t h e p a t i e n t b e c o n s u l t e d prior t o excision, T h e a p p l i c a t i o n of liquid n i t r o g e n t o t h e t u m o r
because the patient's age, occupation, and c a v i t y is a t y p e of c r y o s u r g e r y . T h e liquid n i t r o g e n
lifestyle i n f l u e n c e t h e t y p e of p r o c e d u r e t o be brings the temperature of the b o n e to -21 °C
p e r f o r m e d . T h o s e p a t i e n t s w h o a r e frail or do causing a 1-cm zone of cryonecrosis in the
not w a n t e x t e n s i v e r e c o n s t r u c t i o n m a y b e best surrounding bone (Marcove and Miller 1969,
treated with amputation. The primary aim is S h e t h et al 1995). W h i l e p e r f o r m i n g c r y o s u r g e r y ,
p r e s e r v a t i o n of life a n d t h e r e f o r e c u r a t i v e a m p u - c a r e m u s t b e t a k e n t o retract t h e soft t i s s u e a n d
tation is a l w a y s a consideration. Preoperative neurovascular bundles a w a y from the bone, as
discussion w i t h the patient should c o v e r the pos- low temperatures can cause nerve palsies and
sibility of a m o r e a g g r e s s i v e t u m o r b e i n g found soft t i s s u e n e c r o s i s ( S h e t h et al 1995). B e c a u s e
 T U M O R S O F T H E HAND AND U P P E R LIMB 7

Table 1 Partial list of benign soft tissue lesions of the upper Table 2 Partial list of bone tumors
extremity (Li and Fraumeni 1969)
Benign
Cysts Enchondroma
Inclusion Periosteal chondroma
Ganglion Multiple enchondromatosis
Cutaneous horn (may be associated with a malignancy) Osteochondroma
Dermatofibroma Osteoid osteoma
Fibroma Osteoblastoma
Fibroma of tendon sheath Giant cell tumor of bone
Foreign body reaction Aneurysmal bone cyst
Giant cell tumor of the tendon sheath
Malignant
Glomus tumor
Chondrosarcoma
Gouty mass
Osteogenic sarcoma
Granuloma annulare
Ewing sarcoma
Infectious lesions
Metastatic lesions
Bacterial
Fungal
Viral
Keratoacanthoma (nature of this lesion is still under wide excision resects a rim of normal tissue
investigation)
beyond the reactive zone. A wide margin for
Keratoses
m a l i g n a n t t u m o r s s h o u l d c o n t a i n a 2 - c m cuff of
Actinic*
h e a l t h y t i s s u e ( H a u et al 2002). F r o z e n s e c t i o n s
Arsenical*
taken during the procedure e n s u r e that no resid-
Irradiation*
Seborrheic ual t u m o r is left b e h i n d .
Leiomyoma Radical excision requires r e m o v a l of all the
Lipoma t i s s u e in t h e c o m p a r t m e n t in w h i c h t h e tumor
Melanosis o r i g i n a t e d . T h i s t y p e of e x c i s i o n is r e s e r v e d for t h e
Myxoma most aggressive malignant tumors, such as
Neurilemmoma a high-grade chondrosarcoma. For example,
Neurofibroma
a radical excision of an intramedullary tumor
Nevi
r e q u i r e s t h e entire b o n e to b e r e s e c t e d . R a d i c a l
Nodular fasciitis
e x c i s i o n often results in a u s e l e s s h a n d a n d a m p u -
Porokeratosis
Pyogenic granuloma tation m a y offer a better f u n c t i o n a l o u t c o m e .
Sweat gland tumors
Vascular tumors
Arteriovenous malformations Incidence of tumors of the
Hemangioma
Lymphangioma
hand and upper limb (see
Tables 1 and 2)
*May be premalignant
S t a c k (1960) a n a l y z e d 300 c o n s e c u t i v e tumors.
Of t h e s e 61 p e r c e n t w e r e g a n g l i o n s , 10 p e r c e n t
c r y o s u r g e r y i n d u c e s a w i d e z o n e of b o n e injury, e p i d e r m a l i n c l u s i o n c y s t s , a n d 29 p e r c e n t o t h e r
late f r a c t u r e s d o o c c u r . T h i s c o m p l i c a t i o n c a n b e d i a g n o s e s . Of t h e o t h e r d i a g n o s e s ( n = 27) 17 p e r
p r e v e n t e d b y filling t h e d e f e c t w i t h b o n e graft or c e n t w e r e b o n e t u m o r s , 14 p e r c e n t skin c a n c e r s ,
c e m e n t ( M a r c o v e et al 1978, E n n e k i n g et al 1993). 11 p e r c e n t t e n d o n s h e a t h , 10 p e r c e n t v a s c u l a r
Treating the t u m o r cavity w i t h phenol theore- t u m o r s , a n d 33 per cent miscellaneous t u m o r s .
tically d e s t r o y s t h e t u m o r c e l l s t h r o u g h c o a g u l a -
t i o n of c e l l u l a r p r o t e i n s . A l l of t h e s e t r e a t m e n t s
a r e u s e d to e x t e n d c u r e t t a g e f r o m a n i n t r a l e s i o n a l Incidence of malignant tumors
r e s e c t i o n into a m a r g i n a l r e s e c t i o n .
W i d e e x c i s i o n is i n d i c a t e d for m o r e a g g r e s s i v e In a s t u d y of 73 p a t i e n t s a d m i t t e d o v e r 20 y e a r s
tumors, such as low-grade osteosarcomas. A for management of malignant tumors of the
8 T U M O R S O F T H E HAND

h a n d , t h e i n v e s t i g a t o r s f o u n d t h a t s q u a m o u s cell Enneking W F (1986) A system of staging musculoskeletal

tumors accounted for 78 per cent, basal cell neoplasms, Clin Orthop Mar: 9-24.
t u m o r s 10 p e r c e n t , a n d m e l a n o m a s a c c o u n t e d
for 3 per cent. Of the remaining 9 per cent, Enneking WF, Dunham W , Gebhardt M C et al (1993) A
2 per c e n t w e r e c h o n d r o s a r c o m a s , a n d 1 per c e n t system for the functional evaluation of reconstructive
procedures after surgical treatment of tumors of the
f i b r o s a r c o m a s ( K e n d a l l et al 1969).
musculoskeletal system, Clin Orthop Rel Res 286:241-6.

Fleegler E (1994) Tumors - Surgical Principles. In:

References Kasdan ML, Amadio P C , Bowers W H , eds. Technical

Tips For Hand Surgery. Hanley and Belfus, Inc., Mosby:
St Louis: 219-27.
American Joint Committee on Cancer (1997) American
Joint Committee on Cancer Staging Manual, 5th edn.
Lippincott-Raven: Philadelphia. Fletcher T (2001) Cytogenetic analysis of soft tissue
tumors. In: Weiss S W , Goldblum J R , eds. Enzinger and
Athanasian E A , W o l d L E , Amadio PC (1997) Giant cell Weiss's Soft Tissue Tumors, 4th edn. Mosby: St. Louis:
tumors of the bones of the hand, J Hand Surg Am 125-46.
22:91-8.
Garcia J , Bianchi S (2001) Diagnostic imaging of tumors
Averill R M , Smith R J , Campbell C J (1980) Giant-cell of the hand and wrist, Eur Radiol 11:1470-82.
tumors of the bones of the hand, J Hand Surg Am
5:39-50. Ghelman B (1998) Biopsies of the musculoskeletal
system, Radiol Clin North Am 36:567-80.
Biscaglia R, Bacchini P, Bertoni F (2000) Giant cell tumor
of the bones of the hand and foot, Cancer 88:2022-32. Greenspan A, Remagen W (1998) Differential Diagnosis
of Tumors and Tumor Like Lesions of Bone and Joints.
Brien E W , Mirra J M , Kerr R (1997) Benign and malignant Lippincott-Raven: NewYork.
cartilage tumors of bone and joint: their anatomic and
theoretical basis with an emphasis on radiology, patho- Hau A, Kim I, Kattapuram S , Hornicek FJ et al (2002)
logy and clinical biology, Skeletal Radiol 26:325-53. Accuracy of CT-guided biopsies in 359 patients with
musculoskeletal lesions, Skeletal Radiol 31:349-53.
Campbell DA, Millner PA, Dreghorn CR (1995) Primary
bone tumours of the hand and wrist, J Hand Surg Br HeareTC, Enneking WF, Heare M M (1989) Staging tech-
20:5-7. niques and biopsy of bone tumors, Orthop Clin North
Am 20:273-85.
Clark W , Reimer R, Greene M et al (1978) Origin of
familial malignant melanoma from heritable Jaffe H (1958) Tumors and Tumorous Conditions of the
melanocytic lesions (the B-K mole syndrome), Arch Bones and Joints. Lea & Febiger: Philadelphia.
Dermatol 114:732-8.
Kendall T, Robinson D, Masters F (1969) Primary malig-
Eckardt J J , G r o g a n T J (1986) Giant cell tumor of bone, nant tumors of the hand, Plast Reconstr Surg 44:37-40.
Clin Orthop Rel Res 204:45-58.
Li F, Fraumeni J (1969) Soft-tissue sarcomas, breast can-
Enneking W , Spanier S , and Goodman M (1980) The cer, and other neoplasms, a familial syndrome? Ann
surgical staging of musculoskeletal sarcoma, J Bone Intern M e d 71:747-52.
Surg 62A:1027-30.
Marcove RC, MillerTR (1969)Treatment of primary and
Enneking W , Spanier S , Malawer M (1981) The effect of metastatic bone tumors by cryosurgery, J Am Med
the anatomic setting on the results of surgical proce- Assoc 207:1890-^1.
dures for soft parts sarcoma of the thigh, Cancer
47:1005-22. Marcove RC, W e i s LD, Vaghaiwalla MR et al (1978)
Cryosurgery in the treatment of giant cell tumors of
Enneking W F (1983) Musculoskeletal Tumor Surgery. bone. A report of 52 consecutive cases, Cancer
Churchill Livingstone: N e w York. 41:957-69.
 T U M O R S O F T H E HAND AND U P P E R LIMB 9

Moore T M , Meyers M H , Patzakis M J et al (1979) Closed S i m o n M A (1982) Biopsy of musculoskeletal tumors,

biopsy of musculoskeletal lesions, J Bone Joint Surg J Bone Joint Surg 64:1253-7.
61:375-80.
Smith R J (1990) Benign and Malignant BoneTumors of
Moser R J r , Parrish W (2001) Radiologic evaluation of the Hand, 3rd edn. W B Saunders: Philadelphia: 5510-28.
soft tissue tumors. In: Weiss S W , Goldblum J R , eds.
Enzinger and Weiss's SoftTissueTumors, 4th ed. Mosby: Stack H (1960)Tumors of the hand, Br Med J 1:919-22.
St. Louis: 45-102.
Vander Griend RA, Funderburk CH (1993) The treatment
O'Donnell R J , Springfield D S , Motwani HK et al (1994) of giant-cell tumors of the distal part of the radius,
Recurrence of giant-cell tumors of the long bones after J Bone Joint Surg 75:899-908.
curettage and packing with cement, J Bone Joint Surg
76:1827-33. W h a l e n G , Ingberd E (1989) Inhibition of tumor cell
attachment to extracellular matrix as a method for pre-
Patel M R , Desai S S , Gordon S L et al (1987) venting tumor recurrence in a surgical w o u n d , Ann
Management of skeletal giant cell tumors of the pha- Surg 76:758-64.
langes of the hand, J Hand Surg Am 12:70-7.
Wittig J C , Simpson B M , Bickels J et al (2001) Giant cell
Sheth D S , Healey J H , Sobel M et al (1995) Giant cell tumor of the hand: superior results with curettage,
tumor of the distal radius, J Hand Surg Am 20:432-40. cryosurgery, and cementation, J Hand Surg Am 26:546-55.
2
Hand tumors at l'Institut de la Main
Caroline Leclercq, Elena Mateva, Carlos Rueda
and the members of l'Institut de la Main, Paris, France

Table I Soft tissue tumors

Introduction
Tumor %
F o r t h e p u r p o s e o f t h e s y m p o s i u m of I'lnstitut d e
la M a i n h e l d o n t h e 2 1 - 2 2 M a r c h 2003 (Paris, Ganglia 59.5
France), dedicated to t u m o r s of t h e hand and Mucous cyst 11
u p p e r l i m b , w e r e v i e w e d t h e files of all t u m o r s of Giant cell tumor 10
t h e h a n d t r e a t e d at I'lnstitut d e la M a i n b e t w e e n Vascular tumor 4.7
1995 a n d 2002. T h e r e w e r e 1952 files a v a i l a b l e . Glomus tumor 4.6
A m o n g t h e s e , t u m o r s of t h e soft t i s s u e s w e r e b y Lipomas 3.6
Nerve tumors 3
far t h e m o s t f r e q u e n t (87.8 p e r c e n t ) , t u m o r s of
Fibromatous tumors 1.6
b o n e a c c o u n t i n g for 6.6 p e r c e n t , a n d t u m o r s of
Miscellaneous 2
t h e skin a n d nail for 5.5 p e r c e n t . Surprisingly,
t h e r e w e r e t w i c e a s m a n y f e m a l e s (68 p e r c e n t ) a s
m a l e s (32 p e r c e n t ) in t h i s s e r i e s .

p r e d o m i n a n c e (66 p e r c e n t v e r s u s 34 p e r c e n t ) ,
but the age at occurrence was much older
Soft tissue tumors ( a v e r a g e 60 y e a r s ) , a s m u c o u s c y s t s a r e u s u a l l y
related to D I P joint d e g e n e r a t i v e arthritis. T h e
T h e r e w e r e 1714 t u m o r s of t h e soft t i s s u e s in t h e long finger w a s the most frequently involved
s e r i e s ( T a b l e 1). (37 per cent) and the little f i n g e r the least
G a n g l i a , w h i c h a r e not, in fact, t r u e t u m o r s , but f r e q u e n t l y (12 p e r c e n t ) .
a r e g e n e r a l l y i n c l u d e d in s e r i e s of h a n d tumors, Giant cell tumors of the tendon sheath
r e p r e s e n t m o r e t h a n half of t h e soft t i s s u e t u m o r s a c c o u n t e d for 10 p e r c e n t of soft t i s s u e t u m o r s
(59 per cent) in t h i s s e r i e s . T h e y o c c u r r e d at (173 c a s e s ) . W o m e n w e r e a g a i n m o r e f r e q u e n t l y
all a g e s ( 1 - 9 0 y e a r s ) , w i t h a n a v e r a g e a g e of i n v o l v e d t h a n m e n (107 v e r s u s 66), a n d a v e r a g e
39 y e a r s . T h e y involved three times as many a g e a t s u r g e r y w a s 49 y e a r s . T h e v a s t m a j o r i t y o f
w o m e n a s m e n (72 p e r c e n t v e r s u s 28 p e r c e n t ) . these lesions involved the fingers (119 cases),
G a n g l i a i n v o l v e d m o s t l y soft t i s s u e s of t h e w r i s t w h e r e a s the metacarpal area w a s involved in
(76 p e r c e n t ) , l e s s f r e q u e n t l y t h e f i n g e r s (19 per 11 c a s e s , a n d t h e w r i s t in s e v e n .
c e n t ) , a n d rarely t h e m e t a c a r p a l a r e a (3 p e r c e n t ) . Vascular tumors are difficult to classify.
In 2 per cent of cases the ganglion was Angiodysplasias include angiomas and
i n t r a o s s e o u s , m o s t l y in t h e c a r p a l b o n e s . a n e u r y s m s , w h e t h e r acquired or congenital. True
M u c o u s c y s t s a r e a s p e c i f i c t y p e of ganglia, vascular tumors are extremely rare. O u r series
involving the dorsum of the D I P joint. T h e y comprised 82 v a s c u l a r t u m o r s (4.7 per cent),
a c c o u n t e d for 11 p e r c e n t of soft t i s s u e t u m o r s in i n c l u d i n g 62 c a s e s of a n g i o m a s , f o u r a n e u r y s m s ,
o u r s e r i e s . A s in g a n g l i a , t h e r e w a s a l a r g e f e m a l e two lymphangiomas, and s e v e n true vascular
12 T U M O R S O F T H E HAND

Table 2 Vascular tumors Table 3 Bone tumors

Vascular tumors No. of cases Bone tumors No. of cases

Angioma 62 Chondroma 70
Aneurysm 4 Osteochondroma 19
Lymphangioma 2 Intraosseous ganglion 19
Thrombosis 5 Osteoid osteoma 10
Angioleiomyoma 6 Osteochondromatosis 2
Venous malformation 1 Intraosseous epidermal cyst 2
Hemangioendothelioma 1 Primary malignant tumor 4
Hamartoma 1 Metastasis 2

Total 82 Total 128

tumors. T h e figures for e a c h t y p e of v a s c u l a r tissue t u m o r s , there w a s a large f e m a l e predomi-

tumor a r e d i s p l a y e d in T a b l e 2 . A v e r a g e a g e at n a n c e (67 p e r c e n t ) . A v e r a g e a g e at s u r g e r y w a s
surgery w a s 43 y e a r s , w i t h a f e m a l e to m a l e pre- 40.3 y e a r s ( r a n g e 2-77 y e a r s ) . N i n e t y - f i v e p e r c e n t
d o m i n a n c e of 55 : 27. of t h e b o n e t u m o r s w e r e b e n i g n , 3 p e r c e n t w e r e
Our s e r i e s i n c l u d e d 79 g l o m u s t u m o r s of t h e primary malignancies, a n d 2 per cent w e r e bone
h a n d (4.6 p e r c e n t of soft t i s s u e t u m o r s ) , a l m o s t metastases.
e x c l u s i v e l y l o c a t e d in t h e f i n g e r s , a n d m o s t l y in T h e f r e q u e n c e of e a c h t u m o r t y p e is r e p o r t e d
the fingertips (pulp or subungual). Seventy in T a b l e 3 . C h o n d r o m a s w e r e b y f a r t h e most
per c e n t o c c u r r e d in w o m e n . T h e t h u m b a n d ring f r e q u e n t b o n e t u m o r s of t h e u p p e r l i m b (56 p e r
finger w e r e involved m o r e frequently, but this cent), located mainly in t h e p h a l a n g e s (60 out
w a s not statistically significant. of 70). O s t e o c h o n d r o m a s w e r e t h e s e c o n d m o s t
L i p o m a s a r e not a s f r e q u e n t in t h e h a n d a s in frequent bone tumors (16 p e r c e n t ) , w i t h the
t h e p r o x i m a l part of t h e l i m b . T h e r e w e r e 62 lipo- s a m e i n c i d e n c e a s i n t r a o s s e o u s g a n g l i a (16 p e r
mas in t h i s s e r i e s (3.6 p e r c e n t ) , 4 5 of which c e n t ) , w h i c h a r e not, in fact, b o n e t u m o r s , but
involved the wrist and hand, the rest being r a t h e r soft t i s s u e l e s i o n s w i t h i n t h e b o n e .
l o c a t e d m o r e p r o x i m a l l y in t h e u p p e r l i m b . T h e r e The m a j o r i t y of bone tumors in t h i s series
w a s a 1 : 1 s e x ratio. A v e r a g e a g e at s u r g e r y w a s were located in t h e p h a l a n g e s (66 p e r cent),
54 y e a r s . w h e r e a s 20 p e r c e n t w e r e in t h e c a r p u s , 8.5 p e r
N e r v e t u m o r s i n v o l v e d 53 c a s e s in this s e r i e s c e n t in t h e m e t a c a r p a l s , a n d t h e r e m a i n d e r in t h e
(3 p e r c e n t of soft t i s s u e t u m o r s ) . T h e m o s t f r e - forearm and arm.
q u e n t t y p e , b y far, w a s s c h w a n n o m a s (44 c a s e s ) ,
a n d t h e r e w e r e six c a s e s of n e u r o f i b r o m a s , o n e of
w h i c h w a s part of v o n R e c k l i n g h a u s e n ' s d i s e a s e .
Average a g e w a s 46 y e a r s , and the male to
f e m a l e ratio w a s 1.
Skin tumors
Fibromatous tumors involved mostly fibromas
The i n c i d e n c e of skin a n d nail t u m o r s in our
or histiocytofibromas. There w e r e three cases
s e r i e s w a s 5.5 p e r c e n t (108 c a s e s ) . T h i s f i g u r e is
of m i x o i d f i b r o m a s , a n d o n e c a s e of fibroma-
p r o b a b l y not r e p r e s e n t a t i v e of t h e t r u e i n c i d e n c e
t o s i s . T h e i r i n c i d e n c e in soft t i s s u e t u m o r s w a s
of skin t u m o r s in t h e h a n d , a s a l a r g e n u m b e r a r e
1.6 p e r c e n t .
t r e a t e d in d e r m a t o l o g y u n i t s r a t h e r t h a n in h a n d
u n i t s s u c h a s o u r s . T h e m o s t f r e q u e n t of t h e s e
tumors were epidermal and sebaceous cysts

Bone tumors (40 p e r c e n t ) , m e l a n o m a s (11 p e r c e n t ) , p y o g e n i c

g r a n u l o m a s (10 p e r c e n t ) , a n d B o w e n ' s d i s e a s e (9
There were 128 bone tumors, representing per c e n t ) . T a b l e 4 s h o w s t h e f i g u r e s for e a c h kind
6.6 p e r c e n t of t h e w h o l e s e r i e s . A s in t h e soft of skin t u m o r .
 H A N D T U M O R S AT L'INSTITUT DE LA MAIN 13

Table 4 Skin tumors lesions, such a s Dupuytren's disease, and post-

traumatic neuromas. Other reported series are
Skin tumors No. of cases m u c h s m a l l e r : P o s c h (1956: 679 c a s e s ) , B o g u m i l l
(1975: 129 c a s e s ) , J o h n s o n (1985: 543 c a s e s ) , a n d
Epidermal and sebaceous cyst 44
G l i c e n s t e i n et al (1988: 471 c a s e s ) . O u r s e r i e s of
Melanoma 12
Pyogenic granuloma 11 1952 c a s e s b r i n g s f u r t h e r e v i d e n c e t h a t soft t i s s u e
Bowen's disease 10 t u m o r s a r e t h e m o s t f r e q u e n t t y p e o f t u m o r in t h e
Carcinoma 7 h a n d , t h a t m a l i g n a n c y is v e r y rare in t h e h a n d ,
Nevus 7 a n d s h e d s a n e w light o n t h e s e x ratio, w o m e n
Wart 5 being involved twice as much a s m e n . A s a w h o l e ,
Keratosis 3 the most frequent l e s i o n s w e r e : g a n g l i a (52 p e r
Miscellaneous 9
cent), mucous c y s t s (9.8 per cent), giant cell
Total 108 t u m o r s (8.8 p e r c e n t ) , g l o m u s t u m o r s (4 p e r c e n t ) ,
chondromas (3.5 p e r c e n t ) , a n g i o m a s (3.1 per
cent), lipomas (3.1 per cent), schwannomas
(2.2 p e r c e n t ) , a n d e p i d e r m a l a n d s e b a c e o u s c y s t s

Malignant tumors (2.2 p e r c e n t ) .

A s a w h o l e , t h e r e w e r e 42 m a l i g n a n t t u m o r s in t h i s
s e r i e s , s h o w i n g a n i n c i d e n c e of 2.1 per cent of all
t u m o r s . M o s t w e r e c u t a n e o u s (30 c a s e s ) , including References
12 m e l a n o m a s , 11 B o w e n ' s d i s e a s e , six epithe-
Bogumill GP, Sullivan D J , Baker Gl (1975) Tumors of the
liomas, and o n e Merckel tumor. Malignant tumors hand, Clin Orthop 108:214-22.
of t h e soft t i s s u e s (six c a s e s ) i n c l u d e d f o u r p r i m a r y
sarcomas and two metastatic carcinomas. Bone Glicenstein J , Ohana J , Leclercq C (1988) Tumours of the
m a l i g n a n c y (six c a s e s ) i n v o l v e d f o u r p r i m a r y sar- Hand. Springer-Verlag: Berlin: 230.
c o m a s a n d t w o metastatic c a r c i n o m a s .
Haber M H , Alter A H , W h e e l o c k M C (1965)Tumors of the
hand, Surg Gynecol Obstet 121:1073-80.

Conclusion J o h n s o n J (1985)Tumorous lesions of the hand, J Hand

Surg 10A:284.
T h e l a r g e s t s e r i e s of t u m o r s of t h e h a n d reported
to date was by Haber in 1965. It comprised Posch J L (1956)Tumors of the hand, J Bone Joint Surg
2321 cases, but included many non-tumoral 38A:517-39.
3
Imaging of tumors and tumor-like
lesions of the hand and fingers
NicolasTheumann and Jean-Luc Drape

Introduction Table 1
tissues
Relative magnetic resonance signal intensity of

T u m o r s a n d t u m o r - l i k e l e s i o n s of t h e h a n d a r e
T1-weighted T2-weighted
m o r e f r e q u e n t l y b e n i g n l e s i o n s rather t h a n n e o -
p l a s i c d i s e a s e s ( B e r q u i s t 2001). Different imaging Cortical bone very low very low
methods including plain f i l m ultrasound ( U S ) , Calcium very low very low
c o m p u t e d t o m o g r a p h y (CT), a n d magnetic reso- Tendon/ligament low low
n a n c e i m a g i n g ( M R I ) of t h e h a n d a n d w r i s t c a n b e Yellow marrow high intermediate
performed. Plain film X-rays must be obtained Red marrow low intermediate
Fat high intermediate
in all patients since they are extensive and
Most tumors intermediate high
p a n o r a m i c . U S , if p e r f o r m e d b y a n e x p e r i e n c e d
o p e r a t o r , c a n a c c u r a t e l y a s s e s s t h e internal struc-
t u r e of t h e m a s s a n d its relation w i t h a d j a c e n t
nerves and vessels. Moreover, color Doppler U S
and/or b o n e scintigraphy a r e essential to e v a l u a t e
c a n a s s e s s t h e i n t e r n a l v a s c u l a r i t y . B e c a u s e of its
t h e p r e s e n c e of m e t a s t a t i c l e s i o n s .
multiplanar capability and excellent tissue
contrast, M R I has recently r e p l a c e d C T in the
a s s e s s m e n t of t h e e x t e n t of t h e m a s s . M R I is a n
i m p o r t a n t m e t h o d for a s s e s s i n g d i s o r d e r s of t h e Bone tumors
m u s c u l o s k e l e t a l t i s s u e s . M R I is particularly s e n s i -
t i v e t o b o n e m a r r o w a n d is h i g h l y effective in Bone tumors of the hand are uncommon.
d e t e c t i n g a n d c h a r a c t e r i z i n g a w i d e v a r i e t y of soft H o w e v e r , b e n i g n t u m o r s a n d tumor-like c o n d i t i o n s
t i s s u e c o n d i t i o n s . A d v a n c e s in s u r f a c e coil t e c h - occur more frequently than malignant neoplasms
n o l o g y h a v e i n c r e a s e d t h e u s e f u l n e s s of M R I in (Alam et al 1999, C a m p a n a c c i 1999). T a b l e 2
the evaluation of joint disease. MR images summarizes the most c o m m o n benign and malig-
d e p e n d o n p a r a m e t e r s that reflect t i s s u e c h e m i c a l n a n t b o n e lesions in t h e h a n d ( W i l n e r 1982, D a h l i n
c h a r a c t e r i s t i c s ( s e e T a b l e 1). A l t h o u g h phospho- and U n n i 1986, M c C u l l o u g h a n d T h o m i n e 1995,
rus, n i t r o g e n , a n d s o d i u m n u c l e i m a y b e i m a g e d C a m p a n a c c i 1999). A s a g e n e r a l r u l e , clinical f i n d -
w i t h M R I , it is t h e d i s t r i b u t i o n of t h e hydrogen ings and standard radiography allow accurate
n u c l e u s t h a t f o r m s t h e b a s i s of M R I . H o w e v e r , C T d i a g n o s i s of t h e m a j o r i t y of b o n e t u m o r s l o c a t e d
r e m a i n s t h e m e t h o d of c h o i c e for e v a l u a t i n g b o n e in t h e h a n d a n d w r i s t . C T r e m a i n s t h e best m o d a l -
i n v o l v e m e n t of soft t i s s u e t u m o r s a n d s m a l l b o n e ity for a s s e s s m e n t of b o n e d e s t r u c t i o n , w h e r e a s
tumors such a s osteoid osteoma. W h e r e a malig- MRI can evaluate bone marrow involvement and
nant tumor is considered, thoraco-abdominal t u m o r e x t e n s i o n in t h e a d j a c e n t soft t i s s u e s .
16 T U M O R S OF THE HAND

Table 2 Most common bone tumors in the hand (percentage involvement are useful features. E n h a n c e m e n t
of each tumor in the hand) after injection of g a d o l i n i u m is not s p e c i f i c .
Chondroblastoma of the hand is extremely
Percentage
r a r e . Clinical a n d s o m e i m a g i n g f i n d i n g s a r e s i m i -
lar t o t h o s e of e n c h o n d r o m a , a l t h o u g h chondro-
Benign
Enchondroma 54 blastoma m o r e c o m m o n l y affects t h e e p i p h y s i s
Giant cell tumor 12 rather t h a n t h e d i a p h y s i s a n d m a y p r e s e n t a l a r g e
Intraosseous ganglia 8.5 a r e a of b o n e m a r r o w a n d soft t i s s u e e d e m a w h i c h
Osteoid osteoma 6 is v e r y w e l l d e p i c t e d o n M R I . L o c a l r e c u r r e n c e
Aneurysmal bone cyst 6 after s u r g i c a l r e m o v a l a n d m a l i g n a n t d e g e n e r a -
Osteochondroma 4 t i o n c a n o c c u r ( G a r c i a a n d B i a n c h i 2001).
Osteoblastoma 4
Chondromyxoid fibroma 4
Exostosis 3
Fibrous dysplasia 3 Giant cell tumors
Benign vascular tumor 2.5
G i a n t cell t u m o r s r e p r e s e n t a b o u t 5 p e r c e n t of
Malignant
b e n i g n b o n e t u m o r s ( M a n a s t e r a n d D o y l e 1993).
Malignant vascular tumor 6
T h e y a r e u s u a l l y o b s e r v e d in y o u n g p a t i e n t s a n d
Malignant fibrous histiocytoma 3
Chondrosarcoma 2 o r i g i n a t e f r o m t h e e p i p h y s i s a n d c a n e x p a n d into
Metastasis 0.5 the metaphysis and diaphysis. Local aggressive
Osteosarcoma 0.4 b e h a v i o r e x p l a i n s t h e h i g h r a t e of r e c u r r e n c e after
Fibrosarcoma 0.2 s u r g e r y . R a d i o g r a p h i c a l l y , a p u r e lytic l e s i o n in
t h e m e t a p h y s i s is o b s e r v e d ( F i g . 2). It c a n e x t e n d
to t h e s u b c h o n d r a l b o n e . T h e m a r g i n s c a n b e w e l l
o r p o o r l y d e f i n e d . B o t h C T a n d M R I a d d little t o
t h e d i a g n o s i s . M R I f e a t u r e s m a y s h o w a r e a s of
However, when a malignant tumor is
l o w signal intensity o n T 2 - w e i g h t e d i m a g e s s u g -
suspected, exhaustive presurgical evaluation and
g e s t i n g t h e p r e s e n c e of h e m o s i d e r i n deposition
diagnostic biopsy are warranted. D u e to their
( A o k i et al 1996).
superficial l o c a t i o n , m a l i g n a n t t u m o r s of t h e h a n d
c a n b e d i a g n o s e d e a r l y a n d t r e a t e d s u c c e s s f u l l y if
their possibility is kept in m i n d ( B o g u m i l l 1988).

Aneurysmal bone cysts

A n e u r y s m a l bone cysts are rarely s e e n about the
Enchondromas
h a n d ( F u h r a n d H e r n d o n 1979). T h e m o s t c o m -

E n c h o n d r o m a is t h e m o s t c o m m o n b e n i g n b o n e m o n locations are the phalanges a n d metacarpal

t u m o r in t h e h a n d a n d w r i s t . A b o u t 40 p e r c e n t of bones followed by carpal bones. Plain film

t h e s e t u m o r s i n v o l v e t h e h a n d ( B o g u m i l l 1988). d e m o n s t r a t e s a lytic e n l a r g e d l e s i o n , w h i c h often

M o s t are detected incidentally o n routine plain c a u s e s a b l o w - u p of t h e i n v o l v e d b o n e . S e p t a t i o n s

films or after pathologic fractures. W h e n the or t r a b e c u l a t e s a r e c o m m o n . M R I s h o w s a w e l l -

lesion becomes symptomatic without fracture, defined, lobulated, septated lesion with multiple

malignant degeneration should be considered f l u i d - f l u i d l e v e l ( H u d s o n 1984). F l u i d - f l u i d levels

a n d e v a l u a t e d ( B e r q u i s t 2001). R a d i o g r a p h i c a l l y , a r e not s p e c i f i c for a n e u r y s m a l b o n e c y s t s , s i m i -

radiolucent well-defined medullar lesions, con- lar f e a t u r e s h a v e b e e n d e s c r i b e d in o t h e r b e n i g n

taining thin orbicular, tiny punctuated a n d scat- and malignant lesions.

tered calcifications of t h e cartilage matrix are

o b s e r v e d ( F i g . 1). M a g n e t i c r e s o n a n c e f e a t u r e s of
e n c h o n d r o m a are lobular high signal intensity o n Intraosseous ganglion cysts
T2-weighted spin echo s e q u e n c e s , or isointense
s i g n a l o n T 1 - w e i g h t e d s e q u e n c e s in c o m p a r i s o n Intraosseous ganglion cysts appear commonly
w i t h m u s c l e s . C o r t i c a l d e s t r u c t i o n a n d soft t i s s u e a s s m a l l , w e l l - c i r c u m s c r i b e d , o v a l lytic lesions,
 IMAGING O F T U M O R S AND TUMOR-LIKE L E S I O N S 17

Figure 1

Fractured enchondroma of the base of second metacarpal

bone. (a) Plain film, (b) coronal STIR image, (c) axial
Tl-weighted spin echo images and id) axial fat-saturatedT1-
weighted postenhanced spin echo image. Note the strong
b enhancement of this benign tumor.

bordered by a sclerotic ring o n plain film of

Malignant tumors
the wrist a n d h a n d . Internal m u c u s content is
s h o w n o n M R I ( l o w to intermediate signal on M a l i g n a n t b o n e t u m o r s are rare. H o w e v e r , m e t a s -
T1- and high signal on T2-weighted images) t a s e s of t h e h a n d a r e m o r e f r e q u e n t t h a n p r i m a r y
( T a n a k a 1995). bone tumors. Phalanges are more commonly
 18 T U M O R S OF THE HAND

Figure 2 Figure 3

Giant cell tumor of the fourth metacarpal bone. Plain film. Chondrosarcoma of the third metacarpal bone.

involved than metacarpal and wrist bones.

Radiographically, metastases appear a s aspecific
lytic a g g r e s s i v e l e s i o n s . P l a i n f i l m a n d M R I a r e Table 3 Most common soft tissue tumors in the hand
the best imaging methods to evaluate tumor (percentage of each tumor in the hand)
extension a n d relation with surrounding struc-
t u r e s ( F i g . 3). H o w e v e r , t o d a t e , M R I h a s not b e e n Percentage
u s e f u l for h i s t o l o g i c a l d i f f e r e n t i a t i o n of m a l i g n a n t
Benign
bone lesions.
Intraosseous ganglia
Glomus tumor 52
Giant cell tumor of the tendon sheath 44
Hemangioma 12
Soft tissue masses Nodular fasciitis 11
Lipoma 6
Soft tissue t u m o r s are m o r e frequent than b o n e
t u m o r s in t h e h a n d a n d w r i s t . T h e m a j o r i t y of soft Malignant
t i s s u e m a s s e s a r e b e n i g n (Table 3) ( L e c l e r c q a n d Epithelioid sarcoma 32
Malignant fibrous histiocytoma 0.2
G l i c e n s t e i n 1995).
Fibrosarcoma 4
R a d i o g r a p h s m a y d e m o n s t r a t e v a s c u l a r calcifi-
Extraskeletal chondrosarcoma 18
c a t i o n s , localized soft t i s s u e m a s s e s , a n d a d j a c e n t
 IMAGING O F T U M O R S AND TUMOR-LIKE L E S I O NS 19

b o n e c h a n g e s . M R I w i t h its m u l t i p l a n a r capability
and e x c e l l e n t soft t i s s u e contrast remains the
method of choice. U S remains an effective
examination if performed by a n experienced
radiologist.

Benign tumors
Ganglion cysts
W r i s t g a n g l i a a r e t h e m o s t c o m m o n soft t i s s u e
m a s s of t h e h a n d a n d w r i s t . T h e y a r e p a i n l e s s ,
l o c u l a t e d m a s s e s , filled b y m u c o u s fluid a n d lined
by a fibrous w a l l , located close to t h e sensorial
tissue of t h e t e n d o n sheath or joint c a p s u l e
( L e c l e r c q a n d G l i c e n s t e i n 1995, R a z e m o n 1995).
T h e m o s t c o m m o n site of g a n g l i o n c y s t s is t h e
d o r s a l a s p e c t of t h e w r i s t a n d distal interpha-
langeal joints. Ganglia c a n c o m m u n i c a t e w i t h the
adjacent joint cavity b y a o n e - w a y v a l v e , w h i c h
a l l o w s t h e s y n o v i a l fluid t o m o v e f r o m o n e j o i n t
to t h e cyst. H o w e v e r , they m a y enlarge or resolve
spontaneously. M R I demonstrates a well-defined
lesion w i t h l o w signal intensity o n T 1 - a n d high
s i g n a l i n t e n s i t y o n T 2 - w e i g h t e d i m a g e s ( F i g . 4)
(Blam e t a l 1998). C o m p l i c a t e d ( h e m o r r h a g e )
Figure 4
g a n g l i a m a y c a u s e v a r i a t i o n in s i g n a l intensity.
Deep ganglion cyst originating from the scaphotrapezoid
Giant cell tumors of the tendon sheath joint and extending through the carpal tunnel. Sagittal
T2-weighted image.
G i a n t cell t u m o r s of t h e t e n d o n s h e a t h ( G C T T S )
a r e t h e s e c o n d m o s t c o m m o n soft t i s s u e m a s s in
the hand. G C T T S a r e localized f o r m s of v i l l o -
m o s t b e n i g n t u m o r s a r e g a n g l i o n c y s t s , g i a n t cell
nodular tenosynovitis (Leclercq and Glicenstein
t u m o r s of t h e t e n d o n s h e a t h , e p i d e r m o i d c y s t s ,
1995). T w o f o r m s of G C T T S c a n o c c u r : t h e local
a n d h e m a n g i o m a s . Clinically, t h e y d o n o t differ
form (nodular tenosynovitis) presents a s a nodu-
f r o m l i p o m a , e n c o u n t e r e d in o t h e r l o c a t i o n s . M R I
lar m a s s t y p i c a l l y in t h e h a n d o r w r i s t ; t h e dif-
s h o w s a high signal o n b o t h T 1 - a n d T 2 - w e i g h t e d
f u s e d f o r m of w h i c h t a k e s p l a c e n e a r l a r g e j o i n t s ,
imaging a n d c a n accurately depict the extension
represents a n extra-articular extension of pig-
of t h e l e s i o n . A l t h o u g h , t h e y m a y b e w e l l - d e f i n e d
mented villonodular s y n o v i t i s ( G o o d m a n et a l
septation or lobulated appearance, there is a
1997). C h a r a c t e r i s t i c p a t h o l o g i c f i n d i n g s include
n o n - f a t s i g n a l in s i m p l e b e n i g n l i p o m a ( D o o m s
loose connective tissue with multinucleated giant
et a l 1985).
cells a n d h e m o s i d e r i n inclusions. B o n e e r o s i o n ,
T h e r e a r e a n u m b e r o f different l i p o m a s , inter-
d u e t o t h e p r e s s u r e of t h e m a s s o n b o n e c o r t e x
m u s c u l a r o r i n t r a m u s c u l a r l i p o m a , l i p o m a of t h e
c a n o c c u r . M R I s h o w s i s o s i g n a l t o t h a t of m u s c l e
tendon sheaths, a n d neural fibrolipoma ( A m a d i o
on T 1 - and heterogeneous intensity on T2-
et al 1988). I n t r a m u s c u l a r l i p o m a s m a y infiltrate
w e i g h t e d s e q u e n c e s ( F i g . 5).
between muscle fibers, showing an irregular
m a r g i n c o m p a r e d w i t h t h e w e l l - d e f i n e d m a r g i n of
Lipomas benign lipomas a n d c a n extend along tendons
L i p o m a s a r e t h e fifth m o s t f r e q u e n t b e n i g n t u m o r (Fig. 6). N e r v e f i b r o l i p o m a s are slow-growing
of t h e h a n d ( 5 p e r c e n t ) ( G o o d m a n et al 1997).The tumors, predominantly present during early
20 T U M O R S OF THE HAND

b C

Figure 5

Giant cell tumor of the flexor common profundus tendon. (a) Plain film: volar thickening of the soft tissues and bone erosion
of the base of the distal phalanx. (b) Sagittal T2-weighted gradient echo image: peripheral hemosiderin deposits. (c) Pre- and
postenhanced axial T1-weighted spin echo image.

a d u l t h o o d . T h e m o s t c o m m o n n e r v e i n v o l v e d is ( B e r q u i s t a n d K r a n s d o r f 2003). A l t h o u g h skin a n d
t h e m e d i a n n e r v e (80 p e r c e n t ) . C a r p a l t u n n e l subcutaneous lesions are easily diagnosed at
syndrome is t h e most common clinical sign physical examination, d e e p h e m a n g i o m a requires
( S i l v e r m a n a n d E n z i n g e r 1985). M R I s h o w s s e r - a n a d d i t i o n a l i m a g i n g m e t h o d for p r o p e r e v a l u a -
piginous structures, hypointense on T 1 - a n d T2- tion ( T h e u m a n n et al 2001).
w e i g h t e d i m a g e s , c o r r e s p o n d i n g to t h e fibrous R a d i o g r a p h s r e v e a l r o u n d e d r e g u l a r calcifica-
c o m p o n e n t b e d d i n g in a d i p o s e t i s s u e ( h i g h s i g n a l tions within the mass, corresponding to phle-
o n b o t h s e q u e n c e s ) ( F i g . 7) ( M i l l e r et al 1994). b o l i t h e s c o n t a i n e d in t h e l o w - f l o w v e s s e l s . U S a n d
color Doppler, easily demonstrate the vascular
o r i g i n of t h e m a s s . M R I a l s o s h o w s c h a r a c t e r i s t i c
Vascular tumors or malformations f e a t u r e s . S e r p i g i n o u s l o w s i g n a l intensity struc-
H e m a n g i o m a s a r e t h e fourth c o m m o n e s t soft tissue t u r e , w i t h i n h i g h s i g n a l intensity o n T 1 - w e i g h t e d
b e n i g n lesion of t h e h a n d (7 per cent) ( T h e u m a n n i m a g e s , t h e l e s i o n s a r e diffusely h y p e r i n t e n s e o n
et al 2001). Hemangiomas may contain non- T2-weighted sequences. Phlebolithes are seen
v a s c u l a r e l e m e n t s , s u c h a s fatty t i s s u e , s m o o t h as round a r e a s of l o w s i g n a l intensity ( F i g . 8)
muscle, fibrous tissue, a n d bone. H e m a n g i o m a s ( B e r q u i s t a n d K r a n s d o r f 2003).
a r e u s u a l l y classified a c c o r d i n g to v e s s e l size a s M R I r e m a i n s a n e x c e l l e n t m e t h o d to e v a l u a t e
c a v e r n o u s (large v e s s e l ) or capillary ( s m a l l v e s s e l s ) t h e size, m a r g i n , a n d r e l a t i o n s h i p w i t h s u r r o u n d i n g
 IMAGING O F T U M O R S ANDTUMOR-LIKE LESIONS 21

Figure 7

Fibrolipoma of the median nerve. Sagittal T1-weighted spin

echo image.

1992, T h e u m a n n et al 2002). T h e y a r e arterio-

v e n o u s a n a s t o m o s e s r e s p o n s i b l e for t h e r m o r e g -
ulation. T h e y present a s afferent arteriol and
b tortuous arteriovenous anastomoses collecting
v e i n s a n d a neurovascular s y s t e m that regulates
Figure 6
f l o w t h r o u g h t h e a n a s t o m o s e s ( T h e u m a n n et al
Intramuscular lipoma. (a) Coronal T1-weighted spin echo 2002). G l o m u s t u m o r s are very small hamar-
image and (b) axial fat-saturated T1-weighted postenhanced tomas of the neuromyoarterial apparatus
image. (< 1 c m ) . T h e y c a n o c c u r a n y w h e r e in t h e b o d y but
most are located in the fingertips, usually
beneath the finger n a i l s . M o s t l e s i o n s o c c u r in
3 0 - 5 0 - y e a r - o l d p a t i e n t s . C l i n i c a l d i a g n o s i s is often
structures. M R angiography confirms the vascular
difficult a n d m a y b e d e l a y e d f o r u p t o 5 y e a r s
nature and m a y reveal the vessels feeding the
( B o u d g h e n e et al 1998). G l o m u s t u m o r s r e p r e s e n t
lesion.
a b o u t 1.5-5 p e r c e n t of all h a n d t u m o r s ( D r a p e
et al 1996). L e s i o n s m a y b e p a i n f u l , often e x a c e r -
Glomus tumor b a t e d b y c h a n g e s in t e m p e r a t u r e .
Glomus bodies are present in the dermis D e t e c t i o n a n d a c c u r a t e localization is critical a s
t h r o u g h o u t t h e b o d y , but t h e y a r e m o s t e v i d e n t in a complete resection is m a n d a t o r y to relieve
t h e d i g i t s of t h e h a n d a n d f o o t ( G r e e n s p a n et al s y m p t o m s ( D a l r y m p l e et al 1997). R a d i o g r a p h s
22 T U M O R S O F T H E HAND

c d

Figure 8

Vascular malformation of the fourth finger. (a) Longitudinal color Doppler view, (b) axial T1-weighted spin echo image:
vascular channels on the ulnar side of the finger, (c) coronal fat-saturated postenhancedT1-weighted coronal slice: enhance-
ment mixed with flow void artifacts and, (d) magnetic resonance angiography.
 IMAGING OF T U M O R S AND TUMOR-LIKE L E S I O N S 23

Figure 9

Subungual glomus tumor. (a) Plain film: dorsal bone erosion,

(b) axial T1-weighted spin echo image, (c) postenhanced T1-
weighted image and (d) magnetic resonance angiography:
delayed acquisition. Strong enhancement of the tumor. d

can demonstrate smooth bone erosion Lesions are well defined and hypointense on
( D a l r y m p l e et al 1997). M R I a n d M R a n g i o g r a p h y T1 and hyperintense o n T 2 (Fig. 9).They present a
h a v e a n i m p o r t a n t role in d e t e c t i o n a n d c h a r a c - homogeneous high e n h a n c e m e n t after intra-
t e r i z a t i o n of g l o m u s t u m o r s ( D r a p e e t al 1996). venous gadolinium injection ( D a l r y m p l e et al
24 T U M O R S OF THE HAND

Figure 10

Schwannoma of the deep branch of the ulnar nerve. Axial

postenhanced T1-weighted image.

Figure 12

Desmoid tumor. (a) Axial T1-weighted spin echo image and

(b) T2-weighted fast spin echo image. Low signal on
T2-weighted image of the tumor infiltrating the fist web.

Nerve tumors
Neurofibromas and s c h w a n n o m a s are rare in
the wrist and hand. Multiple neurofibromas can
be found in von Recklinghausen's disease.
S c h w a n n o m a s present a s a fusiform mass and
more commonly affect the ulnar nerves
( A n d e r s o n et al 1988). B e n i g n n e r v e t u m o r s s h o w
Figure 11 i s o i n t e n s i t y to m u s c l e s o n T 1 - a n d h y p e r i n t e n s i t y
o n T 2 - w e i g h t e d i m a g e s ( F i g . 10) ( A n d e r s o n et al
Synovialosarcoma of the wrist. Axial postenhanced 1988).The 'split f a t ' s i g n r e p r e s e n t s t h e p e r i p h e r a l
T1-weighted image. The tumor infiltrates the carpal tunnel. r i m of fat s i g n a l i n t e n s i t y s u r r o u n d i n g t h e l e s i o n .
T h e t a r g e t s i g n ( s e e n in n e u r o f i b r o m a s ) is a l o w
central region with high peripheral signal inten-
sity o n T 2 - w e i g h t e d s e q u e n c e s , c o r r e s p o n d i n g t o
1997, T h e u m a n n e t at 2002). S u r g i c a l e x c i s i o n
central fibrosis and surrounding myxomatosis
r e m a i n s t h e best treatment for g l o m u s t u m o r s .
t i s s u e ( B e r q u i s t 2001).
R e c u r r e n c e is c o m m o n ( 5 - 5 0 p e r c e n t ) ( T h e u m a n n
et al 2002). P o s t - s u r g e r y , M R I h a s p r o v e d to b e a
v e r y u s e f u l m e t h o d t o differentiate scare from
tumor recurrence with that p r e s e n t e d in non-
Malignant soft tissue tumors
typical glomus tumor presentation (Theumann Soft tissue s a r c o m a s of the hand are very
et al 2002). uncommon (Weiss and Goldblum 2001).
 IMAGING O F T U M O R S ANDTUMOR-LIKE LESIONS 25

Malignant sarcomas show bone erosions and Berquist T, Kransdorf M (2003) Tumors and tumor-like
s o m e t i m e s matrix calcifications on plain films. conditions. In: Berquist TH, ed. MRI of the Hand and
M R I a l l o w s t h e b e s t a s s e s s m e n t of t u m o r e x t e n - Wrist. Lippincott Williams & Wilkins: Philadelphia.
sion. Although differentiation between benign
and m a l i g n a n t l e s i o n s at M R I r e m a i n s difficult, Blam 0, Bindra R, MiddletonW, Gelberman R (1998)The
occult dorsal carpal ganglion: usefulness of magnetic
i n t e r n a l irregularity is t h e m o s t s p e c i f i c criteria in
resonance imaging and ultrasound in diagnosis, Am J
the d i a g n o s i s of a malignant tumor (Manaster
Orthop 27:107-10.
and Doyle 1993). S a r c o m a s a r e t y p i c a l l y p o o r l y
marginated.They are hypointense o n T 1 - and not
Bogumill G P (1988) Tumors of the wrist. In: Lichtmann
homogeneously hyperintense on T2-weighted D M e d . The Wrist and its Disorders. S a u n d e r s :
sequences. They show irregular enhancement Philadelphia: 373-84.
after i n t r a v e n o u s g a d o l i n i u m i n j e c t i o n ( F i g . 11).
Necrotic areas which do not enhance may be B o u d g h e n e FP, Gouny P, Tassart M et al (1998)
noted. Dynamic MRI sequences can help to Subungual glomus tumor: combined use of MRI and
d i a g n o s e s a r c o m a w h e n t h e c u r v e of v i s u a l i z a - three-dimensional contrast M R angiography, J Magn
tion s h o w s n e o a n g i o g e n e s i s criteria. M R I also Reson Imaging 8:1326-8.
r e m a i n s t h e best i m a g i n g m e t h o d for d e t e c t i o n of
r e c u r r e n c e . D e m o n s t r a t i o n of l o c a l n o d u l e s w i t h Campanacci M (1999) Bone and Soft Tissue Tumors.
Springer-Verlag: New York.
e n h a n c e m e n t after g a d o l i n i u m i n j e c t i o n is the
b e s t c r i t e r i o n of l o c a l r e c u r r e n c e .
Dahlin D, Unni K (1986) Bone Tumors: General Aspect
D e s m o i d t u m o r s are locally a g g r e s s i v e benign
and Data on 8542 Cases, 4th edn. Charles C Thomas:
lesions that s h o w a malignant c o m p a r t m e n t on Springfield, IL.
MRI. The presence of longitudinal low signal
i n t e n s i t y ( c o r r e s p o n d i n g to f i b r o m a t i s s u e ) w i t h i n Dalrymple NC, Hayes J , B e s s i n g e r V J et al (1997) MRI of
an heterogeneous m a s s , should suggest the diag- multiple glomus tumors of the finger, Skeletal Radiol
n o s i s ( F i g . 12). T h e v a s c u l a r i z a t i o n of desmoid 26:664-6.
tumor with dynamic M R I sequences shows a
quick a n d i m m e d i a t e e n h a n c e m e n t a s m a l i g n a n t Dooms GC, Hricak H, Sollitto RA, Higgins C B (1985)
t u m o r ( K a s a k o w a et al 1999). Lipomatous tumors and tumors with fatty component:
MR imaging potential and comparison of MR and CT
results, Radiology 157:479-83.

References Drape J L , Idy-Peretti I, Goettmann S et al (1996)

Standard and high resolution magnetic resonance
Alam F, Schweitzer M E , Li X X et al (1999) Frequency imaging of glomus tumors of toes and fingertips, J Am
and spectrum of abnormalities in the bone marrow of the Acad Dermatol 35:550-5.
wrist: MR imaging findings, Skeletal Radiol 28:312-17.
Fuhr S E , Herndon J H (1979) Aneurysmal bone cyst
Amadio P C , Reiman H M , Dobyns J H (1988) involving the hand. A review and report of two cases,
Lipofibromatous hamartoma of nerve, J Hand Surg Am J Hand Surg 4:152-9.
13:67-75.
Garcia J , Bianchi S (2001) Diagnostic imaging of tumors
Anderson M , Kaplan P, Dussault R et al (1988) Magnetic of the hand and wrist, Eur Radiol 11:1470-82.
resonance imaging of the wrist, Curr Probl Diagn Radiol
27:187-229. Goodman H J , Richards A M , Klaassen M F (1997) Use of
magnetic resonance imaging on a large lipoma of the
Aoki J , Tanikawa H, Ishii K et al (1996) MR findings hand: a case report, Aust NZ J Surg 67:489-91.
indicative of hemosiderin in giant-cell tumor of bone:
frequency, cause, and diagnostic significance, Am J Greenspan A, McCahan J P , Vogelsang P, Szabo R M
Roentgenol 166:145-8. (1992) Imaging strategies in the evaluation of soft tissue
hemangiomas of the extremities: correlation of the find-
BerquistT (2001) MRI of the Musculoskeletal System, ings on plain radiography, angiography, CT, MRI and
4th edn. Lippincott Williams & Wilkins: Philadelphia: ultrasonography in 12 histologically proven cases,
773-841. Skeletal Radiol 21:11-8.
26 T U M O R S OF THE HAND

Hudson T (1984) Fluid levels in aneurysmal bone cysts: Razemon J P (1995) Les kystes du poignet. ln:Tubiana R
A CT feature, Am J Roentgenol 142:1001-4. ed. Chirurgie de la Main. Masson: Paris: 627-33.

KasakowaY, Okoda K, Khashimoto M et al (1999) Extra- Silverman TA, Enzinger F M (1985) Fibrolipomatous
abdominal desmoid tumor of the hand: a case report hamartoma of nerve. A clinicopathologic analysis of
and review of the literature, Tokyo J Exp Med 189: 26 cases, Am J Surg Pathol 9:7-14.
163-79.
Tanaka H, ArakiY,Yamamoto H et al (1995) Intraosseous
ganglion, Skeletal Radiol 24:155-77.
Leclercq C, Glicenstein J (1995) Tumeurs des parties
molles de la main. In: Tubiana R ed. Chirurgie de la
Main. Masson: Paris: 753-72. Theumann NH, Bittoun J , Goettmann S et al (2001)
Hemangiomas of the fingers: M R imaging evaluation,
Radiology 218:841-7.
Manaster B J , Doyle A J (1993) Giant cell tumors of bone,
Radiol Clin North Am 31:299-323.
Theumann NH, Goettmann S , Le Viet D et al (2002)
Recurrent glomus tumors of fingertips: M R imaging
McCullough C J , T h o m i n e J - M (1995) Tumeurs osseuses evaluation, Radiology 223:143-51.
de la main. In: Tubiana R ed. Chirurgie de la Main.
Masson: Paris: 732-52. Weiss S , Goldblum J (2001) Enzinger and W e i s s ' soft
tissue tumors, 4th edn. Mosby: S t . Louis.
MillerTT, Potter H G , McCormack RR (1994) Benign soft
tissue masses of the wrist and hand: M R I appearances, Wilner D (1982) Radiology of bone tumors and allied
Skeletal Radiol 23:327-32. disorders. Saunders: Philadelphia.
4
Giant cell tumor of
the tendon sheath
Daniel V Egloff and Debora loanna

Introduction
2 0 $$

18 -

16 -
G i a n t cell t u m o r of t h e t e n d o n s h e a t h ( G C T T S )
14 -
w a s first d e s c r i b e d in 1852 b y C h a i s s a i g n a c . It is
$$
12 -
a rarely encountered lesion in routine clinical $$
$$
$$
10 -
p r a c t i c e , w i t h o n l y o n e or t w o c a s e s a y e a r per "o
$$ 8 -
100 0 0 0 p o p u l a t i o n ( L o o i et al 1999). H o w e v e r , it z
6 -
is t h e s e c o n d m o s t c o m m o n soft t i s s u e t u m o r , t h e
4 -
commonest being synovial ganglion. In our
2 -
c e n t e r of h a n d s u r g e r y , it r e p r e s e n t s 0.5 per c e n t
or a p p r o x i m a t e l y o n e c a s e for e v e r y 200 e l e c t i v e 0 -

0-9 10-19 20-29 30-39 40-49 50-59 60-69 70-79 80-89 90-99
operations.
Age (years)
M a n y different n a m e s h a v e b e e n g i v e n , such a s
'tumeur à myéloplaxes' ( u s e d in French o n l y ) , Figure 1
nodular tenosynovitis, pigmented villonodular
synovitis, fibrous xanthoma, benign synovioma, Age and frequency in our series.
a n d s c l e r o s i n g h e m a n g i o m a ( R o d r i g u e s et al 1998,
Reilly et al 1999, A l - Q a t t a n 2 0 0 1 , P e h e t al 2001).
G C T T S o c c u r s in a d u l t s w i t h m o s t c a s e s o c c u r -
ring in t h e f o u r t h d e c a d e , but t h e a g e of p a t i e n t s
c a n r a n g e f r o m 6 to 81 y e a r s , w i t h a f e m a l e to (1) Neoplastic (supported by studies based o n
male predominance of 1.25-2.7: 1 (Rodrigues D N A a n a l y s i s ) ( A b d u l - K a r i m et al 1992, D e
et al 1998, U r i b u r u a n d L e v y 1998, L o o i e t al 1999, Saint Aubain Somerhausen and Fletcher
D e S a i n t A u b a i n S o m e r h a u s e n a n d F l e t c h e r 2000, 2000);
A l - Q a t t a n 2 0 0 1 , P e h e t al 2001). In o u r s e r i e s of (2) Inflammatory;
72 p a t i e n t s , t h e m e a n a g e w a s 53 y e a r s ( r a n g e (3) Trauma-induced (history of antecedent
12-82 y e a r s ) , w i t h t w o p e a k s , o n e at 50 a n d t h e trauma is p r e s e n t in b e t w e e n 1 a n d 50 p e r
o t h e r a t 70 y e a r s w i t h a f e m a l e to m a l e p r e d o m i - c e n t in s t u d i e s , but m o s t of t h e t i m e there
n a n c e of 1.8 : 1 ( F i g . 1). is a p o o r a s s o c i a t i o n w i t h t r a u m a , l e s s t h a n
Its c a u s e a n d p a t h o g e n e s i s a r e u n c l e a r and 5 per cent, 4.2 per cent in our series)
controversial. Several hypotheses have been (Rodrigues et al 1998, Looi et al 1999,
p r o p o s e d ( F l a n d y a n d H u g h s t o n 1987, F r o i m s o n A l - Q a t t a n 2001);
1987, H a n s e n et al 1988, G l o w a c k i a n d W e i s s 1995, (4) Immune-mediated;
R e i l l y et al 1999): (5) D i s t u r b a n c e of lipid m e t a b o l i s m .
28 T U M O R S O F T H E HAND

G C T T S is c o n s i d e r e d to b e a b e n i g n t u m o r , but
w i t h a h i g h l e v e l of local r e c u r r e n c e . R a r e c a s e s
Clinical features
have been reported to g i v e rise to metastatic In o u r s e r i e s , t h e d e l a y b e t w e e n a p p e a r a n c e a n d
d i s e a s e ( C a s t e n s a n d H o w e l l 1979, N e i l s o n a n d first m e d i c a l c o n s u l t a t i o n w a s 16.9 m o n t h s ( d a t a
K l a n 1989, D e S a i n t A u b a i n S o m e r h a u s e n a n d a v a i l a b l e for 47 cases) and has ranged from
Fletcher 2000). 2 m o n t h s to 20 y e a r s in o t h e r s e r i e s ( R o d r i g u e s
In m o s t c a s e s (70 p e r c e n t ) , G C T T S is localized et al 1998, L o o i et al 1999, A l - Q a t t a n 2001).This is
in t h e h a n d s , but it c a n o c c u r in o t h e r j o i n t s , par- d u e p r i m a r i l y to t h e a b s e n c e of p a i n , but a l s o t h e
ticularly knees, ankles, a n d e l b o w s (Rodrigues v e r y s l o w g r o w t h of t h e t u m o r p l a y s a part. T h e
et al 1998, D e S a i n t A u b a i n S o m e r h a u s e n a n d long delay before a recurrence also suggests a
F l e t c h e r 2000). s l o w g r o w t h . In o u r s e r i e s t h e a v e r a g e d e l a y f o r
t h e s e v e n r e c u r r e n c e s w a s 52 m o n t h s .
Although usually painless, s y m p t o m s pro-
v o k e d b y a c t i v i t y a n d c o m p r e s s i o n of a d j a c e n t
Classification s t r u c t u r e s m a y o c c u r , s u c h a s ( L o o i et al 1999, P e h
et al 2001):
S e v e r a l classifications h a v e been described:
(1) S e n s o r y d i s t u r b a n c e ( G i b b o n s et al 2002);
(2) Nerve involvement giving rise t o p a i n and
(1) In 1968, B y e r s et al c l a s s i f i e d t h e f a m i l y of
numbness;
pigmented villonodular synovitis into two
(3) S n a p p i n g a n d triggering m a y be present a s
types (De Saint Aubain Somerhausen and
t h e m a s s r i s e s in s t r a t e g i c p o s i t i o n s in t h e
F l e t c h e r 2000):
fingers;
(4) M o v e m e n t restriction ( L o o i et al 1999, R e i l l y
(a) Localized nodular type (extra-articular): et al 1999);
c o m m o n l y s e e n in t h e h a n d . (5) Tenosynovitis m a y occur with acute onset
(b) Diffuse t y p e : ( K r u i t h o f et al 2002).
(i) C o m m o n l y s e e n in l a r g e j o i n t s s u c h
a s t h e k n e e , but c a n o c c u r in t h e In o u r s e r i e s of 72 c a s e s , p a t i e n t s c o m p l a i n e d of:
wrist or h a n d . painless swelling, without functional impairment
(ii) T h i s t y p e is m o r e a g g r e s s i v e l o c a l l y in 73.6 p e r c e n t of c a s e s , s w e l l i n g w i t h p a i n ( m o s t
and recurrence is m o r e frequent. often slight) in 16.7 p e r c e n t , s w e l l i n g w i t h f u n c -
T h e t r e a t m e n t is m o r e r a d i c a l , w i t h tional i m p a i r m e n t in 5.5 p e r c e n t , a n d swelling
potentially m o r e complications. a s s o c i a t e d w i t h p r e v i o u s t r a u m a in 4.2 p e r c e n t .
T h e s e s y m p t o m s w e r e a l m o s t identical t o pre-
(2) A n o t h e r classification based o n the t e n d e n c y s e n t i n g s y m p t o m s in o t h e r s e r i e s . In 76-90 per
of GCTTS for recurrence and invasion of c e n t of c a s e s , t h e c o m p l a i n t is a p a i n l e s s s w e l l i n g .
adjacent structures has been correlated by F o r t w o patients t h e d i s c o v e r y of t h e t u m o r w a s
recent immunohistochemical research fortuitous: o n e presented w i t h s y m p t o m s of a
( G r o v e r et al 1998). G i a n t cell t u m o r s w i t h a c a r p a l t u n n e l f r o m a G C T T S in t h e c a r p u s . T h e
deficit of e x p r e s s i o n of t h e g e n e n m 23 a r e s e c o n d patient h a d s y m p t o m s of arthritis b e t w e e n
more aggressive and are associated with a the scaphoid and capitate.The tumor occupied the
high r e c u r r e n c e r a t e . T h e n e g a t i v i t y of this joint s p a c e a n d w a s p r e s e n t in t h e c a p i t a t e .
g e n e is a c t u a l l y u s e d t o d e t e r m i n e t h e p r o g -
n o s i s of m e l a n o m a ( L o o i et al 1999, G a l l i a n i
et al 2000).
(3) GCTTS can be classified according to Location
m a c r o s c o p i c ( t u m o r s u r r o u n d e d o r not sur-
rounded with a pseudocapsule) or micro- T h e c o m m o n e s t s i t e s a r e t h e first t h r e e digits,
scopic (high or l o w cellularity) a p p e a r a n c e particularly the index finger ( T a b l e 1), similar
( A l - Q a t t a n 2001). observations h a v e been noted in o t h e r series
 GIANT CELL TUMOR OF THE TENDON SHEATH 29

Table 1 Sites of giant cell tumors of the tendon sheath in Table 3 Extension of giant cell tumors of the tendon sheath
our series of 72 cases in our 72 cases

No. of cases Percentage Percentage

Thumb 9 12.5 No extension 52.7

Index finger 25 34.7 Tendon sheath 20.8
Middle finger 14 19.4 Joint capsule or periosteum 15.2
Ring finger 6 8.3 Intra-articular 11.1
Little finger 7 9.7 Intraosseous 2.7
Palm 6 8.3
Wrist 5 6.9

underneath the skin. S o m e t i m e s G C T T S extends

into a d j a c e n t s t r u c t u r e s ( T a b l e 3 ) . T r u e e x t e n s i o n
Table 2 Level of location of giant cell tumors of the tendon into a j o i n t is r a r e a n d b o n e i n v a s i o n e v e n m o r e
sheath in our series of 72 cases seldom.
T h e o r i g i n of t h e t u m o r is difficult to deter-
No. of cases Percentage
m i n e , a l t h o u g h in o u r s e r i e s all f o u r surgeons
DIP and IP of 34 47.2 a t t e m p t e d to f i n d its o r i g i n . T h r e e s t r u c t u r e s w e r e
the thumb incriminated: the t e n d o n s h e a t h , the joint c a p s u l e ,
PIP 21 29.1 and the p e r i o s t e u m . T r u e infiltration of these
MP 8 11.1 structures either macroscopically or histologically
CM 3 4.1 w a s never documented.
Midcarpal 6 8.3

DIR distal interphalangeal joints; IR interphalangeal joint;

PIP. proximal interphalangeal joints; M P metacarpopha-
langeal joint; C M carpometacarpal joint. Differential diagnosis
(evaluation by the four
surgeons of our series)
( R o d r i g u e s et al 1998, R e i l l y et al 1999, A l - Q a t t a n
2 0 0 1 , P e h e t al 2001), e x c e p t for o n e A s i a t i c s e r i e s , In o u r s e r i e s , t h e clinical differential diagnosis
in w h i c h t h e c o m m o n e s t site w a s t h e ring f i n g e r between s c h w a n n o m a , neurinoma, neurofibroma,
( L o o i et al 1999). It s e e m s t h a t t h e r e is a p r e d o m i - synovial ganglion, epidermic cyst, surnumerous
n a n c e of t h e right s i d e ( U r i b u r u a n d L e v y 1998, muscle, myxoid cyst, and tenosynovial cyst w a s
R e i l l y et al 1999). T h e l e s i o n is f o u n d m o r e f r e - undertaken by the four s u r g e o n s .
q u e n t l y in t h e v o l a r t h a n in t h e d o r s a l a s p e c t of
t h e h a n d a n d w r i s t ( v o l a r site in 63.8 p e r c e n t of
c a s e s in o u r s e r i e s a n d 57 per c e n t of c a s e s in t h e
s e r i e s of Reilly et a l (1999)). L e s i o n s c a n a l s o b e Histopathology
c i r c u m f e r e n t i a l (5.5 p e r c e n t of o u r c a s e s ) .
Grossly, GCTTS is seen as a rubbery, well-
T h e l e s i o n s a r e m a i n l y l o c a t e d at t h e l e v e l of
e n c a p s u l a t e d m u l t i n o d u l a r m a s s , w i t h s m o o t h but
t h e i n t e r p h a l a n g e a l j o i n t s (76.3 p e r c e n t of l o c a -
l o b u l a t e d c o n t o u r . It c a n b e t a n , b r o w n or y e l l o w
t i o n s ( T a b l e 2)).
in color, d e p e n d i n g o n t h e p r o p o r t i o n of foam
cells a n d d e g r e e of h e m o s i d e r i n d e p o s i t i o n w i t h i n
t h e l e s i o n ( R o d r i g u e s et al 1998, F o l p e et al 1999,

Clinical status G a l l i a n i et al 2000, P e h et al 2 0 0 1 , R o d r i g u e z -

P e r a l t o et al 2001). In o u r s e r i e s t h e a v e r a g e size
T h e l e s i o n is t y p i c a l l y p a l p a b l e , of v a r i a b l e c o n - w a s 2.5 m l , w i t h 78.5 p e r c e n t of t u m o r s being
sistency, often multinodular and painless, smaller than 3 ml ( F i g . 2 ) , this c o r r e l a t e d with
attached to d e e p e r structures but freely mobile o b s e r v a t i o n s of o t h e r s (Table 4 ) .
30 T U M O R S OF THE HAND

M i c r o s c o p i c a l l y , t h e l e s i o n is s u r r o u n d e d b y a Table 4 Size of tumors in other series

collagenous capsule subdivided into smaller
No. of
nodules. It is c h a r a c t e r i s t i c a l l y hypervascular.
cases Tumor volume
GCTTS is composed of multinucleated giant
cells, p o l y h e d r a l histiocytes, f o a m cells, a n d m a c r o - 0.5-3 cm
Rodrigues et al 28
p h a g e s w i t h p i g m e n t a t i o n f r o m intra- a n d extra- (1998)
cellular h e m o s i d e r i n d e p o s i t i o n , p r e s u m a b l y a s a Reilly et al (1999) 107 < 1 cm in 25% of cases,
result of r e p e a t e d h e m o r r h a g e ( F i g . 3). In s t r o m a 1-2 cm in 59%, and
reticulin a n d c o l l a g e n f i b e r s a r e p r e s e n t . T h e r e > 2 cm in 16%
are varying d e g r e e s of f i b r o s i s and cellularity.
S o m e t i m e s , cellular a t y p i a is p r e s e n t ( L o o i et al
1999). A t y p i c a l mitotic f e a t u r e s f r e q u e n t l y e n c o u n -
normal structures which can be displaced by the
t e r e d a r e f o c a l p h e n o m e n a w i t h i n t h e g i a n t cell
p a t h o l o g i c m a s s but n e v e r infiltrated b y it ( F i g . 4).
t u m o r s . It m a y indicate a n a c t i v e l y g r o w i n g lesion
T h e surgical excision h a s to be c o m p l e t e to
that is likely t o recur but s h o u l d not b e c o n f u s e d
r e d u c e t h e r e c u r r e n c e risk. It is n o t uncommon
w i t h m a l i g n a n c y ( R o d r i g u e s et al 1998, A l - Q a t t a n
t h a t partial e x c i s i o n of t h e t e n d o n s h e a t h o r j o i n t
2001). T h e h i s t o p a t h o l o g i c a l differential d i a g n o s i s
c a p s u l e is r e q u i r e d t o e n s u r e c o m p l e t e r e m o v a l
includes g r a n u l o m a t o u s lesions, necrobiotic gran-
of t h e l e s i o n . J o i n t d e b r i d e m e n t a n d a r t h r o d e s i s
u l o m a s , x a n t h o m a , f i b r o m a of t h e t e n d o n s h e a t h ,
may b e n e c e s s a r y in s o m e c a s e s , particularly
synovial sarcoma, rhabdomyosarcoma, myxoid
when there is intra-articular invasion ( F i g . 5).
c y s t ( B a t t a et al 1999), b e n i g n f i b r o u s h i s t i o c y t o m a ,
O f t e n , if t h e m a s s is r e l a t i v e l y l a r g e , s e v e r a l s m a l l
and plexiform s c h w a n n o m a .
c o n t a g i o u s satellite l e s i o n s w i l l b e n o t e d e x t e n d -
ing into t h e t e n d o n s h e a t h a n d s y n o v i u m . It is
important that they be removed. In case of

Treatment i n t r a o s s e o u s e x t e n s i o n , e n u c l e a t i o n of t h e l e s i o n
w i t h c u r e t t a g e a n d irrigation of t h e r e s i d u a l c a v i t y
At operation the tumor is r e a d i l y r e c o g n i z e d : it m u s t b e p e r f o r m e d . If t h e r e is a l a r g e d e f e c t , a
is multicolored between yellow and brown, c a n c e l l o u s b o n e graft c a n b e r e q u i r e d (7 p e r c e n t
multinodular, s o m e t i m e s w i t h a thin s h e a t h , but of c a s e s in t h e s e r i e s of U r i b u r u a n d L e v y 1998).
always well delineated from the surrounding Amputation of the digit, even in recurrent
 GIANT CELL TUMOR O F T H E T E N D O N SHEATH 31

d e

Figure 3

(a) M R I : tumor occupying the total length of the synovial sheath of the flexor tendons. (b) Perioperative view. (c) Perioperative
view after removal of the tumor. (d) Histology of the tumor. (e) Hemosiderin deposits.
32 T U M O R S O F T H E HAND

a b

с d

e f
 GIANT C E L L T U M O R OF THE TENDON SHEATH 33

g n

Figure 4

(a) Preoperative palmar view. (b) Preoperative side view. (c) Peroperative after skin incision. (d) Peroperative palmar extension.
(e) Peroperative neurovascular pedicle preserved. (f) Excised specimen 'en bloc', (g) Postoperative at 1 year side view.
(h) Postoperative at 1 year palmar view.

d i s e a s e , is u s u a l l y not i n d i c a t e d ( R o d r i g u e s et al Table 5 Complications among our 72 cases

1998, U r i b u r u a n d L e v y 1998, L o o i et al 1999).
No. of cases Percentage
W h e n t h e lesion is c i r c u m f e r e n t i a l , a circular
t e c h n i q u e c a l l e d ' d e s d e n t s d e la m e r ' h a s b e e n 3 4.2
Scar dysesthesia
p r o p o s e d b y M a r c u c c i a n d F o u c h e r in 1991, L o r é a Hematoma 1 1.4
et al a n d R R o g e r o ( p e r s o n a l c o m m u n i c a t i o n , 1994) Proximal interphalangeal 1 1.4
to r e d u c e t h e cicatrization failures. In t h e m a j o r i t y joint stiffness
of c a s e s , t h e p o s t o p e r a t i v e c o u r s e is u n e v e n t f u l . In Lesion of the motor 1 1.4
o u r s e r i e s t r e a t m e n t w a s t e r m i n a t e d 2 w e e k s after branch of the ulnar
t h e o p e r a t i o n in 65 per c e n t of p a t i e n t s . T h e r e w e r e nerve in the palm
six c o m p l i c a t i o n s , of w h i c h t h r e e w e r e s c a r d y s e s -
thesia (Table 5). In o t h e r s e r i e s , m o s t c o m p l i c a t i o n s
c o n c e r n e d s c a r s (Table 6).
Table 6 Surgical complications in the series of 107 cases of
T h e r e c u r r e n c e r a t e of G C T T S is h i g h . It r a n g e s
Reilly et al (1999)
f r o m 5 to 55 p e r c e n t ( A l - Q a t t a n 2001) (Table 7). In
o u r s t u d y , s e v e n c a s e s (9.7 p e r c e n t ) r e c u r r e d . Percentage
A m o n g our recurrent c a s e s , the m e a n a g e w a s
h i g h e r t h a n in n o n - r e c u r r e n t c a s e s (55.3 v e r s u s Painful scar 11
52.6 y e a r s ) . T h e m e a n t u m o r v o l u m e w a s also Numbness 6
Stiffness 6
higher (3.7 m l v e r s u s 2.3 m l in non-recurrent
Infection 2
cases). W e noticed that in t w o - t h i r d s of these
Necrosis of a local rotation flap 1
c a s e s , t h e site w a s t h e distal i n t e r p h a l a n g e a l ( D I P )
joint. T h e mean duration of clinical evolution
b e f o r e t h e first o p e r a t i o n w a s c l e a r l y h i g h e r in
r e c u r r e n t c a s e s t h a n in n o n - r e c u r r e n t c a s e s (42.5 ten fields, a n d t w o of t h e s e c a s e s presented w i t h
v e r s u s 15.7 m o n t h s ) . a r e c u r r e n c e (33.3 p e r c e n t ) .
In six c a s e s , t h e p a t h o l o g i s t d e s c r i b e d s i m p l e T h e r e w e r e 64 t u m o r s t h a t w e r e r e m o v e d e n
p r e s e n c e o r p r e s e n c e of m o r e t h a n 11 m i t o s e s p e r b l o c , f o u r of w h i c h r e c u r r e d (6.25 p e r c e n t ) . N i n e
T U M O R S OF THE HAND

Figure 5

(a) Peroperative: large tumor beneath the extensor com-

munis tendons. (b) Peroperative after removal of the tumor.
(c) Excised specimen 'en bloc', (d) Carpometacarpal joint
fusion of the long fingers with an iliac bone graft. (e) X-ray
2 years after tumor excision, 1 year after plate removal.
 GIANT CELL TUMOR OF T H E T E N D O N SHEATH 35

Table 7 Recurrence rates reported in literature

No. of cases Recurrence rate (%)

Uriburu and Levy (1998) 15 (with intraosseous invasion) 13

Loréa et al (2001) 25 (circumferential tumors) 24
33 if bone invaded
57 if joint invaded
Byers et al (1968) 26 27
Rodriguez-Peralto et al (2001 ) 28 29
Al-Qattan (2001) 43 12
38 if not encapsuled
Rao and Vigorita (1984) 46 29
Grover et al (1998) 52 15
Looi et al (1999) 53 7
Reilly et al (1999) 107 26

t u m o r s w e r e r e s e c t e d Into t w o or m o r e f r a g m e n t s , (58 p e r c e n t r e c u r r e n c e v e r s u s 9 p e r c e n t in o t h e r
and three of t h e s e t u m o r s recurred (33.3 per sites) ( R e i l l y et al 1999).
c e n t ) . A l t h o u g h this is o b v i o u s l y not statistically
s i g n i f i c a n t , it s u g g e s t s t h a t it is s a f e r to r e m o v e
t h e l e s i o n e n b l o c . E l e c t r o c o a g u l a t i o n of t h e p r e -
s u m e d site o f o r i g i n w a s p e r f o r m e d b y s u r g e o n s Conclusions
in 20 c a s e s . N o r e c u r r e n c e s h a v e b e e n r e p o r t e d in
these cases. G i a n t cell t u m o r of t h e t e n d o n sheath, which
In t h e d i g i t s , often a c o l l a t e r a l n e r v e lies o n t h e s h o u l d not b e c o n f u s e d w i t h t h e m o r e a g g r e s s i v e
tumor. A c c o r d i n g to our series this c a n be safely g i a n t cell t u m o r of b o n e , i s , after t h e s y n o v i a l
p r e s e r v e d , a s n e u r o l y s i s w a s m e n t i o n e d in 29 of g a n g l i o n , t h e m o s t f r e q u e n t m a s s e n c o u n t e r e d in
t h e c a s e s a n d n o n e of t h e s e r e c u r r e d . In s o m e t h e soft t i s s u e of t h e h a n d . It c a n b e e n c o u n t e r e d
cases small branches w e r e removed because at a n y a g e , is m o r e f r e q u e n t a r o u n d t h e fifth,
their preservation w o u l d h a v e required separa- sixth, a n d s e v e n t h d e c a d e s , a n d m o r e prevalent
t i o n of t h e t u m o r . in w o m e n . T h i s b e n i g n t u m o r , w h i c h h a s s e v e r a l
T h e m e a n d e l a y of r e c u r r e n c e in o u r c a s e s w a s n a m e s ('tumeur a myéloplaxes', villonodular syn-
52.5 m o n t h s ( r a n g e 15-90 m o n t h s ) . T h e first o p e r - ovitis) is v e r y often easily diagnosed clinically
ation is t h e b e s t c h a n c e for cure. A m o n g our b e c a u s e of its s l o w g r o w t h a n d a b s e n c e of s y m p -
seven recurrences, two recurred several times t o m s of p a i n o r f u n c t i o n a l i m p a i r m e n t . It p r e s e n t s
g i v i n g a r e c u r r e n c e rate after a first r e c u r r e n c e of a s a h a r d m a s s , often m u l t i s p h e r i c non-adherent
28.6 p e r c e n t c o m p a r e d to 9.6 p e r c e n t after t h e to the s k i n . T h e clinical differential diagnosis
initial r e m o v a l . T h e f o l l o w i n g f e a t u r e s s e e m t o includes synovial ganglion and epidermoid cyst,
p r e s e n t a h i g h e r r e c u r r e n t risk ( A l - Q a t t a n 2 0 0 1 , a l t h o u g h t h e latter often h a s e v i d e n c e of relation-
L o r é a et al 2001): i n c o m p l e t e r e m o v a l , j o i n t p r o x - s h i p s w i t h t h e skin. Often n o i m a g i n g is p e r f o r m e d
imity, b o n e i n d e n t a t i o n on radiographs, multi- a n d nor is it n e c e s s a r y . M R I is helpful in t h e p r e s -
locular tumor, if tumor is not completely e n c e of a l a r g e l e s i o n o r in c a s e of r e c u r r e n c e t o
surrounded with a pseudocapsule (Al-Qattan d e t e r m i n e its e x t e n s i o n .
2001), h i g h c e l l u l a r i t y on microscopic examina- T r e a t m e n t c o n s i s t s of s u r g i c a l r e m o v a l . S k i n
t i o n ( B y e r s et al 1968, R a o et al 1984), e x p r e s s i o n i n c i s i o n is d e t e r m i n e d b y t h e localization of t h e
deficit of g e n e n m 23 ( G r o v e r et al 1998), mitotic tumor but must follow the principles of hand
activity o n m i c r o s c o p i c e x a m i n a t i o n ( B y e r s et al s u r g e r y . It is r e a d i l y recognized b y its yellow-
1968, R a o et al 1984), a n d l o c a t i o n at t h e D I P j o i n t b r o w n i s h c o l o r a n d its m u l t i l o b u l a t e d s h a p e . It is
36 T U M O R S OF THE HAND

p r e f e r a b l e to r e m o v e t h e lesion en bloc. N o b l e Froimson Al (1987) Benign solid tumors, Hand Clin

structures, including the neurovascular pedicle, 3:213-17.
c a n b e p r e s e r v e d w i t h o u t i n c r e a s i n g t h e risk of
recurrence. A joint should be opened only if Galliani t, Cassiani G , Valmori A , Falcieri E (2000)
e x t e n s i o n of t h e t u m o r , in c o n t i n u i t y , is o b v i o u s . Giant cell tumor of tendon sheath: a light and electron
microscopic study, J Submicrosc Cytol Pathol 32:69-76.
If t u m o r is p r e s e n t in t h e j o i n t , a n arthrodesis
s h o u l d b e c o n s i d e r e d e s p e c i a l l y for t h e D I P a n d
Gibbons C L M H , Khwaja HA, Cole A S et al (2002) Giant-
c a r p a l j o i n t s . E l e c t r o c o a g u l a t i o n of t h e p r e s u m e d
cell tumour of the tendon sheath in the foot and ankle,
site of o r i g i n s e e m s to b e efficient in p r e v e n t i n g
J Bone Joint Surg B r 84:1000-3.
recurrence.

Glowacki KA, Weiss A P C (1995) Giant cell tumors of

tendon sheath, Hand Clin 11:245-53.

Grover R, Grobbelaar A O , Richman P I , Smith P J (1998)

References Measurement of invasive potential provides an accurate
prognostic marker for giant cell tumour of tendon
Abdul-Karim F W , El-Naggar A K , J o y c e M J et al (1992) sheath, J Hand Surg 23B:728-31.
Diffuse and localized tenosynovial giant cell tumor and
pigmented villonodular synovitis: a clinicopathologic Hansen P, Nielsen PT, Wahlin A B (1988) Pigmented
and flow cytometric D N A analysis, Hum Pathol villonodular synovitis of the extensor tendon sheaths in
23:729-35. a child, J Hand Surg 13B:313-14.

Al-Qattan M M (2001) Giant cell tumours of tendon Kruithof E, Baeten D, Dierckxsens P et al (2002)
sheath: classification and recurrence rate, J Hand Surg Tenosynovitis with acute onset: unexpected giant cell
Br 26:72-5. tumour of the tendon sheath, Ann Rheum Dis 61:847.

Batta K,Tan CY, Colloby P (1999) Giant cell tumour of the Looi KP, Low CK, Yap Y M (1999) Pigmented villonodular
tendon sheath producing a groove deformity of the nail synovitis of the hand in the Asian population, Hand
plate and mimicking a myxoid cyst, Br J Dermatol Surg 4:81-5.
140:780-1.
Loréa P, Medina J , Navarro R, Foucher G (2001) Récidive
Bueno Horcajadas A , Lopez Lafuente J , de la Cruz des tumeurs à cellules géantes des gaines tendineuses
Burgos R et al (2002) Ultrasound and MR findings in digitales après exérèse par une voie d'abord dite des
tumor and tumor-like lesions of the fingers, Eur Radiol 'dents de la mer'. A propos de 25 cas, Ann Chir Plast
Epub J U N 1S. www.springeronline.com. Esthét 46:607-10.

Byers PD, Cotton R E , Deacon O W et al (1968) The Marcucci L, Foucher G (1991) Les 'dents de la mer' dans
diagnosis and treatment of pigmented villonodular l'exérèse des tumeurs circumdigitales dites 'à cellules
synovitis, J Bone Joint Surg 50B:290-305. géantes' des gaines tendineuses, Ann Chir Plast Esthét
36:442-6.
Castens P H B , Howell R S (1979) Malignant giant
cell tumor of tendon sheath, Virchows Arch 382: Neilson A L , KlanT (1989) Malignant giant cell tumour of
237-43. synovium and locally destructive pigmented villonodular
synovitis: ultrastructural and immunohistochemical
De Saint Aubain Somerhausen N, Fletcher C D M (2000) study and review of the literature, Hum Pathol 20:
Diffuse-type giant cell tumor, Am J Surg Pathol 765-71.
24:479-92.
Peh W C G , W o n g Y, S h e k T W H , Ip W Y (2001) Giant cell
Flandy F, Hughston J C (1987) Pigmented villonodular tumour of the tendon sheath of the hand: a pictorial
synovitis, J Bone Joint Surg 69A:942-9. essay, Aust Radiol 45:274-80.

Folpe AL, Morris R J , Weiss S W (1999) Soft tissue giant Rao A S , Vigorita V J (1984) Pigmented villonodular
cell tumor of low malignant potential: a proposal for the synovitis (giant cell tumour of the tendon sheath and
reclassification of malignant giant cell tumor of soft synovial membrane): a review of eighty-one cases,
parts, Mod Pathol 12:894-902. J Bone Joint Surg 66A:76-94.
 GIANT CELL TUMOR OF T H E T E N D O N SHEATH 37

Reilly KE, Stern P J , Dale J A (1999) Recurrent giant cell similar to bone giant cell tumor: a case report and
tumors of the tendon sheath, J Hand Surg 24A:1298-302. literature review, Pathol Int 51:60-3.

Rodrigues C, Desai S , Chinoy R (1998) Giant cell tumour Uriburu IJF, Levy VD (1998) Intraosseous growth of giant
of the tendon sheath: a retrospective study of 28 cases, cell tumors of the tendon sheath (localized nodular
J Surg Oncol 68:100-3. tenosynovitis) of the digits: report of 15 cases,
J Hand Surg 23A:732-36.
Rodriguez-Peralto J L , Lopez-Barea F, Fernandez-
Delgado J (2001) Primary giant cell tumor of soft tissues
5
Hand chondromas
Caroline Leclercq

Introduction Table 1 Ray involvement in the series of chondromas

[n = 70) at l'Institut de la Main (2003)

C h o n d r o m a s , a l s o r e f e r r e d to a s ' e n c h o n d r o m a s ' ,
Finger n
a r e t h e m o s t f r e q u e n t b o n e t u m o r s in t h e h a n d ,
a c c o u n t i n g for 50-60 per c e n t ( C a m p b e l l et al Thumb 7
1995). T h e y r e p r e s e n t 70 of t h e 120 b o n e t u m o r s Index 12
in o u r s e r i e s at l'Institut d e la M a i n (Institut d e la Middle 10
M a i n 2003). T h e h a n d is a l s o t h e m o s t frequent Ring 16
site of c h o n d r o m a s (70 p e r c e n t in C a m p a n a c c i ' s Little 20
s e r i e s of 334 skeletal c h o n d r o m a s ( C a m p a n a c c i
1990)). T h e l a r g e s t s e r i e s of h a n d chondromas
w a s published b y T a k i g a w a in 1971 (110 c a s e s )
( T a k i g a w a 1971). M o s t c h o n d r o m a s a r e solitary, c h o n d r o m a s of t h e f o r e a r m b o n e s ( t w o r a d i u s ,
b u t o c c a s i o n a l l y t h e r e c a n b e s e v e r a l localizations t w o u l n a ) , a n d 16 of t h e h u m e r u s .
o n the s a m e finger ray, or o n t h e s a m e h a n d Chondromas may be revealed by several
(multiple chondromas), or even on the same features. The most frequent c i r c u m s t a n c e is a
hemi-skeleton (Ollier's disease). p a t h o l o g i c f r a c t u r e of t h e i n v o l v e d b o n e ( N o b l e
a n d L a m b (1974): 7 5 p e r c e n t of c a s e s ; J e w u s i a k
et al (1971): 59 per cent), following a minor
t r a u m a ( F i g . 1). A s w e l l i n g m a y a l s o r e v e a l t h e
Clinical presentation t u m o r , a n d a l t h o u g h it is m o s t often p r e s e n t at first
e x a m i n a t i o n , it is not a l w a y s a r e a s o n for c o n s u l -
C h o n d r o m a s affected 56 w o m e n (70 p e r c e n t of t a t i o n . T h e s w e l l i n g is of a h a r d , b o n y c o n s i s t e n c y ,
c a s e s ) a n d 14 m e n in o u r s e r i e s (Institut d e la M a i n p a l p a t e d a s a r e g u l a r i n c r e a s e in t h e size of t h e
2003), a l t h o u g h this is not a classical finding b o n e w i t h ill-defined b o u n d a r i e s . In distal l o c a l -
( G l i c e n s t e i n et al 1988). B e s i d e s Ollier's d i s e a s e , izations, it m a y a l s o e n l a r g e t h e f i n g e r n a i l ( F i g . 2).
w h i c h is u s u a l l y d e t e c t e d d u r i n g e a r l y c h i l d h o o d , It is o f t e n , b u t not a l w a y s , t e n d e r u n d e r p r e s s u r e .
c h o n d r o m a s c a n o c c u r at all a g e s (11-77 y e a r s in P a i n less f r e q u e n t l y r e v e a l s a c h o n d r o m a . W h e n
o u r s e r i e s ) w i t h a p e a k at a r o u n d 35 y e a r s . T h e y p r e s e n t , it is often a s s o c i a t e d w i t h a pathologic
affect b o t h h a n d s e q u a l l y , a n d p r e d o m i n a t e o n t h e fracture. T h e r e h a v e been a few reports of a
u l n a r f i n g e r s ( s e e T a b l e 1) ( G l i c e n s t e i n et al 1988). c h o n d r o m a of t h e distal p h a l a n x r e v e a l e d b y a
Chondromas of t h e h a n d affect mostly the r u p t u r e of t h e distal insertion of t h e flexor p r o -
proximal phalanx, then equally the metacarpals, f o n d u s t e n d o n f r o m t h e p h a l a n x ( O g u n r o 1983,
a n d t h e m i d d l e p h a l a n x . T h e distal p h a l a n x is less Vaz a n d B e l c h e r 1998). In a n u m b e r of c a s e s , t h e
frequently involved, and the carpus very rarely t u m o r is a s y m p t o m a t i c a n d t h e d i a g n o s i s is m a d e
(Emecheta et a l 1997). In C a m p a n a c c i ' s s e r i e s o n X - r a y s p e r f o r m e d for o t h e r r e a s o n s ( 4 0 - 2 0 p e r
t h e r e w e r e 233 c h o n d r o m a s of t h e h a n d , f o u r c e n t of c a s e s ) .
40 T U M O R S OF THE HAND

Figure 1

Pathologic fracture of a chondroma of the proximal phalanx.

Radiologic features
T h e t y p i c a l a s p e c t o f a c h o n d r o m a is a c e n t r a l ,
round, well-defined osteolytic a r e a ( F i g . 3 ) . In
early cases the bony contour is intact, but in
a d v a n c e d c a s e s , it m a y a p p e a r to b e e x p a n d e d or
'blown out', with progressive thinning of the
c o r t e x a d j a c e n t to t h e t u m o r . H o w e v e r , t h e r e is n o
cortex r u p t u r e e x c e p t in t h e c a s e of a p a t h o l o g i c
fracture.
T h e c h o n d r o m a is u s u a l l y initially l o c a t e d in
t h e m e t a p h y s i s of b o n e , it t h e n e x p a n d s both b
transversally a n d longitudinally t o w a r d s the dia-
Figure 2
p h y s i s . A s it e x p a n d s it m a y a l s o a s s u m e a p o l y -
cyclic s h a p e (Fig. 4). Fine calcifications may Chondroma of the distal phalanx (middle finger) responsible
p u n c t u a t e t h e t u m o r ( F i g . 5 ) , a n d in o l d l e s i o n s , a for an enlargement of the fingernail (a) clinical aspect
f i n e c a l c i f i e d line m a y s u r r o u n d it. (b) radiographic picture.
 HAND CHONDROMAS 41

Figure 3 Figure 4

Typical radiographic aspect of a central chondroma. Polycyclic aspect of a phalangeal chondroma in a teenager.

In s o m e c a s e s t h e c h o n d r o m a is not c e n t r a l l y
l o c a t e d . T a k i g a w a (1971) h a s c l a s s i f i e d t h e differ-
ent s i t e s into c e n t r a l (58 p e r c e n t ) , e c c e n t r i c (19
p e r c e n t ) , c o m b i n e d ( s e v e r a l l e s i o n s in t h e s a m e
b o n e , 21 p e r c e n t ) , p o l y c e n t r i c (11 p e r c e n t ) , a n d
g i a n t (3 p e r c e n t ) , to w h i c h t h e particular form:
periosteal chondroma must be added
(Lichtenstein and Hall 1952). In t h e case of
periosteal chondroma the tumor develops
b e t w e e n the b o n e cortex and the periosteum.
X-ray imaging shows a radiolucent tumor
a t t a c h e d t o t h e c o r t e x , a n d d e v e l o p i n g into t h e
soft t i s s u e s . It m a y b e b o u n d e d b y a f i n e c a l c i f i e d
l i n e, a n d d o t t e d w i t h m i c r o c a l c i f i c a t i o n s ( F i g . 6).
T h e cortex is e r o d e d at t h e site of a t t a c h m e n t .

Multiple chondroma
Figure 5
M u l t i p l e c h o n d r o m a s m a y d e v e l o p in t h e s a m e
A metacarpal chondroma punctuated with calcifications. b o n e , o n t h e s a m e ray, or o n t h e s a m e h a n d .
42 T U M O R S OF THE HAND

Figure 6 Figure 7

Periosteal chondroma in a 15-year-old boy. Severe involvement of the hand in a multiple enchondro-
matosis of Oilier.

T h e y u s u a l l y o c c u r at a y o u n g e r a g e t h a n s o l i t a r y
c h o n d r o m a s . In r a r e c a s e s t h e y i n v o l v e s e v e r a l
Diagnosis
s i t e s o n o n e half of t h e s k e l e t o n , a condition
k n o w n a s m u l t i p l e e n c h o n d r o m a t o s i s of Oilier. T h e clinical picture of c h o n d r o m a is no t s p e c i f i c .
O n l y b o n e s of e n c h o n d r a l o r i g i n a r e i n v o l v e d , Even though the radiographic imaging should
a n d t h e d i s e a s e m a y be limited to a f e w foci, primarily be directed t o w a r d s c h o n d r o m a because
o r affect t h e e n t i r e half of t h e b o d y . I n v o l v e m e n t o f its h i g h e r f r e q u e n c y , t h e p r e s e n c e o f other
of t h e b o n e s of t h e h a n d is v e r y f r e q u e n t ( F i g . 7 ) , benign radiolucent b o n e t u m o r s should b e c o n -
a n d c a u s e s functional i m p a i r m e nt related to the sidered, especially chondroblastoma, giant cell
subsequent deformities, limitation of joint t u m o r of b o n e , a n d a n e u r y s m a l c y s t , all of w h i c h
motion , a n d recurring pathologic fractures. are extremely rare, a s well a s other radiolucent
 HAND C H O N D R O M A S 43

Histology
T h e m a c r o s c o p i c a s p e c t of c h o n d r o m a is t h a t of a
h y a l i n e c a r t i l a g e , w h i t i s h o r p e a r l y , a n d soft. T h e
h i s t o l o g i c a s p e c t is t h a t o f r e g u l a r cartilaginous
lobules, separated by poorly vascularized fibrous
s e p t a e . T h e c h o n d r o c y t e s a r e n o r m a l in number
and appearance, and display benign characteris-
t i c s : s m a l l n u c l e i of p o o r c h r o m a t i c d e n s i t y w i t h
f e w mitoses. However, a few binucleated cells
may occasionally be o b s e r v e d , as well a s s o m e
d e g r e e of h y p e r c e l l u l a r i t y . T h e c e l l s a r e s c a t t e r e d
in a h y a l i n e or m i x o i d s t r o m a . C a l c i f i c a t i o n s p r o -
d u c e a g r a n u l a r a s p e c t , or e v e n o p a q u e s t r e a k s .
T h i s a s p e c t is not a l w a y s e a s y t o d i s t i n g u i s h f r o m
t h a t of c h o n d r o s a r c o m a , w h i c h is a s l o w l y g r o w -
ing t u m o r , r u p t u r i n g t h e c o r t e x only at a late
stage, with f e w mitoses and limited hypercellu-
larity at h i s t o l o g y .

Etiological aspects
S o m e authors v i e w c h o n d r o m a s a s originating
from a misgrowth of e p i p h y s e a l c a r t i l a g e c e l l s
(Milgram 1982), others as accessory articular
cartilages, and others a s a metaplasia f r o m other
c o n n e c t i v e t i s s u e s ( N o b l e a n d L a m b 1974).
A recent study b y Tallini et al (2002) has
r e p o r t e d f r e q u e n t c h r o m o s o m a l a b n o r m a l i t i e s in
Figure 8
c a r t i l a g i n o u s t u m o r s , w i t h a different a n o m a l y for
Chondroma with large expansion of the cortex, but without e a c h t y p e of t u m o r . T h i s w a s v e r i f i e d in 11 o u t of
rupture or invasion into the soft tissue. t h e 29 c h o n d r o m a s t h e y s t u d i e d . T h e s e findings
a r e v e r y p r o m i s i n g for f u t u r e d i s t i n c t i o n b e t w e e n
chondromas and chondrosarcomas.
images such as those produced by epidermoid
c y s t s , a n d in t h e w r i s t i n t r a o s s e o u s g a n g l i a .
Chondrosarcoma may also be considered
if t h e l e s i o n is p a i n f u l , o r it h a s r u p t u r e d the Spontaneous course
b o n e cortex a n d e x p a n d e d into t h e soft t i s s u e s
(Figs. 8 and 9). A s h a s b e e n reported in the T h e s p o n t a n e o u s c o u r s e of c h o n d r o m a is s l o w .
literature ( B a u e r et al 1995, C a w t e et al 1998), T h e r e h a v e b e e n c a s e s of stabilization, a n d e v e n
t h e differentiation is not e a s y , neither clinically n o r i n v o l u t i o n of t h e l e s i o n .
radiographically, nor e v e n at histology, b e c a u s e T h e p o s s i b i l i t y of a m a l i g n a n t chondrosarco-
t h e r e m a y b e b e n i g n foci a m i d s t m a l i g n a n t o n e s in m a t o u s c h a n g e is still d e b a t e d . C l a s s i c a l l y t h e
chondrosarcoma ( C u l v e r et al 1975), a n d also clinical aspect undergoes obvious changes,
because there are many similarities in the w i t h the a p p e a r a n c e of p a i n , a n d rapid i n c r e a s e
histologic a s p e c t s of t h e t w o t u m o r s ( s e e b e l o w ) . in size. B u t a s t h e t w o t u m o r s a r e difficult to
44 T U M O R S OF THE HAND

Treatment
Curettage is t h e usual treatment for solitary
chondroma. Most authors recommend packing
the residual cavity with either cancellous b o n e ,
allogenic b o n e ( T a n i g u c h i e t a l 1999), c e m e n t
(Bickels et al 2002), or b o n e substitutes (Inoue
et a l 1993, J o o s t e n e t a l 2 0 0 0 ) , b u t o t h e r s d o n o t
f i n d it n e c e s s a r y t o fill t h e d e f e c t ( T o r d a i e t a l
1990). S o m e a u t h o r s , h o w e v e r , d o n o t a d v i s e a n y
surgery, a n d d o no more than a cast immobiliza-
t i o n in c a s e o f a p a t h o l o g i c f r a c t u r e ( N o b l e a n d
L a m b 1974, H a s s e l g r e n 1991). N o b l e a n d L a m b
c o m p a r e d t h e results of abstaining (11 c a s e s ) ,
c u r e t t a g e a l o n e (11 c a s e s ) , a n d c u r e t t a g e with
cancellous graft (18 c a s e s ) . T h e y found the
r e s u l t s t o b e e x c e l l e n t in a l l u n o p e r a t e d cases,
m i n o r r e s i d u a l s y m p t o m s in 4/11 c u r e t t a g e s , a n d
4/18 l i m i t a t i o n s o f m o t i o n , a n d o n e n o n - u n i o n
( f r a c t u r e d c h o n d r o m a ) in c a s e s w i t h c a n c e l l o u s
g r a f t i n g . N e v e r t h e l e s s , w e f i n d it s a f e r t o r e m o v e
chondromas in o r d e r to a v o i d e x p a n s i o n a n d
recurrent fractures.

Operative technique
T h e o p e r a t i o n is p e r f o r m e d u n d e r axillary block,
and a pneumatic tourniquet is a p p l i e d . It c a n
Figure 9 usually b e performed a s a n outpatient procedure,
unless a n iliac bone graft is d e c i d e d upon.
Chondrosarcoma with cortex ruptured and expansion into
In c h o n d r o m a s o f t h e f i n g e r s , t h e a p p r o a c h is
the soft tissues. In this case, a histologic diagnosis of chon-
usually lateral, near the apex of t h e t u m o r .
droma was made after the initial curettage, and the correct
diagnosis was made after recurrence. In metacarpal locations, the approach is
postero-medial o r lateral, o n either side of t h e
e x t e n s o r a p p a r a t u s . W h e n t h e c o r t e x is t h i n n e d , it
c a n u s u a l l y b e e n t e r e d w i t h a knife, a n d a s m a l l
window is p e r f o r m e d . T h e n t h e c h o n d r o m a is
a c c u r a t e l y r e m o v e d w i t h a c u r e t t e (a r o n g e u r is
differentiate, a n early s t a g e of c h o n d r o s a r c o m a not helpful t h e r e , a s t h e c h o n d r o m a t o u s tissue
m a y b e mistaken for c h o n d r o m a , a n d t h e correct is v e r y soft). T h o r o u g h c u r e t t a g e is t h e k e y p o i n t
d i a g n o s i s is u s u a l l y p e r f o r m e d a t a later s t a g e a s f a r a s r e c u r r e n c e is c o n c e r n e d , a s t h e c h o n -
(recurrence) ( F i g . 9 ) ( B o n n e v i a l l e e t a l 1988, droma creates small polycyclic cavities inside t h e
G a u l k e 1997). In t h e l a r g e s t s e r i e s o f c h o n d r o s a r - b o n e , a n d each of t h e s e m u s t b e curetted. O n e
c o m a s of t h e h a n d that have been published should n o t h e s i t a t e t o i n c r e a s e t h e size o f t h e
( D a h l i n 1973, 3 3 1 c a s e s ; O g o s e 1997, 111 c a s e s ) , cortical w i n d o w w h e n n e e d e d .
some of w h i c h were derived from a benign We routinely fill t h e d e f e c t w i t h cancellous
tumor, usually with multiple locations (chondro- b o n e harvested f r o m t h e radial styloid. T h i s c a n
matosis, osteochondromatosis) but never f r o m a b e a v o i d e d in s m a l l c h o n d r o m a s , b u t l e a d s t o a
solitary c h o n d r o m a . much quicker recovery in l a r g e or fractured
 HAND CHONDROMAS 45

c h o n d r o m a s . A m o n g t h o s e w h o d o not routinely c h i l d h o o d . T h e m o s t f r e q u e n t s i g n is a p a t h o l o g i c
fill t h e r e s i d u a l d e f e c t , G o t o e t a l (2002) r e p l a c e s fracture. A swelling m a y also reveal the tumor.
the cortical window, which provides quicker T h e t y p i c a l r a d i o l o g i c a s p e c t is a c e n t r a l , r o u n d ,
restoration of t h e continuity of t h e cortex (3 m o n t h s well-defined osteolytic area located in t h e
versus 8 months). metaphysis.
G i l e s e t a l (1999) a d v o c a t e a d j u n c t i v e t r e a t m e n t T h e correct d i a g n o s i s of c h o n d r o m a is g e n e r a l l y
w i t h C 0 laser in l a r g e c h o n d r o m a s b e f o r e filling
2 m a d e b e c a u s e of its h i g h f r e q u e n c y r a t h e r t h a n
the gap with cancellous bone.The s a m e treatment its clinical or radiologic specificity. Although
a p p l i e s in f r a c t u r e d c h o n d r o m a s . It is n o t u s u a l l y m u c h less f r e q u e n t , c h o n d r o b l a s t o m a , g i a n t cell
necessary to perform a n y b o n e fixation, a s t h e s e b o n e t u m o r , a n e u r y s m a l cyst, a n d c h o n d r o s a r -
l e s i o n s a r e u s u a l l y s t a b l e ( B o n n e v i a l l e e t al 1988). coma must not be forgotten.
Tordai e t a l (1990) w a i t until t h e f r a c t u r e h a s T h e m a c r o s c o p i c a s p e c t of c h o n d r o m a is t h a t
healed before removing t h e chondroma. W e d o of a h y a l i n e c a r t i l a g e , w h i t i s h o r p e a r l y a n d soft.
not b e l i e v e t h i s is n e c e s s a r y , d e s p i t e t h e s e r i e s of H i s t o l o g i c a l l y it c o n s i s t s of n o r m a l c h o n d r o c y t e s
A b l o v e e t a l (2000) o f 16 f r a c t u r e d chondromas which form regular cartilaginous lobules sepa-
indicating a higher i n c i d e n c e of complications rated b y p o o r l y v a s c u l a r i z e d f i b r o u s s e p t a e .
after i m m e d i a t e s u r g i c a l t r e a t m e n t . Differences of o p i n i o n often i m p e d e t h e treat-
In m u l t i p l e c h o n d r o m a t o s i s o f Oilier, s u r g e r y is ment. S o m e advise waiting for t h e fracture to
indicated only in t h o s e c h o n d r o m a s impairing heal, which a l w a y s occurs, before undertaking
function, or w h e n a modification of o n e l e s i o n surgical treatment. Our experience h a s s h o w n
( p a i n , i n c r e a s e in size) i s s u g g e s t i v e of a m a l i g - that surgery c a n be performed without delay.
nant c h a n g e . A biopsy is then performed at t h e Furthermore, there is s o m e d e b a t e a s to w h e t h e r
e d g e of the tumor, a n d histological findings must s u r g e r y is n e c e s s a r y . A l t h o u g h s p o n t a n e o u s h e a l -
b e i n t e r p r e t e d w i t h c a r e , a s s t a t e d earlier. ing h a s b e e n r e p o r t e d , w e r e c o m m e n d c u r e t t a g e
o f t h e l e s i o n b e c a u s e this is t h e o n l y m e t h o d t o
ascertain t h e diagnosis. W h e t h e r the b o n e should
b e grafted after t h e r e m o v a l o f t h e t u m o r r e m a i n s
Recurrence to b e d e t e r m i n e d . G r a f t i n g is a t least indicated
w h e n , after c u r e t t a g e , a f r a c t u r e is t o b e f e a r e d .
Recurrence h a s been reported in 5 p e r c e n t of T h e r e c u r r e n c e rate is a b o u t 5 p e r c e n t . It is
c a s e s b y T a k i g a w a (1971 ) . T h i s is g e n e r a l l y d u e t o g e n e r a l l y d u e t o i n c o m p l e t e r e m o v a l of t h e t u m o r .
i n c o m p l e t e r e m o v a l o f t h e t u m o r . It i s e v i d e n c e d A recurring lesion should a l w a y s b e r e m o v e d ,
b y t h e r e a p p e a r a n c e o f a lytic i m a g e o n X - r a y bearing in m i n d a possible chondrosarcoma
after s o m e m o n t h s . T h e r a d i o l o g i c a s p e c t m a y b e m a l i g n a n c y of w h i c h w o u l d h a v e b e e n m i s s e d at
difficult t o a n a l y z e w h e n b o n e s u b s t i t u t e s have first h i s t o l o g i c examination.
b e e n u s e d t o fill t h e d e f e c t . M o n t e r o e t a l (2002)
a d v o c a t e a long period of follow-up, a s o n e of
their patients h a d a r e c u r r e n c e after 6 years.
Recurring lesion should always be removed, References
b e a r i n g in m i n d a p o s s i b l e c h o n d r o s a r c o m a t h e
Ablove R H , M o y O J , Peimer CA, Wheeler DR (2000)
m a l i g n a n c y of w h i c h could h a v e b e e n m i s s e d at
Early versus delayed treatment of enchondroma, Am J
first h i s t o l o g i c examination.
Orthop 29:771-2.

Bauer H, Brosjo O, Kreicbergs A , Lindholm J (1995) Low

risk of recurrence of enchondroma and low-grade chon-
Conclusions drosarcoma in extremities. 80 patients followed for
2-25 years, Acta Orthop Scand 66:755-60.
Chondromas or enchondromas are the most
f r e q u e n t b o n e t u m o r in t h e h a n d . T h e y o c c u r a t Bickels J , Wittig J C , Kollender Y et al (2002)
any a g e but mostly around 35 y e a r s . T h e y usually Enchondromas of the hand: treatment with curettage
consist of a single lesion. Multiple c h o n d r o m a s or and cemented internal fixation, J Hand Surg
O l l i e r ' s d i s e a s e a r e r a r e a n d d e t e c t e d in e a r l y 27A:870-5.
46 T U M O R S OF THE HAND

Bonnevialle P, Mansat M , Durroux R et al (1988) Les Joosten U, Joist A , FrebelT et al (2000)The use of in situ
chondromes de la main: étude d'une série de trente cinq curing hydroxyapatite cement as an alternative to bone
cas, Ann Chir Main 7:32-44 (in French and English). graft following removal of enchondroma of the hand,
J Hand Surg 25B:288-91.
Campanacci M (1990) Bone and Soft Tissue Tumors,
Springer Verlag: New York. Lichtenstein L, Hall J E (1952) Periosteal chondroma,
J Bone Joint Surg 34A:691-7.
Campbell DA, Millner PA, Dreghorn CR (1995) Primary
bone tumours of the hand and wrist, J Hand Surg Milgram J W (1982) The origins of osteochondromas
20B:5-7. a n d enchondromas: a histopathologic study, Clin
Orthop 174:264-84.
Cawte T Steiner G C , Beltran J , Dorfman HD (1998)
Chondrosarcoma of the short tubular bones of the Montero L M , Ikuta Y , Ishida O et al (2002) Enchondroma
hands and feet, Skeletal Radiol 27:625-32. in the hand: retrospective study - recurrence cases,
Hand Surg 7:7-10.
Culver J E , S w e e t DE, McCue FC (1975) Chondrosarcoma
of the hand arising from a pre-existent benign solitary Noble J , Lamb D W (1974) Enchondromata of bones of
enchondroma, Clin Orthop 113:128-31. the hand. A review of 40 cases, Hand 6:275-84.

Dahlin DC (1973) Bone Tumors, 2nd edn. Charles C.

O g o s e A , Unni K, S w e e R G e t a l (1997) Chondrosarcoma
Thomas: Springfield. of small bones of the hands and feet, Cancer 80:50-9.

Emecheta IE, Bernhards J , Berger A (1997) Carpal

Ogunro O (1983) Avulsion of flexor profundus second-
enchondroma, J Hand Surg 22B:817-19.
ary to enchondroma of the distal phalanx, J Hand Surg
8:315-16.
Giles DW, Miller S J , Rayan G M (1999) Adjunctive treat-
ment of enchondromas with CO laser, Lasers Surg
Takigawa K (1971) Chondroma of the bones of the hand.
2

M e d 24:187-93.
A review of 110 cases, J Bone Joint Surg 53A: 1591-600.

Glicenstein J , Ohana J , Leclercq C (1988) Hand tumours.

Tallini G , Dorfman H, Brys P et al (2002) Correlation
Springer Verlag: Paris: 243.
between clinicopathological features and karyotype
in 100 cartilaginous and chordoid tumors. A report
Goto T, Yokokura S , Kawano H et al (2002) Simple
from the Chromosomes and Morphology ( C H A M P )
curettage without bone grafting for enchondromata of
Collaborative Study Group, J Pathol 196:194-203.
the hand with special reference to replacement of the
cortical window, J Hand Surg 27B:446-51.
Taniguchi Y,TamakiT, Oura H et al (1999) Sintered bone
implantation for the treatment of benign bone tumours
Hasselgren G , Forssblad P,Tornvall A (1991) Bone graft- in the hand, J Hand Surg 24B:109-12.
ing unnecessary in the treatment of enchondromas in
the hand, J Hand Surg 16:139-42.
Tordai P, Hoglund M , Lugnegard H (1990) Is the treat-
ment of enchondroma in the hand by simple curettage
Inoue O, Ibaraki K, Shimabukuro H, Shingaki Y (1993) a rewarding method? J Hand Surg 15B:331-4.
Packing with high-porosity hydroxyapatite cubes alone
for the treatment of simple bone cyst, Clin Orthop Vaz F M , Belcher H J C R (1998) Rupture of the tendon of
293:287-92. flexor digitorum profundus in association with an
enchondroma of the terminal phalanx, J Hand Surg
Institut de la Main (2003) Série de 1952 tumeurs de la 23B:548-9.
main. S y m p o s i u m de l'Institut de la Main sur les
Tumeurs de la Main et du Membre Supérieur, 21-22
Mars, Paris.

Jewusiak E M , Spence KF, Sell K W (1971) Solitary benign

enchondroma of the long bones of the hand, J Bone
Joint Surg 53A:1587-90.
6
Glomus tumor of the hand
Nicolas Favarger

History derived from the

( R o h r i c h et al 1994).
pericytes of Zimmermann

In 1812, W o o d first d e c r i b e d t h e g l o m u s t u m o r a s
a distinct clinical entity: 'a painful s u b c u t a n e o u s
nodule'. T h e nodule's characteristics w e r e hyper-
sensitivity to t e m p e r a t u r e c h a n g e , intermittent
Etiology
s e v e r e p a i n , l o n g d u r a t i o n of s y m p t o m s , s m a l l T h e e x a c t c a u s e of t h e g l o m u s t u m o r is u n k n o w n .
size, a n d f i r m c o n s i s t e n c y . T h e t r e a t m e n t was Some b e l i e v e t h a t c o n g e n i t a l w e a k n e s s in the
s u r g i c a l r e s e c t i o n . In 1924, M a s s o n d e s c r i b e d t h e s t r u c t u r e p r e d i s p o s e s it to r e a c t i v e h y p e r t r o p h y
h i s t o l o g i c a l a p p e a r a n c e of t h e g l o m u s t u m o r a n d , s e c o n d a r y to t r a u m a . M o s t , h o w e v e r , b e l i e v e t h a t
w i t h B a r r e ( B a r r e 1922, B a r r e a n d M a s s o n 1924) it is a h a m a r t o m a b e c a u s e it c o n t a i n s all the
d e s c r i b e d its clinical f e a t u r e s , e m p h a s i z i n g the e l e m e n t s of the n o r m a l g l o m u s body, but s i m p l y
i m p o r t a n c e of e x c i s i o n a s t r e a t m e n t . T h e term in a h y p e r t r o p h i c s t a t e ( R o h r i c h et al 1994).
glomus tumor is a c c r e d i t e d t o P o p o f f (1934), w h o Hereditary g l o m u s t u m o r s h a v e b e e n reported
first d o c u m e n t e d t h i s entity to b e t h e result of a n d differ in t h a t t h e y a r e u s u a l l y m u l t i p l e a n d
hyperplasia of the normal neuromyoarterial may lack pain as a significant complaint
g l o m u s body. ( K o u s k o u k i s 1983).

The normal glomus Pathology

T h e n o r m a l g l o m u s b o d y is a n e u r o m y o a r t e r i a l
mechanoreceptor, a structure that regulates
Macroscopy
b l o o d f l o w a n d skin t e m p e r a t u r e b y b o t h o p e n i n g
T h e t u m o r t y p i c a l l y m e a s u r e s 2 - 3 m m in dia-
a n d c l o s i n g v a s c u l a r s h u n t s at t h e d e r m a l l e v e l .
m e t e r but t h e size c a n b e g r e a t e r t h a n 1.5 c m . It is
T h i s a r t e r i o v e n o u s s h u n t is c o n c e r n e d w i t h ther-
a w e l l - d e f i n e d f i r m o v a l or c i r c u l a r m a s s . Its c o l o r
moregulation ( U p t o n a n d C o o m b s 1995).The glo-
is b e t w e e n pink a n d r e d d i s h - p u r p l e ; h o w e v e r , in
m u s bodies are distributed throughout the body
s o m e c a s e s t h e r e m a y b e n o d i s c o l o r a t i o n . In t h e
b u t a r e m o s t n u m e r o u s in t h e d i g i t s , p a l m s , a n d
m a j o r i t y of c a s e s a s u r r o u n d i n g c a p s u l e is f o u n d .
s o l e s of t h e f e e t .
A n a t o m i c a l l y t h e g l o m u s b o d y is m a d e u p of
f i v e u n i t s : a n afferent a n d a n efferent v e s s e l , a
capillary network known as a Suquet-Hoyer Microscopy
shunt, the intraglomerular reticulum (containing
g l o m u s c e l l s , n e r v e s , a n d interstitial c e l l s ) , a n d a Morphologically, the glomus tumor comprises
c a p s u l e ( L e v e r 1983). G l o m u s c e l l s a r e d e t e r - endothelial-lined vascular spaces (Suquet-
m i n e d to b e s p e c i a l i z e d s m o o t h m u s c l e c e l l s Hoyer's canal) surrounded by epithelioid cells,
48 T U M O R S OF THE HAND

c a l l e d g l o m u s c e l l s , w h i c h a r e t h e m a i n cell t y p e .
T h e r e i s , h o w e v e r , a w i d e v a r i a t i o n in h i s t o l o g i c a l
appearance, with three main types: a vascular
f o r m , a myxoid f o r m , a n d a solid f o r m , depending
o n t h e p r e d o m i n a n t c e l l u l a r pattern w i t h i n e a c h
t u m o r . In a d d i t i o n , n o n - m e d u l l a t e d n e r v e f i b e r s
a r e f o u n d in c l o s e a s s o c i a t i o n w i t h t h e s e g l o m u s
c e l l s ( H e y s 1992).
I m m u n o h i s t o c h e m i c a l analysis s h o w s that the
g l o m u s cells a r e positive for antibodies to actin,
v i m e n t i n , a n d p r o t e i n S 1 0 0 , but n e g a t i v e for a n t i -
d e s m i n a n t i b o d y . T h e v a s c u l a r part of t h e t u m o r is
p o s i t i v e f o r a n t i f a c t o r VIII o r a n t i - U l e x europaeus
a n t i b o d i e s . T h e n e r v o u s f i l a m e n t s a r e p o s i t i v e for
the anti-protein S100 antibody. T h e s e immuno-
Figure 1
histochemical features are not consistent but
helpful in t h e d i a g n o s i s of m o r p h o l o g i c a l l y a t y p i - Subungual glomus tumor of the finger tip, after partial nail
c a l g l o m u s t u m o r s ( A r k w r i g h t et al 1996). resection and tumor excision, tumor on the right side.

m e t a c a r p o p h a l a n g e a l ( M P ) joint, a n d the t h e n a r
and hypothenar area.
Clinical features In t h e s u b u n g u a l l o c a l i z a t i o n ( F i g . 1), t h e g l o -
mus tumor is l o c a t e d w i t h i n a c o n f i n e d space
G l o m u s t u m o r s m a k e u p 1-5 p e r c e n t of t h e soft
b e t w e e n t h e nail b e d a n d distal p h a l a n x . It m a y ,
t u m o r s of t h e h a n d ( F l e e g l e r a n d Z e i n o w i c z 1990).
o n o c c a s i o n , b e l o c a t e d in t h e soft t i s s u e of t h e
p e r i o n y c h i u m . T h e o t h e r c l a s s i c localization in t h e
f i n g e r is in t h e soft t i s s u e w i t h i n t h e p u l p .
A f e w c a s e s of p r i m a r y i n t r a o s s e o u s g l o m u s
Age and sex distribution
tumor have been reported particularly in the
A l m o s t all a u t h o r s h a v e e m p h a s i z e d t h e p r e d o m i - distal p h a l a n x ( S i m m o n s 1992).
n a n c e of g l o m u s t u m o r s in m i d d l e - a g e d w o m e n
( V a n G e e r t r u y d e n et al 1996). Data c o n c e r n i n g t h e
a n a t o m i c distribution a r e m o r e contradictory. F o r Size and number of lesions
e x a m p l e , Carlstedt a n d L u g n e g a r d (1983) f o u n d a
subungual predominance, while G a n d o n et al Usually, the glomus tumor is a u n i q u e small
(1992) f o u n d a p r e d o m i n a n c e of lesions in t h e pulp. lesion (2-5 mm). Occasionally, glomus tumors
G l o m u s t u m o r s a r e r a r e in c h i l d h o o d a n d c o m - are multiple (adjacent digits or multiple finger
p r i s e n o m o r e t h a n 2 p e r c e n t of p e d i a t r i c v a s c u - j o i n t s ( G r a h a m a n d W o l f 1992, N a k a m u r a 1992,
lar m a s s e s ( U p t o n a n d C o o m b s 1995). Moor et al 1999), t h i s occurs more often in
c h i l d r e n . S y n c h r o n o u s g l o m u s t u m o r s in t h e p u l p
a n d t h e s u b u n g u a l r e g i o n of t h e s a m e d i g i t h a v e
a l s o b e e n d e s c r i b e d ( N o o r a n d M a s b a h 1997).
Anatomic site
A l t h o u g h t h e y h a v e b e e n d e s c r i b e d in a v a r i e t y of
localizations, including the s t o m a c h , small intes- Diagnosis
tine, v a g i n a , n o s e , and oral cavity, most g l o m u s
tumors are located in t h e extremities with a Clinical findings
predilection for the fingers and h a n d s . In the
f i n g e r s t h e y a r e l o c a t e d p a r t i c u l a r l y in t h e distal The classic clinical manifestations of glomus
p h a l a n x . T h e r e is n o d i f f e r e n c e b e t w e e n differen t t u m o r , p a r t i c u l a r l y of t h e f i n g e r tip a r e a t r i a d o f
digits. R a r e l o c a l i z a t i o n s in t h e h a n d i n c l u d e t h e s y m p t o m s : p a r o x y s m a l pain, cold sensitivity, a n d
 G L O M U S T U M O R O F T H E HAND 49

exquisite point tenderness about the tumor r e p o r t e d t h a t localization of t h e t e n d e r n e s s t o a n

( C a r r o l l a n d B e r m a n 1972). P a i n is u s u a l l y t h e first a r e a t h e size of a p i n h e a d w a s s u g g e s t i v e of
s y m p t o m . It is a s p o n t a n e o u s p a i n , s o m e t i m e s g l o m u s t u m o r . J o s e p h a n d P o s n e r (1983) d e m o n -
radiating to the forearm or shoulder, with s t r a t e d t h a t t h e c o l d p r o v o c a t i o n of p a i n , u s i n g
m o m e n t s of p a r o x y s m i c e x c r u c i a t i n g p a i n . Direct a p p l i c a t i o n of e t h y l a l c o h o l , w a s s u g g e s t i v e of a
contact with the tumor area also produces a n g l o m u s t u m o r . E k i n et al (1997) u s e d t h e t r a n s i l l u -
excruciating pain. Although hypersensitivity to m i n a t i o n of t h e digit to try to v i s u a l i z e a r e d d i s h
any temperature change can be present, the most m a s s s u g g e s t i v e of a glomus tumor. Hildreth
f r e q u e n t c o m p l a i n t is c o l d i n t o l e r a n c e . In s o m e (1970) d e s c r i b e d , in o n e c a s e , a n i s c h e m i a t e s t
c a s e s , w a r m i n g m a y offer s o m e relief. a n d s u g g e s t e d t h a t t h e m o d u l a t i o n of p a i n a n d
T h e s e s y m p t o m s m a y b e p r e s e n t for up to tenderness by ischemia was indicative of a
50 y e a r s a n d t h e m e a n d u r a t i o n is a b o u t 7 y e a r s . glomus tumor. Giele (2002) in a study of 24
V a n G e e r t r u y d e n et al (1996), in a s e r i e s of 51 patients d e m o n s t r a t e d that t h e Hildreth's test h a d a
glomus tumors of t h e h a n d , r e p o r t e d t h a t the sensitivity of 92 per c e n t a n d a specificity of 91 per
d u r a t i o n of s y m p t o m s b e f o r e t r e a t m e n t a v e r a g e d cent. In this study, t h e L o v e ' s sign w a s 100 per c e n t
10 y e a r s ( r a n g e 1-40 y e a r s ) . Of interest is t h e f a c t s e n s i t i v e but 0 per c e n t specific. W i t h t h e s a m e
t h a t , p a t i e n t s c o n s u l t e d a n a v e r a g e of 2.5 p h y s i - patients, the transillumination h a d a sensitivity of
cians (range 0-7), including psychiatrists, before 23-38 per c e n t w i t h a specificity of 90 per c e n t .
the correct diagnosis w a s m a d e .
C h i l d r e n u s u a l l y c o m p l a i n of l a n c i n a t i n g p a i n
t h a t is e a s i l y p r e c i p i t a t e d b y c o l d w e a t h e r o r a
c o l d b a t h . Direct p r e s s u r e m a y t r i g g e r p a i n , w h i c h Imaging
c h i l d r e n c o m m o n l y localize t o t h e i r f i n g e r t i p s . T h e
c l a s s i c t r i a d o f p a i n , c o l d s e n s i t i v i t y , a n d localized Plain X-rays
t e n d e r n e s s is not often s e e n in y o u n g children
( U p t o n a n d C o o m b s 1995). P l a i n X - r a y s ( a n t e r o p o s t e r i o r a n d lateral) of t h e
distal p h a l a n x s h o u l d b e p e r f o r m e d in all c a s e s ,
e v e n if t h e d i a g n o s i s of g l o m u s t u m o r is o b v i o u s .
Radiography sometimes s h o w s a small impres-
Physical findings s i o n or s c l e r o t i c r e a c t i o n in t h e b o n e a d j a c e n t to
t h e t u m o r a n d w i l l h e l p t o localize m o r e p r e c i s e l y
In c a s e s of s u b u n g u a l g l o m u s t u m o r , a v i s i b l e o r
the tumor. X - r a y s s h o u l d be c o m p a r e d to the
palpable m a s s , a subungual blue discoloration,
normal side. Usually, a posteroanterior v i e w a n d
and a n increased curvature or deforming ridges a lateral v i e w a r e sufficient. A n o b l i q u e v i e w of
o n t h e nail c a n b e f o u n d . T h e p h y s i c a l f i n d i n g s in t h e h a n d w i l l b e n e c e s s a r y if t h e p a t h o l o g y is
suspected glomus tumor of t h e p u l p a r e often s u s p e c t e d t h e r e . O b l i q u e v i e w of t h e finger c a n
v e r y poor, although, a blue spot o n the pulp a n d
s h o w subtle b o n e e r o s i o n . In s o m e rare c a s e s of
a palpable nodule may be present. In many
primary g l o m u s tumor of b o n e ( S i m m o n s et al
c a s e s , t h e p h y s i c a l f i n d i n g s in a n y l o c a t i o n a r e 1992), a n i n t r a o s s e o u s b o n y expansion can be
a b s e n t a n d t h e e x a m i n a t i o n o f t h e f i n g e r is t o t a l l y
s e e n . A lateral v i e w c a n s o m e t i m e s s h o w a p s e u d o -
normal.
thickening of t h e nail b e d , indicative of a m a s s or
t u m o r located in t h e soft tissue ( F o u c h e r et al 1999),
but it is v e r y inconsistent a n d non-specific.

Clinical tests for diagnosis

The d i a g n o s i s of glomus tumor is frequently Ultrasonography
d e l a y e d for n u m e r o u s r e a s o n s . T h e m a i n r e a s o n
is g e n e r a l l y a n o n - s p e c i f i c p r e s e n t a t i o n of the If t h e t u m o r c a n n o t b e localized clinically or w i t h
t u m o r o r a n i n c o r r e c t d i a g n o s i s , for e x a m p l e , of plain X-rays, ultrasonography can be helpful.
n e u r o m a or foreign body. Ultrasonography performed with a high-frequency
S e v e r a l c l i n i c a l t e s t s h a v e b e e n d e s c r i b e d to transducer depicts tumors a s small as 3 m m in
a i d t h e d i a g n o s i s of a g l o m u s t u m o r . L o v e (1944) d i a m e t e r , particularly in t h e p u l p of t h e finger
50 T U M O R S OF THE HAND

( F o r n a g e 1988, B i a n c h i et al 1999). T h e result of neurilemmoma, calcinosis, chronic paronychia,

t h i s e x a m i n a t i o n is v e r y d e p e n d e n t o n t h e r a d i o - epithelial inclusion cyst, g a n g l i o n , fibroma, e x o s -
logist's skill. H o w e v e r , t h e c u r v a t u r e of t h e nail tosis, pacinian hyperplasia, pigmented nevus,
p l a t e m a y c r e a t e artifacts, a n d s m a l l , f l a t t e n e d m e l a n o m a , e t c . T h e differential d i a g n o s i s , r e g a r d -
subungual l e s i o n s a r e difficult t o detect with ing all t h e d i a g n o s t i c p o s s i b i l i t i e s , is v e r y l a r g e .
u l t r a s o n o g r a p h y ( F o r n a g e 1988). However, the combination of patient history,
clinical examination, positive tests, and radio-
l o g i c a b n o r m a l i t i e s , f o c u s e s o n t h e d i a g n o s i s of
g l o m u s tumor. Only the histopathological analysis
Scintigraphy is d e f i n i t i v e l y d i a g n o s t i c .

S c i n t i g r a p h y is u s u a l l y p o s i t i v e , but n o n - s p e c i f i c .
Its v a l u e for t h e d i a g n o s i s of a g l o m u s t u m o r is
very low.
Treatment
Surgery
Magnetic resonance imaging
S u r g i c a l e x c i s i o n of t h e t u m o r is t h e o n l y c u r a t i v e
Magnetic resonance imaging (MRI) and angio-MRI treatment.
are n o w the most accurate tools for diagnosing T h e m o s t p r e c i s e location of t h e t u m o r h a s to
g l o m u s t u m o r . A r t e r i o g r a p h y is n o l o n g e r utilized. b e d e t e r m i n e d p r e o p e r a t i v e y , u s i n g L o v e ' s test.
The signal behavior on M R I depends on the U s u a l l y t h e s u r g i c a l p r o c e d u r e is p e r f o r m e d u n d e r
histological composition of t h e g l o m u s tumor. local o r r e g i o n a l a n e s t h e s i a . B h a s k a r a n a n d a n d
T h e m o r e vascular t y p e s h o w s a high signal o n Navadgi (2002) used a double-tourniquet tech-
T2-weighted images. On angio-MRI an early nique which enabled delineation of t h e lesion
e n h a n c e m e n t is s e e n at arterial t i m e i n c r e a s i n g at m o r e clearly f r o m t h e s u r r o u n d i n g t i s s u e . A m i d -
d e l a y e d v e n o u s acquisition. T h e m o r e cellular or a r m p n e u m a t i c t o u r n i q u e t is first a p p l i e d w i t h o u t
s o l i d t y p e is difficult t o d e t e c t w i t h M R I , its s i g n a l e x s a n g u i n a t i o n o r e l e v a t i o n of t h e l i m b . T h e digit is
b e i n g s i m i l a r t o t h a t of t h e n o r m a l d e r m i s of t h e then exsanguinated a n d a rubber catheter applied
nail bed on all sequences. The mucoid type at t h e b a s e of t h e digit. A t e x p l o r a t i o n t h e w h o l e
of g l o m u s t u m o r p r e s e n t s a faint e n h a n c e m e n t s u r g i c a l field a p p e a r s b l a n c h e d but, after t h e f i n g e r
but has a high signal o n T 2 - w e i g hted images tourniquet is r e l e a s e d , t h e lesion b e c o m e s c o n -
o w i n g t o t h e l a r g e a m o u n t of w a t e r in t h e s t r o m a . gested, while the surrounding tissues remain
Numerous tumors are a combination of t h e s e b l a n c h e d . T h i s t e c h n i q u e is of particular v a l u e in
different h i s t o l o g i c a l t y p e s a n d t h i s m a y e x p l a i n t h e m o r e v a s c u l a r t y p e of g l o m u s t u m o r .
t h e i n c o n s i s t e n t results of M R I in t h e d i a g n o s i s of The surgical approach should be planned
g l o m u s t u m o r . In t h e c a s e of m u l t i p l e glomus a c c o r d i n g to t h e size a n d l o c a t i o n o f t h e t u m o r .
t u m o r s in t h e h a n d o r in t h e s a m e fingertip, a n g i o - T h e r e is c o n t r o v e r s y in t h e literature a b o u t t h e
M R I d e t e c t i o n is helpful ( D r a p e 2002). M R I is a l s o s u r g i c a l a p p r o a c h to s u b u n g u a l t u m o r . T h e lateral
v e r y helpful in r e v e a l i n g a r e c u r r e n c e of g l o m u s t y p e ( F i g . 2) m a y b e e x c i s e d b y a lateral a p p r o a c h
tumor after surgery. T h e u m a n n et al (2002) ( u s e d b y s o m e a u t h o r s ( G i e l e 2002) f o r all l o c a -
r e p o r t e d in a s e r i e s of 24 c a s e s t h a t M R I r e v e a l e d t i o n s of s u b u n g u a l g l o m u s t u m o r ) ( F i g . 3). U s i n g
a n o d u l e c o m p a t i b l e w i t h t h e d i a g n o s i s of a recur- t h i s lateral l o n g i t u d i n a l i n c i s i o n , a r i d g e d e f o r m i t y
rent g l o m u s t u m o r , w h i c h w a s c o n f i r m e d at t h e has sometimes been noted. T h e median subun-
h i s t o p a t h o l o g i c a l a n a l y s i s in 22 c a s e s . gual type m a y require a transungual approach,
w i t h or w i t h o u t nail r e m o v a l . U s i n g t h i s t r a n s u n -
g u a l a p p r o a c h , c a r e f u l nail b e d s u t u r e is m a n d a -
t o r y to a v o i d p o s t o p e r a t i v e nail d e f o r m i t y and
Differential diagnosis cosmetic complications.
T w o p o i n t s a r e i m p o r t a n t to a v o i d r e c u r r e n c e
The differential d i a g n o s i s of glomus tumor is of p o s t o p e r a t i v e p a i n : t h e e n t i r e t u m o r , u s u a l l y
v e r y e x t e n s i v e . It i n c l u d e s n e u r o m a , c a u s a l g i a , e n c a p s u l a t e d , s h o u l d b e r e m o v e d e n b l o c ; a n d it
arthritis, gout, melanoblastoma, neurofibroma, is imperative to search carefully for multiple
 G L O M U S TUMOR OF THE HAND 51

t h e r a p y h a s n e v e r d e m o n s t r a t e d its e f f i c a c y in t h e
t r e a t m e n t of g l o m u s t u m o r . H o w e v e r , in s u p e r -
ficial l e s i o n s , a r g o n l a s e r c a n b e u s e f u l .

Recurrence
After s u r g e r y , m o s t s t u d i e s s h o w a r e c u r r e n c e of
t u m o r in t h e r a n g e of 5 - 1 5 p e r c e n t , w i t h a m a x i -
m u m of 50 p e r c e n t ( B r e n n e r et al 1995). E a r l y
r e c u r r e n c e is d u e m o s t l y to i n c o m p l e t e e x c i s i o n
of the tumor. The p r e s e n c e of unrecognized
multiple t u m o r s c a n a l s o explain t h e e a r l y recurrent
s y m p t o m s . D e l a y e d or late r e c u r r e n c e is u s u a l l y
Figure 2
owing to the development of a new glomus
t u m o r n e a r t h e e x c i s i o n s i t e . D a i l i a n a et al (1999)
Glomus tumor of the pulp: lateral approach.
h a v e reported a g l o m u s t u m o r with four recur-
r e n c e s , p r o b a b l y r e l a t e d to t h e d e v e l o p m e n t of
new tumors. In s u c h a c a s e M R I , particularly
u s i n g t h e n e w g e n e r a t i o n of M R I u n i t s , c a n b e
helpful p r e o p e r a t i v e l y .

Malignant tumor
Malignant transformation of g l o m u s t u m o r s is
exceedingly rare.
Occasionally, g l o m u s tumors display unusual
f e a t u r e s , s u c h a s l a r g e size, d e e p l o c a t i o n , infil-
t r a t i v e g r o w t h , m i t o t i c activity, n u c l e a r p l e o m o r -
p h i s m , a n d n e c r o s i s . In 2 0 0 1 , F o l p e et al (2001),
s t u d i e d 52 c a s e s of u n u s u a l g l o m u s t u m o r s . T h e y
proposed the following classification s c h e m e a n d
criteria:
Figure 3

(1) Malignant glomus tumor: tumors with a

Subungual glomus tumor, after resection.
d e e p l o c a t i o n a n d a size of m o r e t h a n 2 c m ,
or atypical mitotic figures, or m o d e r a t e to
lesions even m i c r o s c o p i c a l l y to prevent recur-
high nuclear g r a d e , a n d > 5 mitotic figures/
r e n c e of s y m p t o m s f r o m u n r e c o g n i z e d t u m o r s .
50HPF.
The recovery period f r o m surgery ranges f r o m
(2) Symplastic glomus t u m o r : t u m o r s w i t h h i g h
2 to 4 w e e k s , a l t h o u g h o c c a s i o n a l l y it m a y t a k e
n u c l e a r g r a d e in t h e a b s e n c e of a n y o t h e r
s e v e r a l m o n t h s f o r t h e p a i n c o m p l e t e l y to s u b -
malignant feature.
s i d e ( R o h r i c h e t al 1994).
(3) Glomus tumor of uncertain malignant poten-
tial: t u m o r s t h a t lack criteria f o r malignant
g l o m u s tumor or symplastic g l o m u s tumor
Others but h a v e h i g h m i t o t i c a c t i v i t y a n d s u p e r f i c i a l
location only, or l a r g e size o n l y , o r deep
If t h e p a t i e n t d o e s no t w a n t o r is m e d i c a l l y unfit location only.
for operation, prostaglandin inhibition therapy (4) Glomagiomatosis: tumors with histological
can lead to at least temporary pain relief. f e a t u r e s of diffuse a n g i o m a t o s i s a n d e x c e s s
Indomethacin has also been used. Radiation g l o m u s cells.
52 T U M O R S OF THE HAND

Using this classification s c h e m e , metastasis w a s Bianchi S , Martinoli C, Abdelwahab I (1999) High-

o b s e r v e d in 38 p e r c e n t of t u m o r s fulfilling t h e frequency ultrasound examination of the wrist and hand,
criteria for malignancy. In contrast metastatic Skeletal Radiol 28:121-9.
d i s e a s e w a s not s e e n in a n y s p e c i m e n c l a s s i f i e d
as symplastic glomus tumor, glomus tumor Brenner P, Krause-Bergmann A , Witting K (1995) Solitary
glomus tumors of the hand. A clinicopathologic evalua-
of uncertain malignant potential, or gloman-
tion study, Langenbecks Arch Chir 380:321-6.
giomatosis. From these 52 cases, only seven
w e r e l o c a t e d in t h e w r i s t o r h a n d . T h e m a l i g n a n t
CarlstedtT, Lugnegard H (1983) Glomus tumor in the
f o r m h a s a p r e d i l e c t i o n for t h e l o w e r e x t r e m i t i e s
hand, Acta Orthop Scand 54:296-302.
( K a y a l et al 2001). W i d e e x c i s i o n of t h e l e s i o n a n d
o n c o l o g i c a l f o l l o w - u p h a s b e e n f o u n d to b e a d e -
Carroll R E , Berman AT (1972) Glomus tumor in the hand.
q u a t e t h e r a p y ( W e t h e r i n g t o n et al 1997). Review of the literature and report of 28 cases, J Bone
Joint Surg 54A:691-702.

Dailiana Z H , Drapé J - L , LeViet D (1999) A glomus tumor

Conclusion with four recurrences, J Hand Surg 24B:131-2.

G l o m u s t u m o r of t h e h a n d is a w e l l d e f i n e d a n a t o - Drape J - L (2002) Imaging of the tumors of the periony-

m o c l i n i c a l entity: rare ( 1 - 5 per c e n t of t h e hand chium, Hand Clin 18:655-70.
t u m o r s ) , b e n i g n in m o s t of t h e c a s e s , m o r e fre-
q u e n t in m i d d l e - a g e d w o m e n , u s u a l l y l o c a t e d in Ekin A , Ozkan M , Kabaklioglu T (1997) Subungual
t h e distal p h a l a n x ( s u b u n g u a l ) , a n d its d i a g n o s i s is glomus tumor: a different approach to diagnosis and
treatment, J Hand Surg 22B:228-9.
f r e q u e n t l y d e l a y e d , m o s t l y b e c a u s e of t h e a b s e n c e
of specific f i n d i n g s at t h e e x a m i n a t i o n . T h e t y p i c a l
Fleegler E J , Zeinowicz R J (1990) Tumors of the perio-
s y m p t o m s a r e p a r o x y s m a l p a i n , c o l d sensitivity,
nychium. Hand Clin 1:113-33.
a n d e x q u i s i t e point t e n d e r n e s s a b o u t t h e t u m o r .
Plain X - r a y s can s h o w s o m e small bone modifica-
Folpe A L , Fanburg-Smith J C , Miettinen M , Weiss S W
tion t h a t m u s t b e c o m p a r e d w i t h t h e c o n t r a l a t e r a l (2001) Atypical and malignant glomus tumors, analysis
finger. M R I a n d a n g i o - M R I w i l l f r e q u e n t l y c o n f i r m of 52 cases, with a proposal for reclassification, Am J
t h e d i a g n o s i s . T h e t r e a t m e n t is careful a n d com- Surg Pathol 25:1-12.
plete e x c i s i o n of t h e t u m o r . R e c u r r e n c e s a r e not
unusual, mostly due to incomplete removal or Fornage B D (1988) Glomus tumors in the fingers:
p r e s e n c e of m u l t i p l e t u m o r s . diagnosis with U S , Radiology 167:183-5.

Foucher G , Le Viet D, Dailiana Z, Pajardi G (1999) Les

tumeurs glomiques de la région unguéale, à propos
References d'une série de 55 patients, Revue Chir Orthop
Reparatrice Appar Mot 85:362-6.
Arkwright S , Bachar M, Vilde F (1996)Tumeurs glomiques
étude clinique, histologique et immunohistochimique, à Gandon F, Legaillard P, Brueton R et al (1992) Forty-eight
propos de 29 cas, Arch Anat Cytol Path 44:160-5. glomus tumors of the hand: retrospective study and four
years follow-up, Ann Chir Main Memb Super 11:401-5.
Barre J A (1922) S u r certaines sympathalgies de la
périphérie des membres: leur traitement chirurgical Giele H (2002) Hildreth's test is a reliable clinical sign
simple, Paris Med 45:311-15. for the diagnosis of glomus tumors, J Hand Surg
27B: 157-8.
Barre J A , Masson P (1924) Etude anatomo-clinique
de certaines tumeurs sous-unguéales douloureuses Graham B, Wolf T W (1992) Synchronous subungual glo-
(tumeurs du glomus neuro-myo-artériel des extremitées), mus tumors in adjacent digits, J Hand Surg 17B:575-6.
Bull Soc Franc P Derm Syphil 31:148-59.
Heys S D , Brittenden P, Atkinson P, Emerin O (1992)
Bhaskaranand K, Navadgi B C (2002) Glomus tumor of Glomus tumor: an analysis of 43 patients and review of
the hand, J Hand Surg 27B:229-31. the literature, Br J Surg 79:345-7.
 G L O M U S TUMOR OF THE HAND 53

Hildreth DH (1970)The ischemia test for glomus tumor: Popoff N W (1934) The digital vascular system with
a new diagnostic test, Rev Surg 27:147-8. reference to the state of the glomus in inflammation,
arteriosclerotic gangrene, diabetic gangrene, thrombo-
J o s e p h FR, Posner M A (1983) Glomus tumor of the angitis obliterans, and supernumerary digits in man,
wrist, J Hand Surg 8A:918-20. Arch Pathol 18:295-330.

Kayal J D , Hampton R W , Sheehan D J , Washington CV Rohrich R J , Hochstein L M , M i l l w e e RH (1994)

(2001) Malignant glomus tumor: a case report and Subungual glomus tumor: an algorithmic approach,
review of the literature, Dermatol Surg 27:837-40. Ann Plast Surg 33:300-4.

Kouskoukis CE (1983) Subungual glomus tumor: a S i m m o n s T J , BasslerTJ, Schwinn CP, Forrester DM (1992)
clinico-pathological study, J Dermat Surg Oncol 9:294. Case report 749, Skeletal Radiol 21:407-9.

Lever W F (1983) Histopathology of the skin. J B Lippincott Theumann N H , Goettmann S , Le Viet D et al (2002)
Co: Philadelphia. Recurrent glomus tumors of fingertips: M R imaging
evaluation, Radiology 223:143-51.
Love J G (1944) Glomus tumors diagnosis and treat-
ment, Mayo Clin Proc 19:918-20. Upton J , Coombs C (1995) Vascular tumors in children,
Hand Clin 11:307-36.
Masson P (1924) Le glomus neuromyo-artériel des
regions tactiles et ses tumeurs, Lyon Chir 21:257-80. Van Geertruyden J , Lorea P, Goldschmidt S , et al (1996)
Glomus tumors of the hand, a retrospective study of
Moor EV, Goldberg I, Westreich M (1999) Multiple glo- 51 cases, J Hand Surg 21B:257-60.
mus tumor: a case report and review of the literature,
Ann Plast Surg 43:436-8. Wetherington W R , Lyle G L , Sangüeza P O (1997)
Malignant glomus tumor of the thumb: a case report,
Nakamura K (1992) Multiple glomus tumors associated J Hand Surg 22A: 1098-102.
with arteriovenous fistulas and with nodular lesions of
the finger joints, Plast Reconstr Surg 90:675-83. W o o d W (1812) On painful subcutaneous tubercle,
Edinb Med J 8:283-91.
Noor A M , Masbah O (1997) Synchronous glomus
tumor in a distal digit: a case report, J Hand Surg
22A:508-10.
7
Osteoid osteoma
of the hand and wrist
Dominique Le Viet and loannisTsionos

Introduction C o n c e r n i n g o s t e o i d o s t e o m a s of t h e h a n d a n d
w r i s t , t h r e e r e l a t i v e l y l a r g e s e r i e s ( B e d n a r et al
T u m o r s of o s s e o u s o r i g i n a r e l e s s f r e q u e n t in t h e (1993), 24 c a s e s ; A m b r o s i a et al (1987), 19 c a s e s ;
u p p e r e x t r e m i t y t h a n soft t i s s u e o n e s . O s t e o i d A l l i e u a n d L u s s i e z (1988), 46 c a s e s ) c o i n c i d e in
o s t e o m a is a b e n i g n o s t e o b l a s t i c t u m o r w i t h a t h a t m e t a c a r p a l i m p l i c a t i o n is l e s s f r e q u e n t t h a n
p r e d i l e c t i o n f o r t h e l o w e r e x t r e m i t y . W h e n in c a r p a l or p h a l a n g e a l ; p h a l a n g e a l o s t e o i d o s t e o -
t h e u p p e r e x t r e m i t y , it u s u a l l y p o s e s d i a g n o s t i c m a s m o r e f r e q u e n t l y c o n c e r n p r o x i m a l or d i s t a l ,
p r o b l e m s w i t h its f r e q u e n t l y s u b t l e c l i n i c a l a n d rarely middle, p h a l a n g e s .
presentation.
O s t e o i d o s t e o m a w a s first d e s c r i b e d a s a s e p -
a r a t e e n t i t y b y J a f f e in 1935. In its t y p i c a l f o r m , it Symptoms and signs
c o n s i s t s of a v a r i a b l y o s s i f i e d o s t e o i d , e m b e d d e d
w i t h i n a s t r o m a of r e l a t i v e l y l o o s e v a s c u l a r c o n - T h e c l a s s i c c l i n i c a l m a n i f e s t a t i o n of o s t e o i d
n e c t i v e t i s s u e . O s s i f i e d t i s s u e is in t h e f o r m of o s t e o m a is f r e q u e n t l y c o n s i d e r e d to b e p a i n ,
w o v e n b o n e . T h e w h o l e l e s i o n is c a l l e d a ' n i d u s ' w h i c h is w o r s e at night a n d r e l i e v e d b y n o n -
a n d m e a s u r e s l e s s t h a n 1 c m in d i a m e t e r steroidal anti-inflammatory drugs (NSAIDs),
( A t h a n a s i a n 1999); it is u s u a l l y s u r r o u n d e d b y a n o t a b l y a s p i r i n . A s to t h e m e c h a n i s m of p a i n , it
r i m of r e a c t i v e s c l e r o t i c b o n e . O c c a s i o n a l l y , t h e s e e m s that it is d u e to a u n i q u e characteristic
n i d u s m a y b e d o u b l e ( A l l i e u et al 1989, M u r e n of this t u m o r : locally i n c r e a s e d s e c r e t i o n of pro-
e t a l 1991). s t a g l a n d i n s a p p e a r s to c a u s e v a s o d i l a t a t i o n a n d
T h e l e s i o n t y p i c a l l y b e c o m e s clinically a p p a r - h e n c e i n c r e a s e d local i n t r a o s s e o u s p r e s s u r e . A
e n t in t h e first t w o o r t h r e e d e c a d e s of life a n d , in l a r g e q u a n t i t y of n e r v e f i b e r s w a s f o u n d in t h e
l a r g e s e r i e s , m e n a r e m o r e f r e q u e n t l y affected r e a c t i v e z o n e c i r c u m s c r i b i n g t h e n i d u s a n d in t h e
t h a n w o m e n ( A m b r o s i a et al 1987, A l l i e u a n d n i d u s itself ( O ' C o n n e l et al 1998). T h e s e n e r v e
L u s s i e z 1988, R o s e n t h a l et al 1998, C a m p a n a c c i f i b e r s a r e f r e q u e n t l y in t h e v i c i n i t y of b l o o d
et al 1999, V a n d e r s c h u e r e n et al 2002). It is a l s o v e s s e l s a n d m a y be s e n s i t i v e to the a b o v e
f o u n d m o r e c o m m o n l y in w h i t e populations c h a n g e s . N e r v e f i b e r s a r e not f o u n d in s u c h a b u n -
( K e n d r i c k a n d E v a r t s 1975). F u r t h e r m o r e , it r e p r e - d a n c e in o t h e r t u m o r s , e v e n in t h e histologically
s e n t s s o m e 11 p e r c e n t of all b e n i g n b o n e t u m o r s i n s e p a r a b l e o s t e o b l a s t o m a s ( O ' C o n n e l et al 1998).
( D a h l i n 1979). F e m o r a l a n d tibial localizations L o c a l t e n d e r n e s s , s w e l l i n g , h e a t , a n d r e d n e s s of
a c c o u n t f o r t h e m a j o r i t y of c a s e s . U p p e r e x t r e m - t h e o v e r l y i n g t i s s u e s m a y a l s o b e p r e s e n t . In fact,
ity o s t e o i d o s t e o m a s m a k e u p 19-31 p e r c e n t of a l t h o u g h characteristic, p a i n is n e i t h e r specific
all c a s e s ( B e d n a r et al 1995); s o m e of t h e s e ( a b o u t for this p a t h o l o g y n o r u n i v e r s a l l y p r e s e n t . T h e
10 p e r c e n t of all o s t e o i d o s t e o m a s ) a r e localized p e r c e n t a g e of p a i n l e s s o s t e o m a s is g e n e r a l l y
in t h e w r i s t a n d h a n d . reported to b e b e t w e e n 1.6 a n d 11 p e r c e n t , s u b j e c t
56 T U M O R S O F T H E HAND

a c

Figure 1

Osteoid osteomas in carpal bones: (a) trapezoid, (b) capitate, and (c) hamate bone.

to v a r i a t i o n a m o n g s e r i e s ( A l l i e u a n d L u s s i e z 1988, (Lisanti et al 1996). Finally, o s t e o i d osteomas

B e d n a r et al 1995).The m a j o r i t y of reports o n p a i n - l o c a t e d n e a r articulations m a y c a u s e r e a c t i v e j o i n t
less o s t e o m a s in t h e h a n d c o n c e r n t h e p h a l a n g e s effusion a n d limitation in t h e r a n g e of motion
( W i s s a n d R e i d 1983, R e x et al 1997, D e S m e t et al ( A m b r o s i a et al 1987, B e d n a r et al 1995). H y p e r -
1998, B a s u et al 1999), c o m m o n l y in c h i l d r e n . T h e h y d r o s i s is s o m e t i m e s d e s c r i b e d ( A m b r o s i a e t a l
c h a r a c t e r of t h e p a i n d e p e n d s o n t h e location of 1987). M e d i a n n e r v e c o m p r e s s i o n resulting f r o m
t h e t u m o r : in c a s e s of o s t e o i d o s t e o m a in t h e a n o s t e o i d o s t e o m a in t h e c a p i t a t e b o n e h a s a l s o
wrist the pain tends to be dull and diffuse, b e e n d e s c r i b e d ( H e r n d o n et al 1974).
w h e r e a s in p h a l a n g e a l localizations it is m o r e e a s -
ily l o c a t e d b y t h e patient. W h e n pain is a b s e n t ,
swelling m a y be the prominent s y m p t o m (Wiss
a n d R e i d 1983), s o m e t i m e s a c c o m p a n i e d b y limi- Radiographic control
t a t i o n in r a n g e of m o t i o n of t h e a d j a c e n t j o i n t s .
S w e l l i n g is g e n e r a l l y c o m m o n e r in t h e h a n d t h a n Osteoid osteomas m a y present on simple X-rays
in t h e f o r e a r m a n d a r m ( B e d n a r et al 1995). For as a radiolucent focus (corresponding to the
o s t e o i d o s t e o m a s s i t u a t e d in digits, e n l a r g e m e n t nidus) surrounded by a sclerotic o s s e o u s rim.
t a k e s t h e f o r m of m a c r o d a c t y l y a n d is a n alerting D e p e n d i n g o n t h e d e g r e e of o s s i f i c a t i o n taking
sign easily detectable by the patient and the place within the nidus, a radioopaque central area
e x a m i n e r ; h o w e v e r , for t h o s e s i t u a t e d in c a r p a l m a y b e v i s i b l e ( F i g s . 1 - 4 ) . T h i s is t h e t y p i c a l r a d i o -
bones, swelling m a y b e a b s e n t , particularly in logic picture of a f u l l - b l o w n o s t e o i d osteoma,
t u m o r s l o c a t e d o n t h e p a l m a r s i d e of c a r p a l b o n e s but radiographic signs may differ according
 OSTEOID O S T E O M A OF THE HAND AND W R I S T 57

Figure 2

Osteoid osteoma in the left ulnar styloid. Tumor is hardly visible (arrowheads) on standard X-rays (a), but can be easily located
on computed tomography scan (b). Bone scan (c) is positive (arrow).
58 T U M O R S OF THE HAND

Figure 3

Osteoid osteoma in distal phalanx. Note deformation of digit and nail (clubbing).

t o t h e localization into t h e b o n e ( E d e i k e n et al to t h e n i d u s is v i v i d , t h e a b o v e d e s c r i p t i o n is r a t h e r
1966) ( c o r t i c a l , m e d u l l a r y , a n d s u b p e r i o s t e a l ( D e relevant, although the reactive bone sclerosis
S m e t 2002)) a n d to t h e t i m e passed between m a y s o m e t i m e s be so d e n s e and the nidus so
o n s e t of s y m p t o m s a n d X - r a y c o n t r o l . In cortical minuscule that the latter may not be
osteoid o s t e o m a s , w h e r e b o n e sclerotic reaction v i s i b l e o n s i m p l e X - r a y s ( F i g . 5). In medullary
 OSTEOID O S T E O M A OF THE HAND AND W R I S T 59

a b c d

Figure 4

Osteoid osteoma in proximal phalanx (a) and (b). Tumor is superficial and not covered by bone cortex. By
palmar approach (c), tumor is extirpated without farther cortical violation (arrow shows the cavity left) and no grafting is done,
(d) Cavity is filled with new bone (arrow) at 6 months postoperation.

Figure 5

Osteoid osteoma in the proximal

phalanx of the right middle finger.
Note fusiform swelling of finger
(a). Nidus is hardly visible (arrow),
located inside a vivid cortical reac-
a b e tion (b). Bone scan is positive (c).
60 T U M O R S OF THE HAND

the contrary, a reactive articular effusion and

s y n o v i t i s m a y f o l l o w its p r e s e n c e a n d p r o v o k e a
d e c r e a s e in r a n g e of m o t i o n . N o n e t h e l e s s , r a d i o -
l o g i c s i g n s of t h e t u m o r d e p e n d o n t h e t i m i n g
of X - r a y c o n t r o l : t y p i c a l s i g n s m a y n o t a p p e a r
before 6 m o n t h s to 2 y e a r s f r o m t h e onset of
symptoms ( M a r c u z z i et al 2002). Radiographic
a p p e a r a n c e m a y a l s o d e p e n d o n t h e t y p e of b o n e
a f f e c t e d . F o r e x a m p l e , o s t e o i d o s t e o m a s in distal
phalanges of the hand frequently appear as
p u r e l y lytic l e s i o n s ( B o w e n et al 1987). S h i f t of
p o s i t i o n of o s t e o i d o s t e o m a f r o m s u b p e r i o s t e a l
to i n t r a m e d u l l a r y l o c a t i o n m a y a l s o b e part of t h e
t u m o r ' s n a t u r a l h i s t o r y ( K a y s e r et al 1998), t h u s
rendering less valid any radiologic classification
of t h e s e t u m o r s .

Three-phase bone scan

Radiolabeled technetium (Tc 9 9 m ) b o n e s c a n is a
valuable tool for the diagnosis of osteoid
o s t e o m a . It p e r m i t s t h e i d e n t i f i c a t i o n of a n a r e a of
increased uptake in t h e delayed views, which
coincides with a nidus actively producing osteoid.
It has been almost universally positive in
o s t e o i d o s t e o m a s later c o n f i r m e d by histologic
Figure 6 e x a m i n a t i o n ( M a r c u z z i et al 2002). T h i s e x a m i n a -
tion can provide confirmation of a suspicion
Osteoid osteoma near metacarpophalangeal joint. Nidus is e s t a b l i s h e d b y s i m p l e X - r a y s or, if X - r a y s a r e
partially ossified (arrowheads). Reactive bone formation at n o r m a l , identify a b o n e area n e e d i n g further e v a l -
distance from the tumor (arrows).
u a t i o n b y i m a g i n g s t u d i e s . It is t h u s c o n s i d e r e d to
b e t h e s e c o n d t e s t in t h e e v a l u a t i o n algorithm
( B e d n a r et al 1995). A s p e c i a l t e c h n i q u e , registra-
osteoid osteomas, reactive sclerosis is less t i o n b o n e s c a n ( H a w k e s et al 1991) ( s u p e r i m p o s i -
prominent and the w h o l e lesion may be less t i o n of s i m p l e r a d i o g r a p h a n d b o n e s c a n ) , m a y
noticeable and be overlooked. Subperiosteal substantially h e l p in localizing t h e t u m o r , e s p e -
o s t e o i d o s t e o m a s a r e r a r e in t h e h a n d ( A l l i e u a n d cially in w r i s t osteoid o s t e o m a s of previously
Lussiez 1988, C h a m b e r l a i n et al 1992). Radio- e x p l o r e d h a n d s ( W i l s o n et al 1997), w h e r e c o m -
logically, t h e y m a y a p p e a r a s s u b t l e radiolucent p u t e d t o m o g r a p h y ( C T ) s c a n m a y fail t o d e t e c t a
a r e a s in t h e vicinity of, but not i n s i d e , b o n e cortex, lesion consistent with this tumor.
to w h i c h they m a y provoke s o m e resorption; a
reactive sclerosis produced by the periosteum
c a n a l s o b e p r e s e n t ( C h a m b e r l a i n et al 1992).
Subperiosteal osteoid o s t e o m a s are said to s h o w Computed tomography
a m o r e p r o n o u n c e d clinical t h a n r a d i o l o g i c pro-
file, a s t h e y a r e s u p e r f i c i a l a n d u s u a l l y provoke C T s c a n m a y b e t h e t h i r d i m a g i n g s t e p in t h e e v a l -
local s i g n s ( s w e l l i n g , t e n d e r n e s s ) ( C h a m b e r l a i n u a t i o n of a s u s p e c t e d o s t e o i d o s t e o m a . W h e n t h e
et al 1992). F i n a l l y , w h e n a n o s t e o i d o s t e o m a is l e s i o n c a n n o t b e i d e n t i f i e d in s i m p l e X - r a y s a n d
intra-articular, it m a y not p r o v o k e cortical r e a c t i v e Tc 99m bone scan s h o w s a focus of increased
b o n e f o r m a t i o n or this m a y be found at s o m e uptake, CT m a y detect the lesion and thus c o n -
d i s t a n c e f r o m t h e f o c u s of t h e t u m o r ( F i g . 6 ) ; o n tribute to diagnosis. E v e n w h e n a diagnosis h a s
 OSTEOID O S T E O M A OF THE HAND AND W R I S T 61

already been m a d e by other m e t h o d s , C T m a y

h i g h l i g h t t h e e x a c t l o c a t i o n of t h e l e s i o n in t h r e e
d i m e n s i o n s , t h u s h e l p i n g in t h e o p e r a t i v e p l a n -
ning. CT sections must be thin e n o u g h (1-2 m m )
to locate the tumor. Occasionally, an osteoid
o s t e o m a m a y not s h o w t y p i c a l s i g n s o n C T s c a n .
In t h e s e c a s e s , a d y n a m i c C T s c a n w i t h intra-
venous contrast enhancement can be used
( M c G r a t h et al 1996), but t h e r a d i a t i o n d o s e is
i m p o r t a n t c o n s i d e r i n g t h e a g e of t h e s e p a t i e n t s .

Magnetic resonance
imaging Figure 7

Magnetic resonance imaging (MRI) focuses more Histological appearance of hand osteoid osteoma. Note
o n soft t i s s u e a l t e r a t i o n s t h a n o s s e o u s o n e s a n d , ramifying trabeculae of osteoid (arrows) and abundant loose
t h e r e f o r e , h a s b e e n c o n s i d e r e d to b e less s u i t a b l e fibrovascular tissue (asterisks).
f o r t h e e v a l u a t i o n of o s t e o i d o s t e o m a s . It m a y
show soft tissue (Woods et al 1993) and
intramedullary edema ( K a w a g u c h i et al 2001)
a r o u n d t h e l e s i o n , but in o s t e o i d o s t e o m a this this t u m o r , b u t d o e s n o t p r o v i d e p r e c i s e s p a t i a l
m a y b e d e c e p t i v e l y a l a r m i n g a s to t h e a g g r e s - i n f o r m a t i o n , a s is p r o v i d e d b y C T or M R I .
s i v e n e s s of t h e t u m o r ( A s s o u n et al 1994). In f a c t ,
s u c h s e r i e s , e a r l y a n d r e c e n t ( D a v i e s e t a l 2002),
r e p o r t i n g M R I to b e less e f f i c a c i o u s in i m a g i n g of
osteoid osteomas than CT, r e f e r r e d to non-
Pathology
e n h a n c e d M R I techniques. Recent reports using Macroscopically, the tumor shows a reddish
gadolinium-enhanced dynamic MRI showed c o l o r a t i o n w i t h g r a n u l a r texture. Microscopically,
e n c o u r a g i n g results ( L i u et al 2003). In f a c t , t h i s t h e p r i m a r y lesion (nidus) is a n o n - i n v a s i v e f o c u s
t e c h n i q u e t a k e s a d v a n t a g e of t h e t u m o r ' s h y p e r - m a d e of o s t e o i d t i s s u e d e m o n s t r a t i n g a s o m e w h a t
v a s c u l a r i t y in o r d e r to a c h i e v e c o n t r a s t e n h a n c e - a t y p i c a l ossification p r o c e s s a n d a l o o s e f i b r o v a s -
ment b e t w e e n it and the surrounding tissues c u l a r c o n n e c t i v e t i s s u e ( F i g . 7). T h e p r o p o r t i o n of
in e a r l y v i e w s . It is t h u s p o s s i b l e to highlight osteoid and fibrovascular tissue m a y vary. T h e
s m a l l l e s i o n s w i t h o u t t y p i c a l a p p e a r a n c e o n CT, lesion is s u r r o u n d e d b y sclerotic r e a c t i v e b o n e for-
e.g. without reactive sclerosis, a s m a y be the case m a t i o n ; t h e intensity of this reaction a l s o v a r i e s
in i n t r a m e d u l l a r y t u m o r s . It m a y a l s o h e l p in dif- d e p e n d i n g o n t h e localization of t h e t u m o r (corti-
ferentiating osteoid o s t e o m a f r o m other hypo- c a l , m e d u l l a r y , p e r i o s t e a l , intra-articular).
vascular lesions with similar radiologic and CT
appearance (e.g. Brodi's abscess, bone cyst).
D y n a m i c C T with contrast e n h a n c e m e n t might
a l s o b e u s e d , but it e x p o s e s t h e s e y o u n g p a t i e n t s Treatment
to a n i n c r e a s e d r a d i a t i o n d o s e .
Medical treatment
O s t e o i d o s t e o m a is a t u m o r w i t h no malignant
Other imaging studies p o t e n t i a l ; it c o m m o n l y p r o v o k e s p a i n interfering
w i t h n i g h t s l e e p , w h i c h is g e n e r a l l y r e s p o n s i v e
A r t e r i o g r a p h y s h o w s b l o o d s t a g n a t i o n in arterial to NSAIDs, particularly acetylsalicylate. Other
a n d v e n o u s p h a s e ( A l l i e u a n d L u s s i e z 1988). It N S A I D s h a v e been tried, with similarly favorable
thus highlights the hypervascular character of r e s u l t s in t e r m s of p a i n relief ( B o t t n e r et al 2001).
62 T U M O R S OF THE HAND

This might be explained by the tumor's expression recur. B y c l a s s i c s u r g i c a l m e t h o d s , t h e n i d u s c a n

of t h e e n z y m e c y c l o o x y g e n a s e - 2 (Cox-2) ( B o t t n e r be r e m o v e d either by curettage or by w i d e exci-
et a l 2 0 0 1 , K a w a g u c h i et a l 2001) a n d h e n c e s i o n ('en b l o c ' ) . C u r e t t a g e a i m s a t e x c i s i o n of
prostaglandin secretion. Existing reports of only the nidus, w h e r e a s by 'en b l o c ' excision, the
r e m i s s i o n after a p e r i o d of c o n s e r v a t i v e treat- l e s i o n is r e m o v e d w i t h h e a l t h y m a r g i n s , w i t h o u t
m e n t i m p l y t h a t 2 t o o c c a s i o n a l l y 20 y e a r s m i g h t t o u c h i n g t h e n i d u s itself. H o w e v e r , w i t h t h e for-
p a s s b e f o r e full s y m p t o m relief ( G o l d i n g 1954, m e r m e t h o d difficulty in a c c u r a t e l y localizing t h e
Vickers et al 1959, Kneisl and Simon 1992). tumor during excision m a y be experienced, a s
W h e t h e r u s e of N S A I D s a c c e l e r a t e s h e a l i n g is t h e l e s i o n t o b e e x c i s e d is r o u t i n e l y of s m a l l size
not k n o w n , a l t h o u g h t h e r e is s o m e e v i d e n c e s u g - ( d i a m e t e r in a r a n g e of s o m e m i l l i m e t e r s ) ; w i t h
gesting this m a y be the c a s e (Kneisl a n d S i m o n t h e latter, t h e size of cortical p i e c e t o b e r e m o v e d
1992). O n t h e o t h e r h a n d , t h i s t u m o r is a l w a y s of is difficult t o d e c i d e a n d f e n e s t r a t i o n l e a d s to a
small size a n d intraoperative localization has v i o l a t i o n of cortical integrity, p o s s i b l y rendering
a l w a y s b e e n a difficulty w h i c h s h o u l d b e c o n s i - the host b o n e fragile.
dered seriously. Nonetheless, some relatively
i n a c c e s s i b l e l o c a l i z a t i o n s of t h e t u m o r ( e . g . in t h e
spine or the pelvis) m a y be fraught with technical
difficulties if s u r g i c a l t r e a t m e n t is c h o s e n , w h i l e
Minimally invasive surgical treatment
others (like in t h e femur a n d tibia) implicate
w e i g h t - b e a r i n g b o n e s to w h i c h a n e x c i s i o n m a y In a n a t t e m p t t o m e e t t h e s e t e c h n i c a l c h a l l e n g e s
p r o v o k e m e c h a n i c a l d e t e r i o r a t i o n a n d t h e risk of and diminish surgical morbidity, progress has
post-surgical fracture. Self-healing potential a n d r e c e n t l y b e e n m a d e in t w o w a y s :
morbidity of surgical treatment might thus
prompt simple observation or medical m a n a g e -
(1) T h e t u m o r m a y b e identified intraoperatively
m e n t ( K n e i s l a n d S i m o n 1992); t h i s w o u l d a l s o b e
u s i n g C T s c a n ( M a r c o v e e t al 1 9 9 1 , K o h l e r
of particular interest in the case of painless
et al 1995, R o g e r e t al 1996, S a n s e t al 1999),
osteoid osteomas. Conservative treatment might
o r a g a m m a - c o u n t e r ( S t y a n d S i m o n s 1982)
b e c h o s e n in t h e a b o v e settings provided that: (1)
(detection of r a d i o l a b e l e d t e c h n e t i u m injected
t h e d i a g n o s i s h a s b e e n m a d e w i t h certainty by
p r e o p e r a t i v e y ) , or with preoperative a d m i n -
i m a g i n g s t u d i e s ; (2) n o a p p a r e n t d e f o r m a t i o n h a s
istration of i n t r a v e n o u s t e t r a c y c l i n e ( A y a l a
b e e n p r o v o k e d b y t h e t u m o r ; (3) w h e n pain is pro-
et al 1986) ( r e n d e r i n g t h e t u m o r fluorescent
v o k e d b y t h e t u m o r , it r e s p o n d s w e l l to N S A I D s a n d
u n d e r direct v i s i o n ) .
is w e l l t o l e r a t e d b y t h e patient for long p e r i o d s ; a n d
(2) Excision can take place using s o m e mini-
(4) w h e n N S A I D s a r e a d m i n i s t e r e d , c l o s e monitor-
m a l l y i n v a s i v e m e t h o d s like drilling ( K o h l e r
ing of t h e patient for side-effects is possible. In
et al 1995, S a n s e t al 1999), a b l a t i o n b y a
practice, this is rarely t h e c a s e in h a n d o s t e o i d
t r o c a r a n d a b i o p s y n e e d l e ( R o g e r et al 1996),
o s t e o m a s , w h i c h a r e superficial e n o u g h to b e e a s -
thermal necrosis via a radiofrequency elec-
ily a c c e s s i b l e a n d frequently p r o v o k e c h a n g e s in
t r o d e ( R o s e n t h a l et al 1992, D e B e r g et al
the hand's appearance, e v e n w h e n they are pain-
1995, R o s e n t h a l et al 1995, 1998), o r photo-
less ( R e x et al 1997, B a s u et al 1999). A n o t h e r indi-
c o a g u l a t i o n of t h e n i d u s b y l a s e r ( G a n g i e t al
cation for conservative treatment might be a
1997), all these techniques being applied
r e c u r r e n c e after excision ( A t h a n a s i a n 1999).
percutaneously.

N o w a d a y s , t h e r e is a t r e n d t o w a r d s CT-assisted
percutaneous techniques, which c o m b i n e assisted
Classic surgical treatment i n t r a o p e r a t i v e identification of t h e n i d u s a n d m i n i -
m a l l y i n v a s i v e i n t r a l e s i o n a l e x c i s i o n w i t h o n e of
E x c i s i o n of o s t e o i d o s t e o m a is d i r e c t e d t o w a r d s t h e m e t h o d s m e n t i o n e d a b o v e . In l a r g e b o n e s or
a b l a t i o n of t h e p r i m a r y p a t h o l o g i c f o c u s of t h e difficult t o a c c e s s skeletal localizations, t h e y offer
t u m o r , i.e. t h e n i d u s . It is a c c e p t e d w o r l d w i d e t h a t s u b s t a n t i a l b e n e f i t s , but t h e y h a v e a l s o o c c a s i o n -
if t h e n i d u s h a s b e e n i n c o m p l e t e l y e x c i s e d o r ally b e e n a p p l i e d t o h a n d o s t e o i d o s t e o m a s ( D e
destroyed, lesion and s y m p t o m s will eventually B e r g et al 1995, G a n g i e t al 1997, V a n d e r s c h u e r e n
 OSTEOID O S T E O M A OF THE HAND AND W R I S T 63

et al 2002). T h e y c a n n o t b e a p p l i e d to c a s e s w i t h
u n c e r t a i n d i a g n o s i s a n d t h e y require u s e of e x p e n -
Report on a personal series
sive material, a s well a s assuring operating theater W e w i l l n o w report o n o u r s e r i e s of 25 o s t e o i d
c o n d i t i o n s in a n i m a g i n g r o o m . W i t h a n y p o s s i b l e o s t e o m a s l o c a t e d in t h e h a n d a n d w r i s t .
artifice used, they cannot consistently ensure
g o o d - q u a l i t y histological m a t e r i a l for e x a m i n a t i o n .
Histological confirmation thus obtained may
remain at moderate levels (40-70 per cent) Patients and methods
( R o s e n t h a l et al 1995, 1998, V a n d e r s c h u e r e n et al
2002) for clinical cases fully compatible with This w a s a retrospective study undertaken on
o s t e o i d o s t e o m a . T h e i r p l a c e in r e m o v a l of h a n d consecutive p a t i e n t s . T h e r e w e r e 12 m e n and
o s t e o i d o s t e o m a s is a matter of d i s c u s s i o n . 13 w o m e n . T h e right h a n d w a s m o r e frequently
T h e e x a c t risk of r e c u r r e n c e after e x c i s i o n o f i m p l i c a t e d (16 c a s e s ) . M e a n a g e w a s 31.5 y e a r s
hand osteoid o s t e o m a s is difficult t o predict. (range 12-68). Mean delay between onset of
Published s e r i e s differ a s to the number of s y m p t o m s a n d i n d e x o p e r a t i o n w a s 23.1 m o n t h s
p a t i e n t s i n c l u d e d , t h e c e r t a i n t y of d i a g n o s i s , t h e ( r a n g e 3-120). F o u r p a t i e n t s (16 p e r c e n t ) h a d
t e c h n i q u e or c o m b i n a t i o n of t e c h n i q u e s used, already been operated upon o n c e or m o r e than
t h e duration of post-excision follow-up, a n d e v e n o n c e , w i t h o u t c u r e a n d in t w o of t h e m n o d i a g n o -
t h e b o d y d i s t r i b u t i o n of t u m o r s e x c i s e d . In t h e s i s h a d b e e n o b t a i n e d b y t h e t i m e of c o n s u l t a t i o n
s e r i e s of A m b r o s i a et al (1987), i n c l u d i n g only for t h i s s t u d y . P a t i e n t d e m o g r a p h i c s a r e s h o w n
hand and wrist osteoid osteomas, recurrence in F i g . 8.
after t h e i n d e x o p e r a t i o n r e a c h e d 25 p e r c e n t . P a i n w a s p r e s e n t in all c a s e s (100 p e r c e n t ) a s
In t h e s e r i e s of B e d n a r et al (1993), c o m p r i s i n g t h e principal s y m p t o m , b e i n g w o r s e d u r i n g n i g h t
u p p e r e x t r e m i t y o s t e o i d o s t e o m a s , s u r g i c a l treat- s l e e p . T w e l v e of t h e p a t i e n t s h a d t a k e n N S A I D s to
m e n t r e s u l t e d in r e c u r r e n c e in 13 p e r c e n t of r e l i e v e p a i n ( a s p i r i n in n i n e of t h e m ) w i t h g o o d
s u b j e c t s (to b e n o t e d , f i v e o f t h e six r e c u r r e n c e s results. T h e s e c o n d m o s t frequent s y m p t o m w a s
o c c u r r e d in t u m o r s l o c a t e d in t h e h a n d ) . In t h e local swelling: eleven patients (44 per cent)
s e r i e s of C a m p a n a c c i et al (1999) ( d i r e c t v i s u a l - c o m p l a i n e d of s o m e i n c r e a s e in v o l u m e of soft
ization a n d intralesional excision, without any tissues overlying the painful area. T w o patients
intraoperative imaging assistance), primary cure (8 p e r c e n t ) in t h i s s e r i e s w e r e a w a r e of s o m e l o s s
r a t e s r e a c h e d 100 p e r c e n t ; in t h i s s e r i e s , t h e r e in r a n g e of m o t i o n in p r o x i m a l interphalangeal
w a s o n l y o n e t u m o r l o c a t e d in t h e h a n d a n d t h e (PIP) joints.
t e c h n i q u e d e s c r i b e d is m o r e s u i t a b l e for long Patient examination r e v e a l e d that pain w a s
bone osteoid osteomas. triggered or aggravated by palpation in all
M o r e o v e r , t h e i m p a c t of u s e of i n t r a o p e r a t i v e p a t i e n t s . A l o c a l t e n d e r n e s s , p r e c i s e l y c o n f i n e d to
imaging modalities and minimally invasive tech- a c i r c u m s c r i b e d a r e a , w a s m o r e p r o n o u n c e d in
n i q u e s in t h e f a i l u r e a n d r e c u r r e n c e r a t e i s e v e n f i n g e r localization. In c o n t r a s t , t u m o r s l o c a t e d in
h a r d e r t o a s s e s s . R a t e s a s l o w a s 5.5 p e r c e n t the wrist g a v e a s o m e w h a t dull, aching pain,
t o a s h i g h a s 24 p e r c e n t h a v e b e e n reported w h i c h w a s r a t h e r difficult to l o c a t e w i t h a c c u r a c y ,
( D e B e r g e t a l 1995, R o g e r e t a l 1996, G a n g i e t a l especially for palmarly situated tumors. For
1997, R o s e n t h a l et al 1998, Sans et al 1999, t u m o r s in t h e p h a l a n g e s , clinical e v a l u a t i o n a d d i -
V a n d e r s c h u e r e n e t al 2002). T h e e f f i c a c y of e a c h t i o n a l l y r e v e a l e d a f u s i f o r m e n l a r g e m e n t of soft
method may vary depending not o n l y o n the t i s s u e e n v e l o p i n g t h e b o n e ( F i g . 5). In t h e distal
e x p e r i e n c e of t h e m e d i c a l t e a m a n d t h e l o c a t i o n phalanx this m a c r o d a c t y l y w a s a c c o m p a n i e d by
of t h e t u m o r , but a l s o o n p a r a m e t e r s of the nail h y p e r t r o p h y a n d c l u b b i n g ( F i g . 3 ) .
technique u s e d (such a s t h e total a m o u n t of T h e f o l l o w i n g i m a g i n g s t u d i e s w e r e u s e d to
e n e r g y d e l i v e r e d to the t u m o r ( V a n d e r s c h u e r e n help diagnosis and/or operative planning: plain
et a l 2 0 0 2 ) ) . it s e e m s t h a t a r e p e a t p r o c e d u r e X-rays, three-phase T c 9 9 m
bone scan, simple
using the same percutaneous technique may t o m o g r a p h i e s , CT, M R I , a n d arteriography. S i m p l e
l e s s e n t h e f a i l u r e r a t e ( G a n g i e t al 1997, D e B e r g X - r a y control w a s u n d e r t a k e n in all p a t i e n t s ; s i n g l e
et a l 1995, R o s e n t h a l et al 1995, V a n d e r s c h u e r e n or c o m b i n e d w i t h b o n e s c a n (in 64 per c e n t of
et al 2002). p a t i e n t s ) , it e s t a b l i s h e d t h e d i a g n o s i s of o s t e o i d
64 T U M O R S OF THE HAND

12 Males

13 Females

9 Left hands

16 Right hands . . .
Age (in decade increments)

Figure 8

Patient demographics. Histogram on the right shows case distribution according to age. There were no patients in the 1st
and 6th decades of life.

o s t e o m a in 89 per c e n t of patients in this s e r i e s . c a r p a l b o n e s w a s e a s e d b y u s e of hypodermic

Bone scans have always demonstrated intense injection n e e d l e s to h i g h l i g h t i n t r a c a r p a l a r t i c u l ar
r a d i o n u c l i d e f i x a t i o n in c i r c u m s c r i b e d locations, lines a n d locate the bone hosting the lesion,
d u r i n g all p h a s e s of t h e i m a g i n g study. A C T s c a n w i t h o u t m u c h d i s r u p t i n g soft t i s s u e a t t a c h m e n t s .
w a s p e r f o r m e d in 60 p e r c e n t of p a t i e n t s a n d w a s I n t r a o p e r a t i v e i m a g e intensifier v i e w s w e r e t a k e n
v e r y helpful in p r e c i s e l y locating t h e t u m o r . M R I w i t h t h e n e e d l e s in p l a c e , to v e r i f y p o s i t i o n . T w o
w a s d o n e in six c a s e s a n d s u b s t a n t i a l l y h e l p e d in t y p e s of e x c i s i o n w e r e u s e d : ' e n b l o c ' excision
d i a g n o s i s in o n e , in c o m b i n a t i o n w i t h t h e patient's w a s p e r f o r m e d in 21 p a t i e n t s (84 p e r c e n t ) a n d
clinical profile. Finally, artériographie s t u d i e s w e r e i n t r a l e s i o n a l c u r e t t a g e in four. ' E n b l o c ' e x c i s i o n
performed in t h r e e patients and showed non- w a s p r e f e r r e d if l o c a l c o n d i t i o n s (size of t u m o r ,
s p e c i f i c h y p e r v a s c u l a r i z a t i o n a n d b l o o d p o o l i n g in size of b o n e h o s t i n g t h e t u m o r ) w e r e f a v o r a b l e .
a r e a s c o i n c i d i n g w i t h t h e t u m o r ' s location ( F i g . 9). W i t h t h i s t e c h n i q u e , a cortical o r c o r t i c o c a n c e l -
O s t e o i d o s t e o m a s of t h e w r i s t a n d h a n d t h u s l o u s ( d e p e n d i n g o n t h e l o c a t i o n of t h e tumor)
diagnosed were anatomically distributed as b o n e block w a s e x c i s e d , c o n t a i n i n g t h e nidus.
f o l l o w s : t e n t u m o r s w e r e l o c a t e d in t h e w r i s t a r e a In 20 c a s e s (80 p e r c e n t ) a b o n e graft w a s u s e d ,
( F i g s . 1 , 2 , 1 0 , a n d 11), t h r e e t u m o r s w e r e f o u n d in either corticocancellous (Figs. 13 and 14)
t h e m e t a c a r p a l b o n e s ( F i g s . 6 a n d 12), a n d t w e l v e (15 c a s e s ) o r c a n c e l l o u s ( F i g . 12) ( f i v e c a s e s ) . T h e
t u m o r s w e r e s i t u a t e d in t h e p h a l a n g e s (four in d e c i s i o n o n b o n e g r a f t i n g w a s t a k e n in relation
distal a n d e i g h t in p r o x i m a l p h a l a n g e s ( F i g s . 3 - 5 , to m e c h a n i c a l c o n d i t i o n s o f t h e h o s t b o n e after
a n d 13-15). F r o m t h e t u m o r s s i t u a t e d in t h e w r i s t , e x c i s i o n : in g e n e r a l , f o r some tumors located
o n e w a s l o c a t e d in t h e distal radial e p i p h y s i s , o n e superficially into b o n e o r s u b p e r i o s t e a l y , bone
in t h e u l n a r s t y l o i d , f o u r in t h e l u n a t e , o n e in t h e g r a f t i n g w a s not u n d e r t a k e n ( F i g . 4 ) . T h e graft w a s
c a p i t a t e , o n e in t h e h a m a t e , a n d t w o in t h e t r a p e - impacted into the cavity left behind by the
zoid b o n e s . e x c i s e d t u m o r . C a n c e l l o u s b o n e graft w a s u s e d
A l l p a t i e n t s w e r e o p e r a t e d b y t h e s e n i o r of u s to try to a c c e l e r a t e filling of t h e c a v i t y b y b o n e
( D . L . V . ) . S u r g i c a l a p p r o a c h to t u m o r s s i t u a t e d in formation; corticocancellous bone w a s thought
 OSTEOID O S T E O M A O F T H E HAND AND W R I S T 65

Figure 9

Arteriography of the upper limb in a

symptomatic patient with normal
X-rays. Note progressive appear-
ance of a hypervascular lesion in
successive views (arrow).

a l s o to e n s u r e s o m e p r i m a r y m e c h a n i c a l solidity
to h o s t b o n e . In t h r e e c a s e s internal f i x a t i o n w a s
p e r f o r m e d to a v o i d a n i m m i n e n t f r a c t u r e after t h e
e x c i s i o n of t h e t u m o r ( F i g . 13). S i m p l e X - r a y c o n -
trol of t h e b o n e b l o c e x c i s e d w a s u n d e r t a k e n after
'en b l o c ' e x c i s i o n s , to c o n f i r m nidus excision.
Additionally, a histological examination of the
excised tissue w a s routinely performed in all
c a s e s . F i n a l l y , all o p e r a t e d p a t i e n t s w e r e f o l l o w e d
u p for a n a v e r a g e 43.5 m o n t h s ( r a n g e 12-124)
e x c e p t four, w h o w e r e lost b e f o r e c o m p l e t i o n of
1 year follow-up.

Results
C o n c e r n i n g t h e f o u r p a t i e n t s lost to f o l l o w - u p , at
their last e v a l u a t i o n n o s i g n of r e c u r r e n c e of t h e
t u m o r w a s f o u n d . In t h e o t h e r 21 p a t i e n t s , t h e
results w e r e a s f o l l o w s : in p a t i e n t s t r e a t e d w i t h
'en bloc' excision, histological examination
always confirmed preoperative diagnosis. This
w a s not p o s s i b l e , t h o u g h , for all f o u r patients
Figure 10 treated with curettage, w h e r e a nidus w a s found
Same patient as in Fig. 2. 'En bloc' excision of the tumor. only twice. Seventeen patients were totally
Ligamentous elements were stabilized on the ulna with the s y m p t o m - f r e e at last f o l l o w - u p e x a m i n a t i o n , w i t h
help of a bone anchor. n o s i g n s of r e c u r r e n c e . P a i n w i t h t h e characteristic
66 T U M O R S OF THE HAND

Figure 11
Same patient as in Figs. 2 and 10. Final postoperative result. Mobility is normal.

nocturnal flare had disappeared immediately c a v i t y e m b e d d e d in c a n c e l l o u s b o n e . It w a s

postoperation, leaving s o m e rapidly declining t h e n b o n e - g r a f t e d , r e s u l t i n g in r e m i s s i o n of t h e
n o n - s p e c i f i c p o s t o p e r a t i v e p a i n of s o m e w e e k s ' patient's s y m p t o m s after s u r g e r y . M a t e r i a l
duration. In f o u r patients persistent pain or e x c i s e d f r o m t h e w a l l s of t h e l e s i o n w a s t u m o r -
limited r a n g e of m o t i o n w a s n o t e d in t h e o p e r a t e d f r e e . F i n a l l y , in o n e p a t i e n t p a i n a n d d e c r e a s e d
a r e a , at f i n a l f o l l o w - u p visit. P a i n d i d not h a v e t h e r a n g e of m o t i o n p e r s i s t e d in t h e i n t e r p h a l a n g e a l
c h a r a c t e r i s t i c s of t h e p r e o p e r a t i v e p a i n (intensity, j o i n t of t h e t h u m b , after e x c i s i o n of a n o s t e o i d
nocturnal aggravation) a n d no nidus has ever o s t e o m a s i t u a t e d in t h e b a s e of t h u m b ' s distal
b e e n v i s i b l e o n X - r a y s p o s t o p e r a t i v e l y . In o n e p h a l a n x . B e c a u s e t h e r a d i o l o g i c i m a g e of t h i s
p a t i e n t l i m i t a t i o n in r a n g e of m o t i o n p e r s i s t e d in joint w a s c o m p a t i b l e w i t h a d v a n c e d d e g e n e r a t i v e
t h e m e t a c a r p o p h a l a n g e a l joint of t h e t h u m b , after arthritis, a n a r t h r o d e s is w a s p e r f o r m e d a n d
e x c i s i o n of a n o s t e o i d o s t e o m a s i t u a t e d in t h e p r o v e d to b e e f f i c a c i o u s in relieving patient's
h e a d of t h e first m e t a c a r p a l . In a n o t h e r , w i t h a symptoms.
t u m o r s i t u a t e d in t h e l u n a t e b o n e a n d e x c i s e d A r e m a r k c a n b e m a d e c o n c e r n i n g r e c o v e r y of
without b o n e grafting, a radiolucent area w a s n o r m a l e x t e r n a l a p p e a r a n c e after e x c i s i o n in
f o u n d in s i m p l e p o s t o p e r a t i v e X - r a y s . T h e l e s i o n c a s e s w i t h p r e o p e r a t i v e d e f o r m a t i o n , a s in digita l
persisted throughout follow-up and w a s thought localizations of t h e t u m o r . E s t h e t i c i m p a i r m e n t
to b e t h e s o u r c e of t h e minor complaints in t a k i n g t h e f o r m of m a c r o d a c t y l y a n d / o r nail
t h i s p a t i e n t . It w a s e x p l o r e d 14 m o n t h s p o s t o p e r - clubbing took t i m e to regress a n d signs w e r e
atively and w a s found to be a liquid-filled p r e s e n t f o r a l o n g t i m e after e x c i s i o n ( F i g . 15).
 OSTEOID O S T E O M A OF THE HAND AND W R I S T 67

a b

Figure 12
Osteoid osteoma in metacarpal neck. Curettage (a) followed by cancellous bone grafting (b). Arrowheads show cavity
created by excision.

Discussion o s t e o m a s in t h e h a n d ( A m b r o s i a et al 1987,
A l l i e u a n d L u s s i e z 1988, B e d n a r et a l 1993,
T h e present series shares s o m e c o m m o n points M a r c u z z i et al 2002).
w i t h other series published to date: (4) T h e t u m o r s h o w e d s o m e predilection for
c a r p a l b o n e s a n d p h a l a n g e s , a n d w a s infre-
(1) Patients w e r e predominantly of y o u n g a g e q u e n t in m e t a c a r p a l s . In t h e f i n g e r s , p r o x i m a l
a n d m o s t l y in t h e i r s e c o n d t o f o u r t h d e c a d e o r distal phalanges w e r e implicated a s is
of life. g l o b a l l y d e s c r i b e d ( A m b r o s i a et a l 1987,
(2) D e l a y b e t w e e n o n s e t of s y m p t o m s a n d o p e r - A l l i e u a n d L u s s i e z 1988, B e d n a r et a l 1993,
ation (reflecting t h e difficulty in m a k i n g a M a r c u z z i et al 2002). W e d i d not f i n d a n y
d i a g n o s i s ) w a s particularly e l o n g a t e d , a s is middle phalanx osteoid osteoma.
often the case in other published work
( A m b r o s i a et al 1987, A l l i e u a n d L u s s i e z 1988, H o w e v e r , s o m e differences with w o r l d literature
B e d n a r et al 1993, M a r c u z z i et al 2002). w e r e noted:
(3) P a i n w a s t h e m o s t f r e q u e n t s y m p t o m in t h i s
s e r i e s , f o l l o w e d b y s w e l l i n g a n d d e c r e a s e in (1) F e m a l e s e x s l i g h t l y p r e v a i l e d in o u r p a t i e n t s .
r a n g e of m o t i o n in a d j a c e n t j o i n t s , a s w a s (2) Pain w a s omnipresent in this series. In
generally found in o t h e r s e r i e s of osteoid contrast, painless osteoid osteomas have
68 T U M O R S OF THE HAND

a b c

Figure 13

Osteoid osteoma in proximal phalanx of small finger, exposed by dorsal approach (a). 'En bloc' excision (excised piece of
bone is marked with stitch) results in structurally important bone loss (b) (arrowheads). Corticocancellous bone grafting
(asterisk) and plate and screw fixation (arrows) ensure stability and bone healing (c).

b e e n d e s c r i b e d in t h e literature, particularly Moreover, the patient routinely presents local

concerning the phalanges and m a y represent swelling and deformation. The latter is more
up to 11 p e r c e n t of all h a n d localizations p r o m i n e n t in l o n g - s t a n d i n g c a s e s a n d t a k e s t h e
( A l l i e u a n d L u s s i e z 1988). f o r m of m a c r o d a c t y l y in t h e digits. Additionally,
(3) Pain w a s a l w a y s alleviated by N S A I D s , w h e n distal p h a l a n x i n v o l v e m e n t p r o v o k e s nail c l u b -
u s e d , w h e r e a s in p u b l i s h e d s e r i e s of hand b i n g . In t h i s p a t i e n t g r o u p , d i a g n o s i s is e a s e d b y
osteoid o s t e o m a s , pain is n o t universally c o m b i n i n g clinical evaluation a n d s i m p l e X - r a y s ,
responsive to NSAIDs (Allieu a n d Lussiez a n d f u r t h e r i m a g i n g s t u d i e s m a y not b e n e c e s -
1988, B e d n a r et al 1993). sary. H o w e v e r , special attention m u s t be paid to
e a r l y p r e s e n t a t i o n s (in t h e first 6 - 1 2 m o n t h s f r o m
Recapitulating the clinical setting of osteoid o n s e t of s y m p t o m s ) , w h e r e t h e n i d u s m a y not y e t
o s t e o m a s in t h e h a n d , w e w e r e a b l e to distin- be discernible.
guish t w o groups, depending on the difficulty T h e s e c o n d g r o u p w a s of osteoid osteomas
m e t in r e a c h i n g t h e c o r r e c t d i a g n o s i s . situated in carpal bones, multi-operated osteoid
T h e first g r o u p w a s of osteoid osteomas situ- osteomas, or osteoid osteomas provoking vivid
ated in phalanges or metacarpals. In s u c h c a s e s osseous reaction.These cases share an increased
t h e t u m o r is r a t h e r e a s i l y identified in s i m p l e difficulty in making the diagnosis by simple
X - r a y s a s a r a d i o l u c e n t l e s i o n in s c l e r o t i c b o n e . radiographs, b e c a u s e of (correspondingly):
 OSTEOID OSTEOMA OF THE HAND AND W R I S T 69

a b c d

Figure 14

Osteoid osteoma of proximal phalanx. Dorsolateral approach (a), excision and corticocancellous bone grafting (b) (arrows).
(c) and (d) Graft incorporation at 1-year follow-up.

(1 ) T h e size a n d l o c a t i o n of t h e b o n e h o s t i n g t h e rather c o m p a t i b l e w i t h this t u m o r , but t h e a b s e n c e

tumor. Carpal bones are small and frequently of pain or of its particular character a s d e s c r i b e d
o v e r l a p p i n g in s o m e X - r a y v i e w s . O n t h e other a b o v e d o e s not e x c l u d e t h e t u m o r . After e x a m i n a -
h a n d , m u c h of t h e i r s u r f a c e is intra-articular tion of t h e patient w i t h special attention to local t e n -
a n d h e n c e not c o v e r e d b y p e r i o s t e u m , s o derness, swelling, and deformation, the first
that reactive b o n e formation may not be i m a g i n g s t u d y to order is s i m p l e X - r a y s . C o m b i n i n g
vivid. clinical setting a n d X - r a y f i n d i n g s m a y s o m e t i m e s
(2) T h e changes a previous operation m a y have b e sufficient to m a k e t h e d i a g n o s i s a n d e v e n to
b r o u g h t t o t h e site of t h e l e s i o n . plan t h e surgical intervention. If X - r a y results a r e
(3) A n intensive bone condensation around the negative or inconclusive, a three-phase bone
n i d u s , w h i c h m a y h i d e it. scintigraphy c a n follow. A negative bone scan
almost certainly excludes osteoid osteoma,
U n d e r these circumstances additional imaging although cases have been published sporadically
studies m a y be used. w i t h n e g a t i v e b o n e s c a n s a n d histologically p r o v e n
A d i a g n o s t i c a l g o r i t h m c o u l d b e e s t a b l i s h e d for osteoid o s t e o m a s ( L e r o y et al 1980, F e h r i n g a n d
u s e in a clinical setting m o r e or less s u g g e s t i v e of a G r e e n 1984). O n t h e other h a n d , b o n e s c a n is n e v e r
h a n d or w r i s t o s t e o i d o s t e o m a ( F i g . 16). First, pain highly a c c u r a t e a s to t h e location of t h e d e t e c t e d
w i t h nocturnal a g g r a v a t i o n a n d relief by aspirin is lesion, not to m e n t i o n that a n i n c r e a s e d uptake in
70 T U M O R S OF THE HAND

c d

Figure 15

Same patient as in Fig. 3. Lateral approach (a). The tumor is excised 'en bloc' (arrow shows excised bone block, arrowheads
show the cavity left) (b). (c) and (d) Result at 3.5 months postoperation.
 OSTEOID O S T E O M A OF THE HAND AND W R I S T 71

Simple X-ray control of y o u n g p a t i e n t s . D i a g n o s i s in e a r l y s t a g e s is

difficult b e c a u s e s y m p t o m s a n d s i g n s m a y s t a y
$$
o c c u l t , a n d c h a n g e s in i m a g i n g s t u d i e s m a y not
Negative X-rays or no a p p e a r b e f o r e m o n t h s or y e a r s f r o m symptom
typical nidus present o n s e t . A h i g h i n d e x of s u s p i c i o n m u s t b e kept for
y o u n g p a t i e n t s , e v e n if s y m p t o m s a r e not t h o s e
T c " bone scan
m
typically described for this tumor. Osteoid osteo-
m a s l o c a t e d in t h e w r i s t , m u l t i - o p e r a t e d o n e s , o r
Typical nidus ' b u r i e d ' in i n t e n s e r e a c t i v e cortical s c l e r o s i s m a y
present present an important difficulty in d i a g n o s i s a n d
Almost excludes
osteoid osteoma n e c e s s i t a t e t h e u s e of a d v a n c e d i m a g i n g t e c h -
n i q u e s , s u c h a s CT. O n t h e o t h e r h a n d , l o c a t i o n of
CT scan the tumor in t h e f i n g e r s frequently produces

Diagnosis of osteoid osteoma p r o n o u n c e d a n d t y p i c a l local c h a n g e s , a n d t h u s

confirmed s i m p l e r a d i o g r a p h s a n d b o n e s c a n m a y suffice
No typical nidus
t o m a k e t h e d i a g n o s i s . S h o u l d d i a g n o s i s not b e
T
e s t a b l i s h e d w i t h u s e of s i m p l e radiographs, a
Dynamic MRI with t h r e e - p h a s e b o n e s c a n a n d CT, M R I , or d y n a m i c
gadolinium enhancement
MRI-scan with gadolinium enhancement may
Early No provide further help.
enhancement enhancement A s h a n d o s t e o i d o s t e o m a s a r e m o r e or l e s s
a c c e s s i b l e f r o m a s u r g i c a l p o i n t of v i e w a n d d o
Figure 16
not implicate weight-bearing bones, minimally
Diagram helping in the diagnostic process of suspected i n v a s i v e s u r g i c a l t e c h n i q u e s w e r e not u s e d in t h i s
osteoid osteoma. Note that standard X-rays are indispens- series. Instead, 'en bloc' excision or curettage w a s
able. A computed tomography (CT) scan may be needed for u n d e r t a k e n , w i t h u s e of b o n e g r a f t i n g , if t h i s w a s
operative planning, even when diagnosis has been confirmed felt t o b e n e c e s s a r y .
otherwise. MRI, magnetic resonance imaging.

b o n e s c a n m a y s o m e t i m e s b e diffuse. Targeted
thin-cut C T s c a n greatly i n c r e a s e s spatial informa-
t i o n o b t a i n e d by s i m p l e X - r a y s a n d b o n e s c a n , a n d
References
s h o u l d b e t h e next i m a g i n g s t u d y of c h o i c e . It AllieuY, Lussiez B (1988) L'ostéome ostéoïde au niveau
p r o v i d e s v a l u a b l e a s s i s t a n c e in d i a g n o s i s a n d / o r de la main. A propos de quarante-six cas, Ann Chir Main
preoperative planning, although other lesions, 7:298-304.
including b o n e infection a n d s i m p l e b o n e c y s t s ,
m a y p r o d u c e similar f i n d i n g s o n C T D y n a m i c C T Allieu Y, Lussiez B, Benichou M , Cenac P (1989) A
w i t h e n h a n c e m e n t using i n t r a v e n o u s contrast m a y double nidus osteoid osteoma in a finger, J Hand Surg
h e l p differentiate t h e s e entities, but it e x p o s e s t h e Am 14:538-41.
patient to higher radiation d o s e s . In borderline
cases, dynamic M R I m a y be used, combined with Ambrosia J M , Wold L E , Amadio PC (1987) Osteoid
e n h a n c e m e n t t e c h n i q u e s , t h u s taking a d v a n t a g e of osteoma of the hand and wrist, J Hand Surg Am
t h e h y p e r v a s c u l a r i t y of this t u m o r . O t h e r diagnostic 12:794-800.
t o o l s s u c h a s s i m p l e t o m o g r a p h y o r arteriography
a r e less f r e q u e n t l y u s e d n o w a d a y s . Assoun J , Richardi G , Railhac J J et al (1994) Osteoid
osteoma: M R imaging versus CT, Radiology 191:
217-23.

Conclusion Athanasian E A (1999) Bone and soft tissue tumors. In:

Green DP, Hotchkiss R N , Pederson W C , eds. Green's
O s t e o i d o s t e o m a s in w r i s t a n d h a n d a r e r e l a t i v e l y Operative Hand Surgery, 4th edn. Churchill Livingstone:
rare b e n i g n t u m o r s affecting t h e q u a l i t y of life Philadelphia 2:2223-53.
72 T U M O R S O F T H E HAND

Ayala A G , Murray J A , Erling M A , R a y m o n d A K FehringTK, Green NE (1984) Negative radionuclide scan

(1986) Osteoid osteoma: intraoperative, tetracycline- in osteoid osteoma. A case report, Clin Orthop
fluorescence demonstration of the nidus, J Bone Joint 185:245-9.
Surg Am 86:747-51.
Gangi A, Dietemann J L , Gasser B et al (1997) Interstitial
Basu S , Basu P, Dowell J K (1999) Painless osteoid laser photocoagulation of osteoid osteomas with use of
osteoma in a metacarpal, J Hand Surg Br 24:133-4. CT guidance, Radiology 203:843-8.

Bednar M S , McCormack J r R R , Glasser D, Weiland A J Golding J S R (1954) The natural history of osteoid
(1993) Osteoid osteoma of the upper extremity, J Hand osteoma with a report of twenty cases, J Bone Joint
Surg Am 18:1019-25. Surg Br 36:218-29.

Bednar M S , W e i l a n d A J , Light TR (1995) Osteoid Hawkes D J , Robinson L, Crossman J E et al (1991)

osteoma of the upper extremity, Hand Clin 11:211-21. Registration and display of the combined bone scan
and radiograph in the diagnosis and management of
Bottner F, Roedl R, Wortler K et al (2001) wrist injuries, Eur J Nucl Med 18:752-6.
Cyclooxygenase-2 inhibitor for pain management in
osteoid osteoma, Clin Orthop 393:258-63. Herndon J H , Eaton R G , Littler J W (1974) Carpal tunnel
syndrome: an unusual presentation of osteoid osteoma
B o w e n CVA, Dzus A K , Hardy DA (1987) Osteoid of the capitate, J Bone Joint Surg Am 56:1715-18.
osteomata of the distal phalanx, J Hand Surg Br
12:387-90. Jaffe HL (1935) 'Osteoid osteoma': a benign osteoblastic
tumor composed of osteoid and atypical bone, Arch
Campanacci M , Ruggieri P, Gasbarrini A et al (1999) Surg 31:709-28.
Direct visual identification and intralesional excision of
the nidus with minimal removal of bone, J Bone Joint KawaguchiY, HasegawaT, Oka S et al (2001) Mechanism
Surg Br 81:814-20. of intramedullary high intensity area on T2-weighted
magnetic resonance imaging in osteoid osteoma: a
Chamberlain B C , Mosher J F , Levinsohn E M , Greenberg possible role of COX-2 expression, Pathol Int 5 1 :
J A (1992) Subperiosteal osteoid osteoma of the hamate: 933-7.
a case report, J Hand Surg Am 17:462-5.
Kayser F, Resnick D, Haghighi P et al (1998) Evidence of
Dahlin DC (1979) Bone tumors, general aspects and data the subperiosteal origin of osteoid osteomas in tubular
on 6221 cases, 3rd edn. Charles CThomas: Springfield, bones: analysis by CT and M R imaging, Am J
Illinois: 75-86. Roentgenol 170:609-14.

Davies M, Cassar-Pullicino V N , Davies A M et al (2002) Kendrick J l , Evarts C M (1975) Osteoid osteoma: a critical
The diagnostic accuracy of MR imaging in osteoid analysis of 40 tumors, Clin Orthop 54:51-9.
osteoma, Skeletal Radiol 31:559-69.
Kneisl J S , S i m o n M A (1992) Medical management c o m -
De Berg J C , Pattynama PMT, Obermann W R et al (1995) pared with operative treatment for osteoid osteoma,
Percutaneous computed-tomography-guided thermo- J Bone Joint Surg Am 74:179-85.
coagulation for osteoid osteomas, Lancet 346:350-1.
Kohler R, Rubini J , Postec F et al (1995) Traitement de
De S m e t L, Spaepen D, Zachee B , Fabry G (1998) l'ostéome ostéoïde par forage résection percutané sous
Painless osteoid osteoma of the finger in a child. Case contrôle tomodensitométrique (F.R.O.P.). A propos de 27
report, Chir Main 17:143-6. cas, Rev Chir Orthop 81:317-25.

De Smet L (2002) Subperiosteal osteoid osteoma of the Leroy V, Couturaud M , Lathelize H et al (1980) Bone
triquetrum mimicking an avascular necrosis, Chir Main scintigraphy is unreliable in studying osteoid osteoma,
21:140-2. Rev Rhum Mal Osteoartic 47:53-6.

Edeiken J , DePalma AF, Hodes P J (1966) Osteoid Lisanti M , Rosati M , Spagnolli G , Luppichini G (1996)
osteoma (roentgenographic emphasis), Clin Orthop Osteoid osteoma of the carpus. Case reports and a
49:201-6. review of the literature, Acta Ortop Belg 62:195-9.
 OSTEOID OSTEOMA OF THE HAND AND W R I S T 73

Liu PT, Chivers F S , Roberts CC et al (2003) Imaging of Rosenthal DI, Hornicek F J , Wolfe M W et al. (1998)
osteoid osteoma with dynamic gadolinium-enhanced Percutaneous radiofrequency coagulation of osteoid
M R imaging, Radiology 227:691-700. osteoma compared with operative treatment, J Bone
Joint Surg Am 80:815-21.
Marcove RC, Heelan RT, Huvos A G et al (1991) Osteoid
osteoma: diagnosis, localization and treatment, Clin Sans N, Galy-Fourcade D, Assoun J et al (1999) Osteoid
Orthop 267:197-201. osteoma: CT-guided percutaneous resection and
follow-up in 38 patients, Radiology 212:687-92.
Marcuzzi A, Acciaro A L , Landi A (2002) Osteoid osteoma
of the hand and wrist, J Hand Surg Br 27:440-3. Sty J , Simons G (1982) Intraoperative 99m technetium
bone imaging in the treatment of benign osteoblastic
McGrath B E , Bush C H , Nelson T E , Scarborough M T tumors, Clin Orthop 165:223-7.
(1996) Evaluation of suspected osteoid osteoma, Clin
Orthop 327:247-52. Vanderschueren G M , Taminiau A H M , Obermann W R ,
Bloem J L (2002) Osteoid osteoma: clinical results with
Muren C, Hoglund M , Engkvist O, J u h l i n L (1991) thermocoagulation, Radiology 224:82-6.
Osteoid osteomas of the hand. Report of three cases
and review of the literature, Acta Radiol 32:62-6. Vickers C W , Pugh DC, Ivins J C (1959) Osteoid osteoma:
a fifteen-year follow-up of an untreated patient, J Bone
O'Connel J X , Nanthakumar S S , Nielsen GP, Rosenberg Joint Surg Am 41:357-8.
A E (1998) Osteoid osteoma: the uniquely innervated
bone tumor, Mod Pathol 11:175-80. Wilson Y, Fogelman I, Evans DM (1997) Detection of an
elusive osteoid osteoma using a registration bone scan,
Rex C, J a c o b s L, Nur Z (1997) Painless osteoid osteoma J Hand Surg Br 22:801-4.
of the middle phalanx, J Hand Surg Br 22:798-800.
W i s s DA, Reid B S (1983) Painless osteoid osteoma of
Roger B , Bellin MF, Wioland M, Grenier P (1996) Osteoid the fingers. Report of three cases, J Hand Surg Am
osteoma: CT-guided percutaneous excision confirmed 8:914-17.
with immediate follow-up scintigraphy in 16 outpatients,
Radiology 201:239-42. W o o d s E R , Martel W , Mandell S H , Crabbe J P (1993)
Reactive soft tissue mass associated with osteoid
Rosenthal Dl, Alexander A , Rosenberg A E , Springfield D osteoma: correlation of M R imaging features with
(1992) Ablation of osteoid osteomas with a percu- pathologic findings, Radiology 186:221-5.
taneously placed electrode: a new procedure, Radiology
183:29-33.

Rosenthal Dl, Springfield D S , Gebhardt M C et al (1995)

Osteoid osteoma: percutaneous radio-frequency abla-
tion, Radiology 197:451-4.
8
Schwannomas
Anne Gray and Chantal Bonnard

S c h w a n n o m a s , like n e u r o f i b r o m a s , a r e c l a s s i f i e d
as peripheral nerve sheath tumors.They are also
known as neurilemomas and neurinomas. They
a r e o n e of t h e f e w t r u l y e n c a p s u l a t e d t u m o r s , a r e
a l m o s t a l w a y s solitary, a n d a r i s e f r o m a p a r e n t
n e r v e . M u l t i p l e s c h w a n n o m a s a r e r a r e but o c c u r
in t w o s e t t i n g s : bilateral e i g h t h n e r v e s c h w a n n o -
m a s , in a s s o c i a t i o n w i t h n e u r o f i b r o m a t o s i s type
( N F 2 ) ; a n d w i t h a s o m a t i c m u t a t i o n of t h e N F 2
gene, a condition called 'schwannomatosis'
( M a c C o l l i n et al 1996). S c h w a n n o m a s a r i s e f r o m
S c h w a n n cells. There are four major forms of
s c h w a n n o m a s : c o n v e n t i o n a l , cellular, plexiform,
a n d m e l a n o t i c . T h e m o s t f r e q u e n t t y p e is c o n v e n -
tional schwannoma, which is histologically Figure 1
b e n i g n , a l t h o u g h o n o c c a s i o n , is d e s t r u c t i v e of
Schwannoma of the median nerve in the upper arm. The
surrounding osseous structures.The second most
fascicles of the nerve are preserved. The tumor is globoid
frequent is c e l l u l a r s c h w a n n o m a ; histologically
with a well-formed capsule.
this tumor can simulate malignant peripheral
nerve sheath tumor ( M P N S T ) . L e s s c o m m o n is
p l e x i f o r m s c h w a n n o m a w h i c h in t h e c e l l u l a r f o r m
a n d in c h i l d r e n c a n a l s o s i m u l a t e M P N S T . T h e can be v i e w e d . Macroscopically the tumor is
least c o m m o n f o r m is t h e m e l a n o t i c f o r m w h i c h often g l o b o i d , t a n c o l o r w i t h a h o m o g e n e o u s l y
is often mistaken for melanoma (Kurtkaya- f i r m texture ( F i g . 1). H e m o r r h a g e , c y s t i c c h a n g e ,
Y a p i c i e r et al 2003). b r i g h t y e l l o w a r e a s rich in lipid-laden histiocytes
Malignant transformation is e x t r e m e l y rare, ( F i g . 2), a n d fibrin a c c u m u l a t i o n m a y s u p e r v e n e ,
although s o m e cases have been documented. but frank n e c r o s i s is not a feature (Kurtkaya-
Y a p i c i e r et al 2003).
Microscopically S c h w a n n cells are generally
a r r a n g e d in t w o c h a r a c t e r i s t i c p a t t e r n s , referred
Histology and to a s A n t o n i A a n d B . T h e t y p e A a r e a s a r e c e l l u -
immunohistochemistry lar, c o m p o s e d of s p i n d l e c e l l s o f t e n a r r a n g e d in a
p a l i s a d i n g f a s h i o n , or in a n o r g a n o i d arrange-
Histologically s c h w a n n o m a s h a v e cells that h a v e ment (Verocay bodies). Verocay bodies have been
t h e f e a t u r e s of differentiated S c h w a n n c e l l s . O f t e n r e c o g n i z e d a s a c a r d i n a l f e a t u r e of s c h w a n n o m a s .
t h e r e is a w e l l - f o r m e d f i b r o u s c a p s u l e . In p r o p e r l y In c o n t r a s t t u m o r c e l l s in t y p e B a r e a s a r e s e p a -
orientated sections the displaced parent nerve r a t e d b y a b u n d a n t fluid t h a t may form cystic
76 T U M O R S OF THE HAND

p a i n f u l m o b i l e m a s s w i t h a T i n e l s i g n r a d i a t i n g in
t h e s e n s o r y d i s t r i b u t i o n of t h e n e r v e . T h e r e is n o
motor deficit. W h e n t h e s c h w a n n o m a d e v e l o p s
w i t h i n a c a n a l it m a y p r o v o k e a n entrapment
s y n d r o m e , w i t h nocturnal paraesthesia a n d slight
s e n s o r y deficits ( D a s G u p t a e t al 1969).
S c h w a n n o m a s of t h e h a n d a n d w r i s t a r e r a r e
a n d less t h a n 1 p e r c e n t o c c u r in t h i s location
( K r a n s d o r f 1995). K r a n s d o r f presented a demo-
g r a p h i c s t u d y in 1995 o n 77 c a s e s of s c h w a n n o m a
of t h e h a n d a n d w r i s t f r o m 39 179 soft tissue
tumors recorded by the United States Armed
F o r c e s Institute. T h i s r e p r e s e n t e d 0.2 p e r c e n t of
t h e soft t i s s u e t u m o r s . V e r y f e w r e c e n t s e r i e s h a v e
Figure 2 b e e n p u b l i s h e d . R o c k w e l l et al (2003) p r e s e n t e d
21 s c h w a n n o m a s in 18 patients c o l l e c t e d over
Schwannoma of the radial nerve at the elbow. Presence of
7 years. He found that they were equally
bright yellow areas which are rich in lipid-laden histiocytes.
The parent nerve is not easily recognized macroscopically. distributed between right and left hands and
wrists. Lesions w e r e located on the volar surfaces
in 81 p e r c e n t of c a s e s . T h e d i s t r i b u t i o n of t h e

spaces. T h e cells have delicate cobweb-like affected n e r v e w a s a s f o l l o w s : digital n e r v e s 52

processes with round nuclei. per cent, c o m m o n digital n e r v e s 19 p e r c e n t ,
T h e i m m u n o p h e n o t y p e of s c h w a n n o m a s is m e d i a n n e r v e 14 p e r c e n t , ulna n e r v e 9.5 p e r c e n t ,
highly distinctive staining for S-100 protein a n d radial n e r v e 5 p e r c e n t . T h e r e w e r e n o l e s i o n s
( S c h e i t h a u e r et al 1997). A l l o p t i m a l l y f i x e d distal to t h e p r o x i m a l I n t e r p h a l a n g e a l j o i n t s of t h e
t u m o r s a r e u n i f o r m l y i m m u n o p o s i t i v e for this d i g i t s a n d i n t e r p h a l a n g e a l j o i n t of t h e thumb.
s t a i n . A s i m i l a r pattern of s t a i n i n g , a l t h o u g h less O t h e r p u b l i c a t i o n s of s e r i e s of s c h w a n n o m a in
f r e q u e n t is s e e n w i t h Leu7. t h e h a n d i n c l u d e t h o s e b y D a s G u p t a e t al (1969)

In c y t o / m o l e c u l a r g e n e t i c s , m u t a t i o n s in t h e w h o p r e s e n t e d 25 c a s e s in 1967, P h a l e n (1976)
N F 2 g e n e at p o s i t i o n 22q12.2 a r e t h e b a s i s of w h o p r e s e n t e d 11 c a s e s , a n d W h i t e (1967) w h o
s c h w a n n o m a f o r m a t i o n in both s p o r a d i c s c h w a n - p r e s e n t e d 12 c a s e s . N o n e of t h e s e s t u d i e s u s e d
n o m a s a n d n e u r o f i b r o m a t o s i s t y p e 2 ( W o l f f et al c u r r e n t h i s t o l o g i c a l s t a i n s a n d c l a r i f i c a t i o n s in t h e
1992). N F 2 is a t u m o r s u p p r e s s o r g e n e that c o d e s classification s y s t e m that have differentiated
for m e r l i n ( s c h w a n n o m i n ) . schwannoma from neurofibroma.
In o u r c e n t e r f o u r o u t of 27 o p e r a t e d s c h w a n -
n o m a s w e r e localized in t h e h a n d o r w r i s t . Of
these, t w o w e r e from the median nerve, o n e w a s
Clinical findings f r o m a b r a n c h of a t h e n a r n e r v e , a n d t h e f o u r t h
w a s localized to t h e d o r s a l s u r f a c e of t h e h a n d .
T h e m o s t c o m m o n l o c a t i o n s for s c h w a n n o m a s
a r e t h e f l e x o r s u r f a c e s of t h e e x t r e m i t i e s (espe-
cially at t h e l e v e l of t h e e l b o w , w r i s t , a n d k n e e ) ,
neck, mediastinum, retroperitoneum, posterior Investigations
spinal roots, a n d c e r e b e l l o p o n t i n e a n g l e ( O b e r m a n
and Sullenger 1967). Schwannoma can occur Routine X-rays can reveal a sharply circumscribed
in individuals of all ages, but shows a peak m a s s . Ultrasound c a n distinguish cystic f r o m solid
i n c i d e n c e b e t w e e n t h e t h i r d a n d sixth d e c a d e s . masses and encapsulated from non-encapsulated
N o sex predilection is e v i d e n t . A s a r u l e , t h e y lesions which c a n be important for diagnosing
g r o w s l o w l y o v e r a p e r i o d of y e a r s . T h e i r size l e s i o n s in t h e h a n d a n d w r i s t .
varies f r o m microscopic, through barely palpable, M a g n e t i c r e s o n a n c e imaging ( M R I ) c a n distin-
to l a r g e r l e s i o n s w h i c h o n p h y s i c a l e x a m i n a t i o n g u i s h f e a t u r e s of a b e n i g n n e u r o g e n i c t u m o r
are mobile. Patients present with a slightly which include a well-defined oval m a s s with the
 SCHWANNOMAS 77

Figure 3

(a) Schwannoma of the common fibular nerve near the

junction of the sciatic nerve at the level of the popliteal
fossa. S P I (in French) refers to tibial nerve. S P E is the
common fibular nerve. Suralis is the sural nerve. The tumor
is globoid, (b) With the dissection of the tumor it is evident
that the tumor can be enucleated while preserving the par-
ent nerve. (c) The fascicles entering the almost enucleated
tumor are non-functional. The rest of the nerve is intact.

l o n g a x i s in line w i t h t h e n e r v e of o r i g i n a n d tomography ( C T ) h a s b e e n u s e d to distinguish

homogeneous intermediate signal on T1- recurrent s c h w a n n o m a s a s it is p o s s i b l e to d e f i n e
weighted s e q u e n c e s (Saifuddin 2003).The t u m o r s t h e extent of t u m o r r e c u r r e n c e e x a c t l y o w i n g t o
are hyperintense on T2-weighted i m a g e s with a l i m i t a t i o n s of M R I in differentiating v i a b l e t u m o r
n o n - h o m o g e n e o u s central low signal intensity f r o m p o s t o p e r a t i v e s c a r t i s s u e ( A n t o c h et al 2002).
(the t a r g e t s i g n ) . T h i s t a r g e t s i g n is a t t r i b u t e d to Electromyography ( E M G ) can be useful in
t h e d i s t r i b u t i o n of A n t o n i A a n d B a r e a s but is e v a l u a t i n g n e u r o l o g i c deficit. In g e n e r a l t h o u g h
also present with neurofibromas. Differentiation the slow growth a n d non-infiltrative nature of
between schwannomas and neurofibromas can s c h w a n n o m a s minimizes nerve dysfunction and
be made by determining the relationship of nerve conduction studies are normal.
t h e t u m o r to t h e n e r v e of o r i g i n . W i t h s c h w a n n o -
mas the nerve has an asymmetric relationship
to the t u m o r ( M a i u r i et al 2001), w h e r e a s t h e
nerve passes through t h e c e n t e r of a neurofi- Treatment
b r o m a o r is o b l i t e r a t e d b y it. N e u r o f i b r o m a s a n d
schwannomas a r e not associated with peritu- Optimal treatment is g r o s s total r e s e c t i o n w i t h
moral edema. s p a r i n g of t h e p a r e n t n e r v e , w h e n o n e is identi-
Positron emission t o m o g r a p h y ( P E T ) is c o n - f i e d . C a r e f u l e n u c l e a t i o n f r o m t h e affected n e r v e
sidered a sensitive tool in the diagnosis of is required to maintain neurologic function
s c h w a n n o m a ( A h m e d et al 2001). P E T / c o m p u t e d (Fig. 3). T h e d i a g n o s i s is e x t r e m e l y important
78 T U M O R S OF THE HAND

preoperativey s o t h a t a n e r v e is not s a c r i f i c e d . In Das GuptaTK, Brasfield RD, Strong E W , Hajdu S I (1969)

t h e s e r i e s of R o c k w e l l et al 2003 of 21 s c h w a n n o - Benign solitary schwannoma (neurilemonas) Cancer
m a s in t h e h a n d a n d w r i s t t h e clinical d i a g n o s i s 24:355-66.
w a s c o r r e c t in o n l y 19 p e r c e n t of c a s e s . G a n g l i o n
was the most common misdiagnosis. Two Kransdorf M J (1995) Benign soft-tissue tumors in a
large referral population: distribution of specific diag-
p a t i e n t s , b e c a u s e of a n i n c o r r e c t d i a g n o s i s , h a d
nosis by age, sex and location, Am J Roentgenol 164:
r e s e c t i o n of digital n e r v e s , o n e d i a g n o s e d a s a
395-402.
neuroma and the other a s a ganglion. Both cases
r e s u l t e d in s e n s o r y l o s s .
Kurtkaya-Yapicier O, Schelthauer B, Woodruff J M (2003)
Although conventional schwannomas are The pathobiologic spectrum of schwannomas, Histol
benign, incompletely excised tumors are capable Histopathol 18:925-34.
of s l o w r e c u r r e n c e . M a l i g n a n t t r a n s f o r m a t i o n is
e x c e e d i n g l y r a r e . T h e e x t e n t of t u m o r resection MacCollin M , Woodfin K, Kronn D, Short M P (1996)
d e p e n d s o n its l o c a t i o n . N i n e t y p e r c e n t of p e r i - Schwannomatosis: a clinical and pathologic study,
p h e r a l l y s i t u a t e d t u m o r s a r e a m e n a b l e to g r o s s Neurology 46:1702-9.
t o t a l r e m o v a l . If i n t r a c r a n i a l , w i t h i n t h e spinal
c o r d o r p a r a v e r t e b r a l d e b u l k i n g of t h e t u m o r is Maiuri F, Donzelli R, Benvenuti D et al (2001)
indicated. Schwannoma of the brachial plexus: diagnostic and
surgical problems, Zentralbl Neurochir 62:93-7.

Conclusions Oberman HA, Sullenger G (1967) Neurogenous tumors

of the head and neck, Cancer 20:1992-2001.

T h e hand surgeon will probably s e e and diagnose

Phalen G S (1976) Neurilemonas of the forearm and
s c h w a n n o m a in t h e h a n d o r w r i s t . T h e a b s e n c e of
hand, Clin Orthop 114:219-22.
s p e c i f i c c l i n i c a l d i a g n o s t i c t e s t s , a n d its v a r i a b l e
clinical p r e s e n t a t i o n m a k e s p r e o p e r a t i v e d i a g n o -
Rockwell G M . T h o m a A , Salama S (2003) Schwannoma
s i s difficult. I m a g i n g in t h e f o r m of M R I of t h e of the hand and wrist, Plast Reconstr Surg 111:1227-32.
h a n d is e v o l v i n g a n d , if p e r f o r m e d , c a n facilitate
the diagnosis. However, the intraoperative Saifuddin A (2003) Imaging tumours of the brachial
a p p e a r a n c e of a r o u n d , w e l l - e n c a p s u l a t e d , p e d u n - plexus, Skeletal Radiol 32:375-87.
culated n e o p l a s m that produces n e r v e enlarge-
m e n t a n d is e a s i l y e n u c l e a t e d f r o m t h e nerve Scheithauer B W , Woodruff J M , Erlandson R A (1997)
s u g g e s t s t h e p r e s e n c e of a s c h w a n n o m a . Tumors of the Peripheral Nervous System. Armed
Forces Institute of Pathology: Washington DC: 105-76.

W h i t e N B (1967) Neurilemonas of the extremities,

References J Bone Joint Surg Am 49:1605-10.

A h m e d A R , W a t a n a b e H, Aoki J et al (2001) Wolff RK, Frazer KA, Jackler RK et al (1992) Analysis of

Schwannoma of the extremities: the role of P E T in chromosome 22 deletions in neurofibromatosis type
preoperative planning, Eur J Nuc Med 28: 1541-51. 2-related tumors, Am J Hum Genet 51:478-85.

Antoch G , EgelhofT, Korfee S et al (2002) Recurrent

schwannoma: diagnosis with PET/CT, Neurology
59:1240.
9
Neurofibroma
Alberto Lluch

Embryology Terminology
T h e e c t o d e r m is initially d i r e c t e d , t h r o u g h the Although considered to be nerve tumors, all
influence of the underlying mesoderm, to tumors in peripheral nerves should be called
become neuroderm by a process known as 'nerve sheath tumors', as the only nervous tissues
neuronal induction. T h e brain a n d spinal cord in a p e r i p h e r a l n e r v e a r e t h e n e r v e f i b e r s . N e r v e
d e v e l o p f r o m a n a r e a of ectoderm called the f i b e r s r e f e r r e d to a s a x o n o r d e n d r i t e depending
n e u r a l p l a t e . T h e l a t e r a l e d g e s of t h e n e u r a l p l a t e o n w h e t h e r t h e y a r e t h e distal p r o l o n g a t i o n o f a
s o o n b e c o m e e l e v a t e d to f o r m neural folds, a n d motor or sensitive neuron. Besides nerve fibers,
j o i n t o g e t h e r d o r s a l l y , in t h e m i d l i n e , f o r m i n g t h e the following cells c a n be found in peripheral
neural tube (Iyer 2000). T h e early cord has t w o nerves: S c h w a n n cells, perineural cells, adipose
a r e a s of p r o l i f e r a t i n g c e l l s , p r o d u c i n g sensory c e l l s , f i b r o b l a s t s , a n d t h e s t r u c t u r a l c e l l s of b l o o d
and motor nerves which begin outgrowth v e s s e l s . N e o p l a s t i c c h a n g e s c a n o c c u r in a n y of
t o w a r d s t h e l i m b b u d d u r i n g t h e t h i r d w e e k of these cells, being responsible for the growth
development. In t h e f o u r t h w e e k , t h e brachial of intraneural ganglions, intraneural lipomas
p l e x u s u n i t e s a n d a d v a n c e s into t h e l i m b b u d . ( M o r l e y 1964, F r i e d l a n d e r et al 1969), a n d intra-
N e r v e g r o w t h a d v a n c e s t o t h e e l b o w at w e e k 5 n e u r a l h e m a n g i o m a s ( P u r c e l a n d G u r d j i a n 1935,
a n d t o t h e f i n g e r s b y w e e k 7. T h e p a t h f o l l o w e d is L o s l i 1952, S t r i c k l a n d a n d S t e i c h e n 1977). T h e s e
very selective and precise. Motor and sensory tumors, despite growing on a peripheral nerve
cell b o d i e s s e n d o u t o n l y o n e v e r y l o n g fiber, a r e c o n s i d e r e d to b e n o n - n e u r o g e n i c in o r i g i n . O n
a x o n a n d d e n d r i t e , r e s p e c t i v e l y , to a p r e d e t e r - t h e o t h e r h a n d , t h e S c h w a n n cell h a s a n e u r o -
m i n e d t a r g e t . T h e n e u r a l c r e s t is r e s p o n s i b l e f o r e c t o d e r m a l o r i g i n , a n d it is f o r t h i s r e a s o n t h a t
s e n s o r y n e r v e s . D e s t r u c t i o n of t h e n e u r a l c r e s t t u m o r s c a u s e d b y a n o m a l o u s g r o w t h of S c h w a n n
p r i o r t o its m i g r a t i o n r e s u l t s in n o r m a l motor c e l l s a r e c o n s i d e r e d to b e t r u e n e r v e t u m o r s , o r
innervation b u t a n a b s e n c e of s e n s o r y n e r v e s nerve sheath tumors.
( B e a t h y 2000).
T h e peripheral nervous s y s t e m originates f r o m
the neural crest, a group of specialized cells
located just dorsal to the neural tube. T h e s e Incidence
c e l l s f o r m a v a r i e t y of p e r i p h e r a l components,
including sensory and autonomic neurons in Peripheral nerve or nerve sheath tumors are
the peripheral ganglia, S c h w a n n cells, and relatively rare, with s c h w a n n o m a s and neuro-
m e l a n o c y t e s of t h e s k i n . T h e c o m m o n e c t o d e r m i c fibromas being the most c o m m o n a m o n g s t t h e m ,
origin explains the relationship between neuro- particularly the latter. T h e i n c i d e n c e has been
f i b r o m a s a n d skin p i g m e n t a t i o n s , referred t o a s r e p o r t e d to r a n g e f r o m 1.02 p e r c e n t ( B u t l e r et al
'café-au-lait' spots. 1960) t o 4.9 p e r c e n t ( S t a c k 1960), a n d is u p t o
80 T U M O R S OF THE HAND

a b

Figure 1

(a) Schematic representation of a schwannoma, depicting an ovoid and well-encapsulated tumor displacing the nerve fasci-
cles. (b) Schematic representation of a plexiform neurofibroma, involving the nerve fascicles which are enlarged in both the
longitudinal and transverse dimensions, resembling worms.

9 p e r c e n t of all t u m o r s s e e n at t h e M a y o C l i n i c m a k i n g excision feasible ( F i g . 1a). N e u r o f i b r o m a s

( N a s c i m e n t o 2000). T h i s high variability c a n b e i n v a d e t h e n e r v e fascicles, distorting a n d e n l a r g -
explained because the reported m e t h o d s are ing t h e m . F o r t h i s r e a s o n , n e u r o f i b r o m a s of l a r g e
different. S o m e a u t h o r s a r e s p e c i a l i s t s in t u m o r nerve trunks cannot be excised with preservation
c a r e , w h i l e o t h e r s a r e s p e c i a l i s t s o n l y in u p p e r of t h e n e r v e f i b e r s t h a t r u n t h r o u g h t h e tumor
extremity t u m o r s , and others are general hand ( F i g . 1b). A l t h o u g h n e u r o f i b r o m a s a r e n o t e n c a p -
s u r g e o n s , w i t h t h e referral c a s e s v a r y i n g b e t w e e n s u l a t e d a n d i n v o l v e n e r v e f a s c i c l e s , t h e y a r e not
t h e m . W h e n l o o k i n g at t h e g e n e r a l p o p u l a t i o n , a d h e r e n t to t h e a d j a c e n t soft t i s s u e s . T h e y c a n b e
t h e r e s u l t s w o u l d o b v i o u s l y differ. W i t h t h i s in of c o n s i d e r a b l e size a n d c a n g r o w a l o n g a l o n g
p e r s p e c t i v e , M o n e y (1950) s u r v e y e d 360 6 7 2 s e g m e n t of t h e n e r v e t r u n k , in w h i c h c a s e t h e y
hospital a d m i s s i o n s o v e r a 3 0 - y e a r p e r i o d a n d o n l y are called plexiform fibromas.
t h r e e s c h w a n n o m a s in t h e u p p e r extremity, a n d Both s c h w a n n o m a s and neurofibromas arise
n i n e n e u r o f i b r o m a s , including u p p e r a n d l o w e r f r o m t h e n e r v e s h e a t h , f r o m a cell w i t h a d o u b l e
e x t r e m i t i e s , w e r e d i a g n o s e d , r e p r e s e n t i n g a total b a s e m e n t m e m b r a n e c h a r a c t e r i s t i c of Schwann
of 0.0033 p e r c e n t of patients s e e n in his h o s p i t a l . cells. N e u r o f i b r o m a s c a n also originate f r o m a
p e r i n e u r a l f i b r o c y t e t h a t is e m b r y o l o g i c a l l y m o r e
primitive than the S c h w a n n cell. S c h w a n n o m a s
Pathology a r e c o m p o s e d a l m o s t e n t i r e l y of S c h w a n n c e l l s ,
while neurofibromas have a more mixed compo-
On macroscopic examination, s c h w a n n o m a s are sition. A disorderly arrangement of Schwann
o v o i d in s h a p e , w e l l e n c a p s u l a t e d , a n d d i s p l a c e , cells, nerve fibers, a n d collagen c a n be o b s e r v e d
but d o not i n v a d e , n e r v e f a s c i c l e s a s t h e y g r o w , in n e u r o f i b r o m a s . M o s t c e l l s a r e f u s i f o r m and
 NEUROFIBROMA 81

irregularly a r r a n g e d , separated by a considerable

a m o u n t of c o l l a g e n . T h e m a t r i x p r e s e n t s n u m e r -
ous c o l l a g e n f i b e r s a n d often mucoid changes
( F i g . 2). H i s t o l o g i c a l e x a m i n a t i o n m a y also dis-
close mast cells, xantoma cells, and lymphocytes
( L o u i s 1987).
Electron-microscopic studies h a v e confirmed
that s c h w a n n o m a s are c o m p o s e d almost entirely
of S c h w a n n c e l l s , w h e r e a s n e u r o f i b r o m a s a r e of
m o r e m i x e d c o m p o s i t i o n . H o w e v e r , it h a s b e e n
o b s e r v e d t h a t S c h w a n n c e l l s not o n l y produce
myelin but also collagen (Enzinger and W e i s s
1983, L o u i s 1987).
The p r e s e n c e of S c h w a n n c e l l s c a n b e c o n -
firmed with antigenic m a r k e r s for vimentin (a Figure 2
c y t o s k e l e t a l f i l a m e n t ) , S 1 0 0 (a calcium-binding
Photomicrograph of a neurofibroma showing a disorderly
p r o t e i n ) , L e u 7 (a g l y c o p r o t e i n ) , a n d m y e l i n b a s i c
arrangement of cells, the majority of which are fusiform,
p r o t e i n (a m y e l i n p o l y p e p t i d e ) (Idler 1995). in a matrix formed primarily by collagen and some mixoid
A f u s i f o r m g r o w t h of t h e m e d i a n n e r v e at t h e tissue.
wrist level, resembling a neurofibroma, has been
d e s c r i b e d b y s e v e r a l a u t h o r s . B e c a u s e of its p r e -
dominant lipomatous cellular component on
m i c r o s c o p i c e x a m i n a t i o n , it h a s b e e n p r o p o s e d m u s c l e , s e e m t o b e infiltrated b y t h e t u m o r , a
that this t u m o r should be n a m e d lipofibromatous neurofibrosarcoma should be suspected. B i o p s y
hamartoma of the median nerve (Johnson is mandatory in those cases where possible
and B o n f i g l i o 1969, T s u g e a n d Ikuta 1973, P a t e l malignant degeneration into a neurofibrosar-
et al 1979). c o m a n e e d s to b e r u l e d out.
Sonography, computed tomography (CT) scan,
a n d m a g n e t i c r e s o n a n c e i m a g i n g ( M R I ) c a n a i d in
t h e d i a g n o s i s of a n e u r o f i b r o m a . M R I T 2 - w e i g h t e d
Diagnosis i m a g e s s h o w t h e s e l e s i o n s a s h a v i n g a brighter
s i g n a l t h a n a d j a c e n t m u s c l e ( M i l l e r et al 1994).
Neurofibromas are asymptomatic, causing a
N e u r o f i b r o m a s c a n b e f o u n d in p e r i p h e r a l a n d
deficit of n e r v e f u n c t i o n o n l y if g r o w t h o c c u r s
autonomic nerves, as well a s in the central
inside unyielding a n a t o m i c c o m p a r t m e n t s , such
n e r v o u s s y s t e m . T h e y c a n p r e s e n t a s solitary o r
a s t h e c a r p a l t u n n e l . A soft t i s s u e m a s s o v e r t h e
m u l t i p l e l e s i o n s , t h e latter c a s e is k n o w n a s n e u r o -
a n a t o m i c c o u r s e of a p e r i p h e r a l n e r v e , w i l l l e a d
f i b r o m a t o s i s or v o n R e c k l i n g h a u s e n ' s d i s e a s e .
t o s u s p i c i o n of t h e p r e s e n c e of a n e u r o f i b r o m a .
D i a g n o s i s of v o n R e c k l i n g h a u s e n ' s d i s e a s e is
C l i n i c a l d i a g n o s i s a l o n e is not p o s s i b l e , a n d
easier, because of its polymorphic clinical
should a l w a y s be confirmed by surgical explo-
manifestation a n d a l s o b e c a u s e t h e n e u r o f i b r o m a s
ration. Macroscopically, a m a s s distorting a n d
e n l a r g i n g t h e n e r v e b o t h in l e n g t h a n d w i d t h , are usually larger a n d n u m e r o u s . V o n Reckling-
g i v i n g t h e a p p e a r a n c e of a t o r t u o u s a n d n o d u l a r h a u s e n ' s d i s e a s e is a v e r y puzzling and bizarre
n e r v e s e g m e n t c a n b e o b s e r v e d . If t h e e p i n e - d i s e a s e that w i l l b e d e s c r i b e d later. S o l i t a r y n e u -
u r i u m is longitudinally divided, the whitish r o f i b r o m a s m a y r e m a i n u n d i a g n o s e d for t h e entire
fibrous tissue invading the nerve fascicles can be life of a p e r s o n , a s t h e y m a y b e located in v i s c e r a
o b s e r v e d , making excision impossible. Diagnosis or other i n a c c e s s i b l e inner o r g a n s ( H e a r d 1962).
is m a d e b y d i r e c t o b s e r v a t i o n of t h e m a s s ,
a l t h o u g h it c a n b e c o n f i r m e d b y b i o p s y a n d h i s -
tological examination. Although neurofibroma
i n v a d e s n e r v e f a s c i c l e s , it is a w e l l - e n c a p s u l a t e d
Prognosis
g r o w t h w h i c h d o e s n o t infiltrate t h e t i s s u e s t h a t
N e u r o f i b r o m a is a b e n i g n t i s s u e g r o w t h which
surround it. If t h e n e i g h b o r i n g tissues, mainly
may undergo malignant degeneration, called
82 T U M O R S OF THE HAND

neurofibrosarcoma. Malignant transformation There are two types of neurofibromatosis,

is r a r e in s o l i t a r y neurofibromas or in m u l t i p l e known as neurofibromatosis 1 (NF1) and neuro-
n e u r o f i b r o m a s i n v o l v i n g t h e skin n e r v e e n d i n g s f i b r o m a t o s i s 2 ( N F 2 ) . E n t i r e l y different g e n e s a r e
( H e a r d 1962, J o h n s o n a n d B o n f i g l i o 1969, T s u g e r e s p o n s i b l e f o r N F 1 a n d N F 2 . N F 1 is e s s e n t i a l l y
a n d Ikuta 1973, P a t e l et al 1979, D u c a t m a n a n d t h e t y p e d e s c r i b e d b y v o n R e c k l i n g h a u s e n . It c a n
S c h e i t h a u e r 1983, E n z i n g e r a n d W e i s s 1983, L o u i s b e s e c o n d a r y to hereditary transmission or s p o n -
1987, M i l l e r et al 1994, Idler 1995). Malignant t a n e o u s a u t o s o m a l d o m i n a n t mutation of the N F 1
d e g e n e r a t i o n is m o r e f r e q u e n t in t h e p r e s e n c e of g e n e located o n the long a r m of chromosome
neurofibromatosis, mainly when the tumor 17 ( S e i z i n g e r et al 1987, v o n D e i m l i n g et al 1995).
i n v o l v e s m a j o r n e r v e t r u n k s , w i t h a r e p o r t e d inci- T h e N F 1 g e n e e n c o d e s a protein called neuro-
d e n c e f r o m 3 to 15 p e r c e n t of p e o p l e w i t h v o n f i b r o m i n , w h i c h is 2818 a m i n o a c i d s l o n g , o n e of
Recklinghausen's disease (Jenkins 1952, Stack t h e l a r g e s t p r o t e i n s . T h e l a r g e size of t h e N F 1
1960, H e a r d 1962, E n n e k i n 1983, E n z i n g e r a n d g e n e e x p l a i n s t h e h i g h n u m b e r of m u t a t i o n s t h a t
W e i s s 1988). c a n o c c u r . N F 1 is o n e of t h e most frequently
T h e difference b e t w e e n solitary a n d multiple i n h e r i t e d d i s e a s e s , p r e s e n t in o n e in e v e r y 3 0 0 0
n e u r o f i b r o m a s is not j u s t t h e i n c i d e n c e of m a l i g - births. A b o u t half of t h e o f f s p r i n g of a p a r e n t w i t h
n a n t d e g e n e r a t i o n , b u t a l s o t h e d e g r e e of m a l i g - neurofibromatosis will present certain stigmata,
nancy. N e u r o f i b r o s a r c o m a s arising f r o m solitary w h i c h c a n b e s o m i l d a s t o g o u n r e c o g n i z e d . It is
n e u r o f i b r o m a s h a v e a 5 - y e a r s u r v i v a l rate of 7 5 inherited as an autosomal dominant disease,
p e r c e n t , a s c o m p a r e d t o o n l y 30 p e r c e n t s u r v i v a l although spontaneous mutations are c o m m o n ,
in p a t i e n t s suffering f r o m v o n R e c k l i n g h a u s e n ' s a s 50 p e r c e n t of c a s e s d o not h a v e a p o s i t i v e
d i s e a s e ( D ' A g o s t i n o et al 1963a, 1963b, G o s h et al f a m i l y history f o r t h e d i s e a s e (Grifith et al 1972).
1973). R a p i d g r o w t h of t u m o r size, p a i n a n d l o s s T h i s m e a n s t h a t a b o u t half of t h o s e w i t h n e u r o -
of n e r v e f u n c t i o n a r e s i g n s of m a l i g n a n t c h a n g e s f i b r o m a h a v e i n h e r i t e d it f r o m a p a r e n t ; t h e o t h e r
in a pre-existing neurofibroma. half being affected b e c a u s e of a spontaneous
mutation with no affected parent.
N F 2 is c a u s e d b y m u t a t i o n s in t h e N F 2 g e n e ,
w h i c h is l o c a t e d in t h e l o n g a r m of c h r o m o s o m e
Neurofibromatosis and 22. To d a t e , r e s e a r c h e r s h a v e n o t f o u n d links

von Recklinghausen's disease b e t w e e n t h e b i o l o g y of t h e N F 1 a n d N F 2 g e n e s .

N F 2 is a r a r e t y p e of n e u r o f i b r o m a t o s i s , with a
A k e n s i d e in 1768 w a s t h e first to report t h e c u t a - p r e v a l e n c e of 0.1 p e r 100 0 0 0 births. Its c l i n i c a l
neous manifestations of neurofibromatosis. In manifestations a r e a l s o different f r o m t h o s e of
1882, Friederich Daniel von Recklinghausen N F 1 , presenting with cataracts, brain and spine
described two patients w i t h nerve t u m o r s and tumors, and acoustic neuromas, impairing
skin a b n o r m a l i t i e s , a n d p r o p o s e d t h e t e r m n e u r o - h e a r i n g a n d c a u s i n g l o s s of b a l a n c e , a s t h e m o s t
fibromatosis. This is a d i s e a s e w h i c h causes characteristic findings.
abnormalities of the tissue derivatives of the Von R e c k l i n g h a u s e n ' s d i s e a s e is o n e of the
three g e r m layers: ectodermal structures such a s most bizarre c o n d i t i o n s that affect t h e human
t h e skin a n d n e r v e s ; m e s o d e r m a l s t r u c t u r e s s u c h b o d y . It is listed a s o n e o f t h e p h a k o m a t o s e s ,
a s b o n e a n d c o n n e c t i v e t i s s u e , in t h e f o r m o f lipo- or neurocutaneous syndromes that affect the
m a s a n d f i b r o m a s ; a n d e v e n certain e n d o d e r m a l skin, e y e , brain, skeleton and other organs.
structures like i n t e s t i n e , e n d o c r i n e o r g a n s , a n d Phakomatosis (neurocutaneous syndrome) w a s a
blood vessels.The most important developmental t e r m c o i n e d b y v a n d e r H o v e in 1921 ( G l i c e n s t e i n
diathesis o c c u r s at the neuroectodermal tissues: et al 1988) to i n c l u d e t h e f o l l o w i n g d e v e l o p m e n t a l
central a n d peripheral n e r v o u s s y s t e m s , a n d skin. abnormalities: neurofibromatosis, Bourneville's
In 1 9 0 0 , T h o m p s o n s u g g e s t e d t h a t t h e d i s e a s e tuberous sclerosis, S t u r g e - W e b e r ' s angiomatosis,
c o u l d b e h e r e d i t a r y . L a t e r it w a s r e c o g n i z e d to and Hippel-Lindau's angiomatosis.
be a neurocristopathy ( B o l a n d e 1981), but the T h e r e a r e a w i d e v a r i e t y of clinical s y m p t o m s ,
m e c h a n i s m by w h i c h the mutant g e n e or g e n e s ranging from 'forms fruste', with incomplete
a c t t o result in s u c h a v a r i e t y of t h e phenotypic e x p r e s s i o n , in w h i c h t h e d i a g n o s i s w i l l b e o v e r -
p r e s e n t a t i o n s o f t h i s d i s e a s e is n o t k n o w n . looked, to pleomorphic manifestations making
 NEUROFIBROMA 83

a b

Figure 3

(a) Skin pigmentations on a patient with von Recklinghausen's disease, known as 'café-au-lait' spots, because of their light-
brown color. The borders are very irregular, and more than three should be present to be considered as von Recklinghausen's
disease. (b) Cutaneous fibromas, also known as molluscum fibrosum, in the forearm of a patient with von Recklinghausen's
disease.

the patient look like a m o n s t e r , s i m i l a r to the

'Café-au-lait' spots
'elephant man'. Just a s the d e g r e e of clinical
e x p r e s s i o n is h i g h l y v a r i a b l e s o t o o is t h e extent, 'Café-au-lait' spots are light-brown macular
involving only o n e finger or the entire body. cutaneous lesions with very irregular borders
For m a n y y e a r s , t w o t y p e s of neurofibroma- (Fig. 3a). They vary in size, a n d are mostly
t o s i s h a v e b e e n identified: N F 1 a n d N F 2 ( R i c c a r d i o b s e r v e d o n t h e trunk, axilla, a n d i n g u i n a l a r e a s .
et al 1981, M a c k i n n o n a n d D e l l o n 1988). N F 1 is Freckling in t h e axillary a n d i n g u i n a l r e g i o n s a r e
the most c o m m o n presentation, accounting for c h a r a c t e r i s t i c c u t a n e o u s s i g n s . A t least f i v e s p o t s
90 p e r c e n t of all n e u r o f i b r o m a t o s i s . T h e most of a m i n i m u m size of 15 m m in d i a m e t e r e a c h
characteristic clinical features are 'café-au-lait' s h o u l d b e f o u n d t o m a k e a definite d i a g n o s i s of
spots, hirsutism, pigmented h a m a r t o m a s in t h e von R e c k l i n g h a u s e n ' s d i s e a s e ( S e i z i n g e r et al
iris k n o w n a s L i s h n o d u l e s , m u l t i p l e neurofibro- 1987). I n c r e a s e d m e l a n i n in t h e b a s a l l a y e r of t h e
mas in t h e p e r i p h e r a l a n d s u b c u t a n e o u s e n d of e p i d e r m i s is f o u n d . B i o p s y w i l l s h o w i n c r e a s e d
nerves, subcutaneous fibromas, sebaceous ade- n u m b e r s of d o p a - p o s i t i v e m e l a n o c y t e s a n d t h e
n o m a s , l i p o m a s , intestinal t u m o r s , b o n e d e f o r m i - presence of giant cytoplasmic granules. Thus,
ties, a n d m e n i n g i o m a s (Strickland a n d Steichen s u p p o r t i n g t h e h y p o t h e s i s that neurofibromatosis
1977). T h e p r e s e n c e of p l e x i f o r m neurofibromas is d u e t o a p r i m a r y d i s o r d e r of n e u r a l c r e s t o r i g i n .
may produce flabby redundant hypertrophy of
soft t i s s u e a n d e v e n g i g a n t i s m of a l i m b .
Multiple s c h w a n n o m a s can also be found in
t h e b r a i n , s p i n a l c o r d , a c o u s t i c n e r v e , a n d intesti- Cutaneous neurofibroma
nal nerves. However, single schwannomas (molluscum fibrosum)
l o c a t e d o n p e r i p h e r a l n e r v e s a r e not u s u a l l y a s s o -
ciated w i t h v o n Recklinghausen's d i s e a s e (Harkin Cutaneous neurofibromas are subcutaneous
1980). T h r e e patients have been reported to n o d u l e s c a u s i n g s m o o t h e l e v a t i o n s of t h e skin,
h a v e v o n R e c k l i n g h a u s e n ' s d i s e a s e in a s s o c i a t i o n localized but not e n c a p s u l a t e d , w i t h a soft to rub-
with Ehler-Danlos syndrome (Turkington and bery consistency.They are painless a n d s l o w grow-
G r u d e 1964, M c K u s i c k 1972), b u t t h i s m a y b e o n l y ing, a n d c a n b e s e s s i l e o r p e d u n c u l a t e d , a n d r e a c h
coincidental. a c o n s i d e r a b l e size. C u t a n e o u s n e u r o f i b r o m a s ,
84 T U M O R S OF THE HAND

a b

Figure 4

(a) Cutaneous manifestations in the hand and forearm of a middle-aged man diagnosed with von Recklinghausen's disease:
nerve fibromas or molluscum fibrosum and a large subcutaneous fibroma covered by hyperpigmented skin and hypertrico-
sis on the dorsum of the hand. (b) Surgery was undertaken solely for esthetic reasons. The subcutaneous tumor was seen
as a large white-grayish and rubbery fibroma, which did not invade the extensor tendons, but partially invaded the fascial
covering of the interosseous muscles. The soft tissue mass and most of the overlying hairy and hyperpigmented skin were
removed obtaining a very satisfactory esthetic result.

a l s o c a l l e d m o l l u s c u m f i b r o s u m , a r e distributed n e u r o f i b r o m a s . T h e o v e r l y i n g skin is u s u a l l y flat-

predominantly along sensory nerves (Fig. 3b).The t e n e d , a n d t h e n e r v e of o r i g i n is r a r e l y seen.
o v e r l y i n g d e r m i s c a n b e h y p e r p i g m e n t e d or w i t h When examined by electron microscopy, the
e x c e s s i v e hair g r o w t h , hypertricosis or hirsutism lesions resemble a neurofibroma.
( F i g . 4 a ) . T h e y c a n a l s o b e f o u n d in t h e t o n g u e a n d
viscera.
C u t a n e o u s n e u r o f i b r o m a s m a y d e v e l o p at a n y
Neurofibromas and other
t i m e in life, a l t h o u g h t h e y a r e r a r e b e f o r e p u b e r t y , nerve tumors
a n d t h e y v a r y in n u m b e r f r o m a f e w t o h u n d r e d s ,
or e v e n thousands. In v o n R e c k l i n g h a u s e n ' s d i s e a s e a v a r i e t y of n e r v e
T h e s e tumors probably originate from sensory t u m o r s a r e s e e n in t h e c e n t r a l n e r v o u s s y s t e m ,
nerve endings. O n section, the tumor is p a l e such a s : m e n i n g i o m a s , s c h w a n n o m a s , neurofi-
grey a n d glistening (Fig. 4b). Microscopically, bromas, acoustic neuromas, and gliomas mainly
the lesions contain S c h w a n n cells a n d scattered of t h e o p t i c n e r v e . H o w e v e r , m o s t f r e q u e n t l y t h e y
fibrocyte-like cells w i t h a m a s s i v e extracellular originate in t h e p e r i p h e r a l n e r v e s , e i t h e r c u t a -
collagenous matrix, as seen in the solitary neous or visceral.
 NEUROFIBROMA 85

a c

Figure 5

(a) Macrodactyly of the index finger and soft tissue enlargement of the thenar eminence. (b) Surgical exploration revealed a
plexiform neurofibroma involving the median nerve starting at the distal third of the forearm, without invading the neigh-
boring structures. Thenar mass enlargement was secondary to subcutaneous fibromas and neurofibromas in the index
digital nerves. No thenar muscle hypertrophy was observed. (c) Longitudinal epineurotomy of the median nerve showing
enlarged nerve fascicles, resembling a tangle of worms of varying sizes. The tumor was not excised.

Neurofibromas can present as solitary or of the tibia and the forearm bones, fibrous
multiple lesions. T h o s e involving a long s e g m e n t cortical d e f e c t s of b o n e , S t u r g e - W e b e r s y n d r o m e ,
of a p e r i p h e r a l n e r v e a r e c a l l e d p l e x i f o r m n e u r o - and L é r i 's m e l o r h e o s t o s i s . S e l l e r s et al (1988)
f i b r o m a s , a n d a r e t y p i c a l of v o n R e c k l i n g h a u s e n ' s n o t e d that 14 o u t of t h e 15 p a t i e n t s w i t h c o n g e n i -
d i s e a s e ( F i g . 5 b ) . T h e n e r v e is e n l a r g e d a n d n o d u - tal p s e u d o a r t h r o s i s of t h e b o n e s of t h e f o r e a r m ,
lous, invading the nerve fascicles. W h e n a longi- r e p o r t e d in t h e literature, w e r e d i a g n o s e d w i t h
tudinal epineurotomy is p e r f o r m e d , the nerve neurofibromatosis. Gould (1918) w a s the first
f a s c i c l e s r e s e m b l e a t a n g l e of w o r m s of v a r y i n g to report t h e a s s o c i a t i o n of neurofibromatosis
sizes ( F i g . 5c). with skeletal alterations, including congenital
p s e u d o a r t h r o s i s of l o n g b o n e s . T h i s relationship
w a s later c o n f i r m e d b y o t h e r a u t h o r s ( D u c r o q u e t
Skeletal anomalies 1927, B a r b e r 1939, A n d e r s e n 1973).
Despite the close association of congenital
S e v e r a l skeletal a b n o r m a l i t i e s h a v e b e e n o b s e r v e d p s e u d o a r t h r o s i s of t h e b o n e s of t h e f o r e a r m w i t h
in a s s o c i a t i o n w i t h v o n R e c k l i n g h a u s e n ' s d i s e a s e : von Recklinghausen's disease, the cause remains
unequal limb lengths, scoliosis, pseudoarthrosis u n k n o w n . T h i s is a r a r e c o n d i t i o n , a s l e s s t h a n
86 T U M O R S O F T H E HAND

40 c a s e s h a v e b e e n d e s c r i b e d in t h e literature,
a n d t h e r e f o r e little i n f o r m a t i o n is a v a i l a b l e t o e l u -
cidate the cause. Several authors h a v e d e m o n -
strated the p r e s e n c e of pathologic tissue at
t h e f o c u s of p s e u d o a r t h r o s i s : h a m a r t o m a s a n d
t h e s a m e t y p e of f i b r o u s t i s s u e a s s e e n in n e u r o -
fibromatosis. Others h a v e been unable to find
S c h w a n n cells by electron microscopy (Sprague
a n d B r o w n 1974, M a t t a r 2 0 0 0 ) . M a t h o u l i n et al
(1993) h y p o t h e s i z e d t h a t this could be due to
inadequate development of the mesodermic
s t r u c t u r e s d u r i n g t h e fifth w e e k of fetal life.

Differential diagnosis
of macrodactyly
Macrodactyly is frequently seen in von
Recklinghausen's disease, but can also be
observed in several other disorders besides
neurofibromatosis, such a s congenital partial
gigantism, Ollier's disease, Mafucci's s y n d r o m e ,
Klippel-Trenaunay-Weber's syndrome, Proteus'
syndrome, congenital lymphedema, melor-
h e o s t o s i s ( L é r i 1922), f i b r o m a t o u s h a m a r t o m a of
the median nerve, a n d other conditions (Shereff
et al 1980).
Ollier's disease (Oilier 1901), also called
e n c h o n d r o m a t o s i s , is a result of f a i l u r e of carti-
l a g e in t h e d i a p h y s i s to o s s i f y , u s u a l l y involving Figure 6
several long b o n e s of t h e h a n d s a n d f e e t , but
never the carpal bones. Nerve territory-oriented macrodactyly of the middle and ring
Mafucci's syndrome (Mafucci 1981), also fingers in a young girl. Divergent macrodactyly is quite
characteristic of neurofibromas when they involve common
called angioplastic enchondromatosis, Kart's
digital nerves. In this case, the median nerve, both digital
syndrome or chondrodystrophy with vascular
nerves to the middle finger and the radial digital nerve of
h a m a r t o m a , is a d y s c h o n d r o p l a s i a in a s s o c i a - the ring finger were involved. Treatment consisted of longi-
tion to hemangiomas. The enchondromas are tudinal division of the flexor retinaculum to relieve median
usually located at t h e e p i p h y s e s c a u s i n g b o n e nerve symptoms of compression.
deformity. Phleboliths are common in the
hemangiomas.
K l i p p e l - T r e n a u n a y - W e b e r ' s s y n d r o m e (Klippel In P r o t e u s s y n d r o m e h e m i h y p e r t r o p h y , m a c r o -
a n d T r e n a u n a y 1900) is c h a r a c t e r i z e d b y hemi- cephaly with exostoses, thickened palms and
hypertrophy and vascular abnormalities such a s soles, elongated neck, s u b c u t a n e o u s lipomas,
v a r i c o s e v e i n s , a n d c a p i l l a r y a n d c a v e r n o u s skin and linear verrucous epidermal nevi can be
hemangiomas. Angioplastic disturbance charac- observed.The n a m e w a s coined from the sea god
terized by the following triad: nevi with in Greek mythology who had the power of
metameric distribution, precocious varicosities a s s u m i n g different f o r m s . J o s e p h M e r r i c k , k n o w n
c o n f i n e d to o n e s i d e of t h e b o d y starting from as the elephant m a n , w a s born with severe body
i n f a n c y o r at birth, a n d h y p e r t r o p h y of u n d e r l y i n g deformities, a n d lived a s a circus s i d e s h o w attrac-
t i s s u e s , p a r t i c u l a r l y of t h e b o n e s w h i c h b e c o m e t i o n in 19th c e n t u r y E n g l a n d . F o r a l o n g t i m e it
l o n g e r a n d thicker. w a s t h o u g h t t h a t h e s u f f e r e d f r o m N F 1 , but in t h e
 NEUROFIBROMA 87

a b

Figure 7

(a) Neurofibroma of the radial digital nerve of the middle finger and its dorsal digital branch. Nerve involvement started prox-
imally at the level of the midpalm and ended at the level of the proximal interphalangeal joint. The radial digital nerve of the
middle finger was split from the ulnar digital nerve of the index finger at the level of the distal common digital nerve.
(b! Using optical magnification and microsurgical instruments, the neurofibroma w a s excised from the radial digital nerve
of the middle finger, thus preserving sensibility. The dorsal branch of the digital nerve was more intimately invaded by the
neurofibroma and, therefore, was removed, as the sensory deficit would be less incapacitating.

late 1980s h e w a s r e - d i a g n o s e d a s h a v i n g P r o t e u s the fingers which receive their s e n s o r y supply

syndrome. f r o m e i t h e r t h e u l n a r or, particularly, t h e m e d i a n
Congenital lymphedema, also known as nerve (Fig. 5a). T h e r e are no cases reported of
Nonne-Milroy disease, is inherited as an s i m u l t a n e o u s i n v o l v e m e n t of all f i n g e r s . A f r e -
autosomal dominant disease, and the cause quent finding in macrodactyly in neurofibro-
s e e m s to be an aplasia or hypoplasia of the m a t o s i s is h y p e r t r o p h y of t h e digital n e r v e a n d
l y m p h a t i c s . Fibrofatty proliferation of t h e m e d i a n the parent nerve, usually the median nerve. T h e
nerve or f i b r o l i p o m a t o u s hamartoma can also soft t i s s u e c o m p o n e n t c o n s i s t s of fibro-adipose
p r e s e n t w i t h m a c r o d a c t y l y ( T s u g e a n d Ikuta 1973). tissue and nerve fibers. W h e n t w o fingers are
Macrodactyly in neurofibromatosis is n e r v e affected, they will diverge from one another,
territory-oriented, m e a n i n g t h a t it o c c u r s o n l y in w i t h a characteristic b a n a n a s h a p e m a c r o d a c t y l y
T U M O R S OF THE HAND
 NEUROFIBROMA 89

(Fig. 6). F i n g e r s gain length a n d w i d t h a s long a s von R e c k l i n g h a u s e n ' s d i s e a s e h a v e little to g a i n

t h e child g r o w s . from operative treatment.
The e x t e n t to w h i c h n e u r o l o g i c f a c t o r s influ- T r e a t m e n t of p s e u d o a r t h r o s i s of t h e f o r e a r m
ence growth is not fully known, but children bones by cast immobilization and conventional
with neurofibromatosis, a s o p p o s e d to those with b o n e grafting is u n s u c c e s s f u l . T h e p r e f e r r e d treat-
paralysis, s h o w a hypertrophy of t h e i r affected ment is a wide resection of the area of
extremity. pseudoarthrosis and reconstruction with a v a s -
cularized f i b u l a r graft (Oilier 1901, K l i p p e l and
Trénaunay 1900, L é r i 1922, S h e r e f f et al 1980,
A l l i e u et al 1981, M a f u c c i 1 9 8 1 , M a t h o u l i n et al
Treatment 1993, Bertolotti 1997, M a t t a r et al 2000).

N e u r o f i b r o m a s d o not r e s p o n d to r a d i o t h e r a p y o r
quimiotherapy (Bertolotti 1997). Its o n l y treat-
m e n t is s u r g i c a l , a l t h o u g h t h e s o l e i n d i c a t i o n is Conclusions
for e s t h e t i c r e a s o n s or s y m p t o m a t i c relief. T h e
flexor retinaculum should be longitudinally N e u r o f i b r o m a s c a n p r e s e n t a s s o l i t a r y or m u l t i p l e
d i v i d e d f o r t h e p u r p o s e of r e l i e v i n g n e r v e c o m - l e s i o n s , t h e latter w i t h o t h e r a s s o c i a t e d clinical
p r e s s i o n s y m p t o m s f r o m n e u r o f i b r o m a s of t h e manifestations are called neurofibromatosis.
m e d i a n nerve inside the carpal tunnel. T h e r e a r e t w o t y p e s of n e u r o f i b r o m a t o s i s : N F 1 is
Isolated cutaneous lesions can be excised. the m o s t c o m m o n a n d refers to that described by
H o w e v e r , large plexiform neurofibromas cannot v o n R e c k l i n g h a u s e n . N F 2 is l e s s c o m m o n a n d h a s
b e e x c i s e d w i t h o u t c a u s i n g a l o s s of n e r v e f u n c - different clinical m a n i f e s t a t i o n s , m a i n l y i n v o l v i n g
tion a s they i n v a d e n e r v e fibers (Fig. 5 c ) . T h e only the central nervous s y s t e m .
p o s s i b l e t r e a t m e n t is m i n i m a l e x c i s i o n for t i s s u e Neurofibromas are tissue malformations
d i a g n o s i s , partial e x c i s i o n to d e b u l k a l a r g e t u m o r rather than true n e o p l a s m s , a n d therefore should
m a s s or w i d e r e s e c t i o n w i t h n e r v e graft r e c o n - p r o b a b l y b e r e f e r r e d t o a s h a m a r t o m a s or t i s s u e
struction. H o w e v e r , s o m e neurofibromas, mainly m a l f o r m a t i o n s rather t h a n t u m o r s ( S e d d o n 1972,
t h o s e affecting monofascicular nerves, such as Strickland and S t e i c h e n 1977). T h e i r genetic
t h e digital n e r v e s , c a n b e e x c i s e d w i t h o u t d a m - origin has been recently confirmed, as being
a g e to n e r v e f i b e r s , if o p t i c a l m a g n i f i c a t i o n and d u e to a n o m a l i e s in t h e l o n g a r m of c h r o m o s o m e
microsurgical instruments a r e u s e d ( F i g . 7). 17 ( N F 1 ) a n d 22 ( N F 2 ) ( R i c c a r d i 1999). S o l i t a r y
M a c r o d a c t y l y c a n be treated by esthetic ray neurofibromas should probably be considered as
finger amputation w h e n involving only o n e finger h a m a r t o m a s a s w e l l , a s t h e r e is n o histological
presenting with gross deformity a n d j o i n t stiff- difference b e t w e e n solitary a n d multiple neuro-
ness s e c o n d a r y to bone involvement. When fibromas. Describing neurofibromas a s solitary
m a c r o d a c t y l y i n v o l v e s o n l y o n e s i d e of t h e finger, may be inappropriate, as other a b n o r m a l n e r v e s
with a banana-like a p p e a r a n c e , the involved digi- m i g h t b e f o u n d if m e a n s w e r e a v a i l a b l e b y w h i c h
tal n e r v e , t h e s u b c u t a n e o u s f i b r o m a s , a n d part of to examine the entire central and peripheral
t h e b o n e if f e a s i b l e , c a n b e r e m o v e d ( F i g . 8 ) . n e r v o u s s y s t e m s in a l i v i n g p e r s o n .
E x c i s i o n of m u l t i p l e n e u r o f i b r o m a s , b a s e d o n N e u r o f i b r o m a t o s i s is a bizarre d i s e a s e w h i c h
t h e i r h i g h e r i n c i d e n c e of m a l i g n a n c y , is not p r a c - c a n i n v o l v e m a n y o r g a n s in t h e b o d y . It c a u s e s
tical a n d h a r d l y f e a s i b l e . P a t i e n t s suffering f r o m abnormalities of t h e t i s s u e d e r i v a t i v e s of the

Figure 8

(a) Macrodactyly of the middle finger involving soft tissue and bone, mainly on its radial half. (b) Surgical exploration disclosed
an enlarged digital nerve which was excised, as a reconstruction with a nerve graft could not be performed. Histological
examination confirmed a neurofibroma. After nerve resection, skin and fibrous subcutaneous tissue were debulked for
esthetic purposes. The bone overgrowth on the radial side of the distal and middle phalanges was streamlined in order to
decrease its size. The radial collateral ligament of the distal interphalangeal (DIP) joint also had to be removed, and the DIP
joint was fused after moderate shortening of the middle and distal phalanges.
90 T U M O R S OF THE HAND

three g e r m layers: ectodermal structures such a s Bolande RP (1981) Neurofibromatosis - the quintessential
t h e skin a n d n e r v e s ; m e s o d e r m a l s t r u c t u r e s s u c h neurocristopathy, Adv Neurol 29:23-32.
a s b o n e a n d c o n n e c t i v e t i s s u e , in t h e f o r m of lipo-
m a s a n d f i b r o m a s ; a n d e v e n certain e n d o d e r m a l Butler ED, Hamill J P , Seipel R S et al (1960)Tumors of the
s t r u c t u r e s like i n t e s t i n e a n d v i s c e r a . T h e k a l e i d o - hand. A ten-year survey and report of 437 cases, Am J
Surg 100:293-303.
s c o p i c p r e s e n t a t i o n of n e u r o f i b r o m a t o s i s is diffi-
cult to understand, although it seems to be
D'Agostino A N , Soule E H , Miller RH (1963a) Primary
s e c o n d a r y t o a g e n e t i c d i s o r d e r in t h e g r o w t h of
malignant neoplasms of nerves (malignant neurilem-
c e l l s o r i g i n a t e d in t h e n e u r a l c r e s t , a s t h e m o s t
momas) in patients without manifestations of multiple
i m p o r t a n t d e v e l o p m e n t a l d i a t h e s i s o c c u r s at t h e neurofibromatosis (von Recklinghausen's disease),
neuroectodermal tissues: central and peripheral Cancer 16:1003-14.
n e r v o u s s y s t e m s , a s w e l l a s skin ( B o l a n d e 1981).
N e u r o f i b r o m a s d o not respond to radio- or D'Agostino A N , Soule E H , Miller RH (1963b) Sarcomas
quimiotherapy. Isolated cutaneous lesions can be of the peripheral nerves and somatic tissues associated
e x c i s e d for esthetic purposes. Large plexiform with multiple neurofibromatosis (von Recklinghausen's
neurofibromas invade nerve fibers, and cannot disease), Cancer 16:1015-27.
b e e x c i s e d w i t h o u t c a u s i n g a l o s s of n e r v e f u n c -
Ducatman B S , Scheithauer B W (1983) Postirradiation
t i o n . T h e m a i n i n d i c a t i o n for s u r g i c a l t r e a t m e n t is
neurofibrosarcoma, Cancer 51:1028-33.
s y m p t o m a t i c , s u c h a s t h e r e l e a s e of a c o m p r e s -
s i o n of t h e m e d i a n n e r v e in t h e c a r p a l t u n n e l . In
Ducroquet R (1927) A propos des pseudoarthroses et
s u m m a r y , the only possible surgical treatments
inflexions congénitales du tibia, Mem Acad Chir 63:
a r e m i n i m a l e x c i s i o n f o r t i s s u e d i a g n o s i s , partial
683-8.
e x c i s i o n to d e b u l k a l a r g e t u m o r m a s s , a n d w i d e
r e s e c t i o n w i t h n e r v e graft r e c o n s t r u c t i o n . Ennekin W F (1983) Musculoskeletal Tumor Surgery
Neurofibromas can undergo malignant degen- Churchill Livingstone: New York: 1271-302.
e r a t i o n into n e u r o f i b r o s a r c o m a s , p a r t i c u l a r l y in
p a t i e n t s suffering f r o m N F 1 . Enzinger F M , Weiss S W (1983) Soft Tissue Tumors. CV
Mosby: St. Louis: 580-656.

Enzinger F M , Weiss S W (1988) Malignant tumors of

References peripheral nerves. In: Enzinger F M , Weiss S W , eds. Soft
TissueTumors, 2nd edn. CV Mosby: S t . Louis: 781-815.
Akenside M (1768) Observations on cancers, MedTrans
Coll Physicians Lond 1:64. Friedlander HL, Rosenberg N J , Graubard D J (1969)
Intraneural lipoma of the median nerve, J Bone Joint
AllieuY, Gomis R, Yoshimura M et al (1981) Congenital Surg 51A:352-62.
pseudoarthrosis of the forearm - two cases treated
by free vascularized fibular graft, J Hand Surg 6A: 475-81. Glicenstein J , Ohana J , Leclercq C (1988) Tumors of
nerves. In: Tumors of the Hand. Springer-Verlag: Berlin:
Andersen K S (1973) Radiological classification of con- 121-35.
genital pseudoarthrosis of the tibia, Acta Orthop Scand
44:719-27. Gosh B C , Gosh L, Havos A G et al (1973) Malignant
schwannoma: a clinopathologic study, Cancer 3 1 :
Barber C G (1939) Congenital bowing and pseudoarthro- 184-90.
sis of lower leg: manifestations of von Recklinghausen's
neurofibromatosis, S u r g Gynecol Obstet 69:618-26. Gould E P (1918) The bone changes occurring in von
Recklinghausen's disease, Q J Med 11:221-8.
Beathy E (2000) Tissue differentiation of the upper
extremity. In: Gupta A , Kay S P J , Schecker LR, eds. The Grifith B H , McKinney P, Monroe C W , Howell A (1972)
Growing Hand. Mosby: London: 33-8. Von Recklinghausen's disease in children, Plast Reconstr
Surg 49:648.
Bertolotti P (1997) Neoplasie dei nervi periferici. In:
Bertolli P, ed. Tumori della mano. Editrice Liguria: Harkin J C (1980) Differential diagnosis of peripheral
Savona: 85-94. nerve tumors. In: O m e r G E , Spinner M , e d s .
 NEUROFIBROMA 91

Management of Peripheral Nerve Problems. WB Miller TT, Potter H G , McCormack RR (1994) Benign soft
Saunders Company: Philadelphia: 657-93. tissue masses of the wrist and hand; M R I appearances,
Skeletal Radiol 23:327-32.
Heard H G (1962) Nerve sheath tumors and v o n
Recklinghausen's disease of the central nervous system, Money RA (1950)Tumors of the peripheral nerves, Aust
Ann R Coll Surg 31:229. NZJ Surg 19:239-45.

Idler R S (1995) Benign and malignant nerve tumors, Morley GH (1964) Intraneural lipoma of the median
Hand Clin 11:203-9. nerve in the carpal tunnel, J Bone Joint Surg 46B:
734-5.
Iyer V G (2000) Development and maturation of the
nervous system. In: Gupta A , Kay S P J , Schecker LR, eds. Nascimento A G (2000) Pathology of soft tissue tumors.
The Growing Hand. Mosby: London: 47-51. In: Gupta A , Kay S P J , Schecker LR, eds. The Growing
Hand. Mosby: London: 857-72.
Jenkins S A (1952) Solitary tumours of peripheral nerve
trunks, J Bone Joint Surg 34B:401-11. Ollier (1901) Presented 2 cases of dyschrondroplasia,
Montpellier Medical School, 1899, Br Med J 1:184.

J o h n s o n R J , Bonfiglio M (1969) Lipofibromatous

hamartoma of the median nerve, J Bone Joint Surg Patel M E , Silver J W , Lipton DE, Pearlman H S (1979)
51A:984-90. Lipofibroma of the median nerve in the palm and digits
of the hand, J Bone Joint Surg 61A:393-7.

Klippel M,Trenaunay P (1900) Noevus variqueux ostéo-

Purcel F H , Gurdjian E S (1935) Hemangiomata of periph-
hypertrophique, J Pratitiens 14:65-70.
eral nerves, Am J Surg 30:541.

Léri A , J o a n n y J P (1922) U n e affection non décrite des

Riccardi V M , Mulvihill J J (1981) Neurofibromatosis (von
os: hiperostéose 'en coulie' sur toute la longeur d'un
Recklinghausen's disease), Adv Neurol 29:1-282.
membre ou 'mélorhéostose'. Bull Méd Hop Paris, 46:
1141-5.
Riccardi V M (1999) Historical background and intro-
duction. In: Friedman J M , Gutmann DH, MacCollin M ,
Losli E J (1952) Intrinsic hemangiomas of the peripheral
Riccardi V M , eds. Neurofibromatosis: Phenotype,
nerves, Arch Pathol 53:226-32.
Natural History, and Pathogenesis. J o h n s Hopkins
University Press: Baltimore: 1-25.
Louis D S (1987) Peripheral nerve tumors in the upper
extremity, Hand Clin 3:311-18.
Seddon H J (1972) Surgical Disorders of Peripheral
Nerves. Williams & W i l k i n s Co: Baltimore: 160-4.
Mackinnon S E , Dellon A L (1988) Neurofibromatosis. In:
Surgery of the Peripheral Nerve. Thieme Medical Seizinger BR, Houleau A , Ozelius L J et al (1987) Genetic
Publishers, Inc: N e w York: 535-49. linkage of von Recklinghausen neurofibromatosis to the
nerve growth factor receptor gene, Cell 49:589.
Mafucci A (1981) Di un caso enchondroma e angioma
multiple, Med Chir 3:399-412. Sellers D S , S o w a DT Moore J R et al (1988) Congenital
pseudoarthrosis of the forearm, J Hand Surg 13A:89-93.
Mathoulin C, Gilbert A , Azze R G (1993) Congenital
pseudoarthrosis of the forearm: treatment of six cases Shereff M J , Posner M A , Gordon M H (1980) Upper
with vascularized fibular graft and review of the litera- extremity hypertrophy secondary to neurofibromatosis:
ture, Microsurgery 14:252-9. case report, J Hand Surg 5:355-7.

Mattar R, Azze R J , Resende M R (2000) Congenital Sprague B L , Brown G A (1974) Congenital pseudo-
pseudoarthrosis of the forearm In: Gupta A , Kay S P J , arthrosis of the radius, J Bone Joint Surg 56A:191-4.
Scheker L R , eds. The Growing Hand. Diagnosis and
Management of the Upper Extremity in Children. Stack HG (1960) Tumours of the hand, Br Med J
Mosby: London: 1035-40. 1:919-22.

McKusickVA (1972) Heritable Disorders of the Connective Strickland J M , Steichen J B (1977) Nerve tumors of the
Tissue, 4th edn. CV Mosby Company: St. Louis: hand and forearm, J Hand Surg 2:285-91.
92 T U M O R S O F T H E HAND

Thompson A (1900) A Neuroma and Neurofibromatosis. von Deimling A , Krone W , M e n o n A G (1995)

Turnball and Speers: Edinburgh. Neurofibromatosis type 1 : pathology, clinical features
and molecular genetics, Brain Pathol 5:153-62.
Tsuge K, Ikuta Y (1973) Macrodactyly and fibro-fatty
proliferation of the median nerve, Hiroshima J Med Sei von Recklinghausen V F (1882) Lieber die multiplen
22:83-101. Fibrome der Haut und ihre Beziehung zu den multiplen
Neuromen. Verlag von August Hirschwald: Berlin.
Turkington R W , Grude EH (1964) Ehler-Danlos syndrome
with multiple fibromatosis, Ann Intern Med 61:549.
 10
Malignant peripheral nerve
sheath tumors
Chantal Bonnard

Introduction n e r v e . E x t r e m e l y rarely, t h e y m a y d e v e l o p in a
pre-existing schwannoma, ganglioneuroma, or
Malignant peripheral nerve sheath tumors ganglioneuroblastoma, or pheochromocytoma.
(MPNST) are defined as any malignant tumor The clinical and pathologic characteristics of
a r i s i n g f r o m o r differentiating into a cell t y p e t h o s e M P N S T differ s i g n i f i c a n t l y f r o m t h e m o r e
restricted to the peripheral nerve sheath c o n v e n t i o n a l M P N S T a n d w i l l not b e d i s c u s s e d
(Schwann cells a n d perineurial cells). T u m o r s h e r e ( S c h e i t h a u e r et al 1999).
arising f r o m the epineurium a n d f r o m peripheral
n e r v e v a s c u l a t u r e a r e excluded f r o m the defini-
t i o n ( K l e i h u e s a n d C a v e n e e 2000). O t h e r t e r m s
for MPNST are neurofibrosarcoma, malignant Histology and
s c h w a n n o m a , or n e u r o g e n i c s a r c o m a . F i v e per
c e n t of soft t i s s u e s a r c o m a s a r e M P N S T T h e inci-
i m mu noh istochem istry
d e n c e of M P N S T in t h e g e n e r a l clinic p o p u l a t i o n is M o s t M P N S T a r i s e f r o m S c h w a n n cells a s s u g -
0.001 per c e n t ( D u c a t m a n et al 1986). gested by the following features: histological
The association with von Recklinhausen's e v i d e n c e of n e u r o f i b r o m a w i t h i n or b e y o n d t h e
d i s e a s e ( n e u r o f i b r o m a t o s i s 1 , N F 1 ) is w e l l k n o w n : t u m o r , S-100 protein e x p r e s s i o n in cells of the
t h e p r e v a l e n c e o f M P N S T in N F 1 is 4.6 p e r c e n t m a j o r i t y of M P N S T , a n d ultrastructural c h a r a c t e r i s -
( D u c a t m a n et al 1986). T h e m o s t c o m m o n b e n i g n tics of S c h w a n n cells b e i n g p r e s e n t in t h e m o s t
t u m o r a s s o c i a t e d w i t h N F 1 is t h e neurofibroma. well-differentiated M P N S T ( W o o d r u f f 1999). In a
One subtype is t h e plexiform neurofibroma significant p r o p o r t i o n of M P N S T , t h e cells a r e s o
(16.8 p e r c e n t of p a t i e n t s w i t h N F 1 p r e s e n t a plex- a n a p l a s t i c that n o S c h w a n n cell differentiation c a n
i f o r m n e u r o f i b r o m a ) ( W a g g o n e r e t al 2 0 0 0 ) , t h a t b e f o u n d . Finally, in a m i n o r i t y of c a s e s , t h e cells
c a n g r o w to a n e n o r m o u s m a s s . W i t h e x t r e m e l y p r e s e n t w i t h t h e characteristics of fibroblasts or
rare e x c e p t i o n s , p l e x i f o r m n e u r o f i b r o m a is s e e n perineurial c e l l s . B e c a u s e of t h e g r e a t v a r i e t y of t h e
only in a s s o c i a t i o n w i t h NF1 (Woodruff 1999). t u m o r a l cell e x p r e s s i o n ( S c h w a n n c e l l s , a n a p l a s t i c
P l e x i f o r m n e u r o f i b r o m a s p r e s e n t a 5 per c e n t life- cells, fibroblasts, perineurial cells), t h e t e r m ' m a l i g -
t i m e risk o f t r a n s f o r m a t i o n into M P N S T ( R e e d a n d nant peripheral n e r v e s h e a t h t u m o r ' or ' M P N S T ' is
G u t m a n n 2001). A m o n g t h e n e u r o f i b r o m a s , o n l y p r e f e r a b l e to restrictive t e r m s including malignant
plexiform neurofibromas carry a significant s c h w a n n o m a or n e u r o f i b r o s a r c o m a .
potential of malignant transformation. Up to Macroscopically a M P N S T presents as a g r a y -
50-60 p e r c e n t of p a t i e n t s w i t h M P N S T suffer tan, firm, opaque mass whereas a neurinoma
f r o m N F 1 ( D u c a t m a n e t a l 1986). o r s c h w a n n o m a is soft a n d t r a n s l u c e n t . A r e a s o f
M o s t M P N S T arise either f r o m a neurofibroma n e c r o s i s a r e o b s e r v e d in 60 p e r c e n t of c a s e s . A
(plexiform) o r d e n o v o in a n o r m a l peripheral MPNST m a y b e f u s i f o r m or g l o b o i d , growing
94 T U M O R S OF THE HAND

c e l l s . T h e m o r e differentiated t h e t u m o r , t h e m o r e
p o s i t i v e t h e s t a i n i n g ; in h i g h - g r a d e undifferenti-
ated MPNST, the staining may be negative,
whereas it is a l w a y s p o s i t i v e in benign well-
differentiated tumors such a s s c h w a n n o m a or
neurofibroma.
Sometimes, ultrastructural findings may be
useful: w h e n cells are largely undifferentiated,
inconspicuous features may suggest Schwann
cell d i f f e r e n t i a t i o n s u c h a s r u d i m e n t a r y cell j u n c -
t i o n s o r w i s p s of b a s e m e n t m e m b r a n e w i t h i n t h e
i n t e r c e l l u l a r s p a c e ( S c h e i t h a u e r 1999).

Figure 1
Molecular genetics
Malignant peripheral nerve sheath tumor (MPNST) on a
popliteal nerve. Note the fusiform appearance of the nerve T h e N F 1 g e n e is l o c a t e d o n c h r o m o s o m e 17 a n d
and the presence of probably intact fascicles at the peri-
f u n c t i o n s a s a t u m o r s u p p r e s s o r g e n e . Its loss of
phery of the nerve. The mass is firm, opaque, and hyper-
expression predisposes to tumor formation
vascular suggesting a MPNST.
( n e u r o f i b r o m a ) . M o r e o v e r , half of M P N S T a l s o p r e -
s e n t m u t a t i o n s in a n o t h e r t u m o r s u p p r e s s o r (p53)
suggesting that multiple genetic events are
required for t h e p r o g r e s s i o n f r o m b e n i g n n e u r o -
eccentrically f r o m the parent nerve (increasing f i b r o m a to M P N S T ( S c h e i t h a u e r et al 1999, R e e d
the difficulty of the peroperative diagnosis a n d G u t m a n n 2001). P o s i t i v e s t a i n i n g for p53 is a
between benign schwannoma and MPNST) n e g a t i v e p r o g n o s t i c factor ( S c h e i t h a u e r et al 1999).
(Fig. 1). T h e M P N S T spreads within the nerve.
T h e r e f o r e it is i m p e r a t i v e that at t h e t i m e of
s u r g e r y frozen s e c t i o n a s s e s s m e n t of proximal
a n d distal n e r v e m a r g i n s s h o u l d b e u n d e r t a k e n . Clinical findings
Microscopically, the histological features are
m o r e v a r i e d t h a n in a n y o t h e r soft t i s s u e s t u m o r s . Localization of a M P N S T in t h e u p p e r e x t r e m i t y
Most M P N S T (85 per cent) are highly cellular, is v e r y rare. In fact, in t h e M a y o Clinic s e r i e s
spindle-cell proliferations (fibrosarcoma-like) ( D u c a t m a n et al 1986) of 120 c a s e s , 46 p e r c e n t of
with a high mitotic index a n d geographic necrosis MPNST a r o s e in t h e trunk, 34 per c e n t in the
( S c h e i t h a u e r et al 1999). O n l y a s m a l l p r o p o r t i o n extremities, a n d 20 per c e n t in t h e h e a d a n d neck
(15 p e r c e n t ) ( D u c a t m a n et al 1986) is l o w - g r a d e a r e a . T h e peripheral n e r v e that is m o s t often c o n -
p r e s e n t i n g a l w a y s w i t h p e r i n e u r i a ! cell differenti- c e r n e d is t h e sciatic n e r v e , f o l l o w e d b y t h e brachial
ation (Woodruff 1999). T h e d i s t i n c t i o n between plexus, the spinal n e r v e roots, v a g u s , f e m o r a l ,
'cellular neurofibroma', 'atypical neurofibroma', median, sacral plexus, popliteal, obturator,
and l o w - g r a d e M P N S T m a y be difficult.Three cri- posterior tibial, and ulnar nerves (Ducatman
teria a r e i m p o r t a n t t o s u g g e s t t h e d i a g n o s i s of et al 1986).The l o w e r e x t r e m i t y is i n v o l v e d t w i c e a s
l o w - g r a d e M P N S T : d e f i n i t e cell c r o w d i n g , g e n e r a l often a s t h e u p p e r extremity. In patients w i t h N F 1 ,
nuclear enlargement (more than three times the M P N S T h a s a m o r e c e n t r a l location t h a n in p a t i e n t s
size of o r d i n a r y n e u r o f i b r o m a n u c l e i ) , a n d h y p e r - w h o d o not h a v e N F 1 ( D u c a t m a n et al 1986).
c h r o m a s i a ( S c h e i t h a u e r e t al 1999). M P N S T presents as an enlarging tumor m a s s
W h e n a M P N S T s h o w s no association with a which may arise from a pre-existing neuro-
peripheral nerve, immunohistochemical studies fibroma, nearly a l w a y s w i t h pain a n d neurologic
may b e u s e f u l . S t a i n i n g for S - 1 0 0 p r o t e i n , L e u 7, deficit. In N F 1 , m o s t , if n o t a l l , M P N S T a r i s e f r o m
or the b a s e m e n t m e m b r a n e c o m p o n e n t s (colla- a n e u r o f i b r o m a ( F i g . 2 ) . T h e m a i n p r o b l e m is t h a t
gen type 4 a n d laminin) m a y be positive. This a plexiform neurofibroma also grows like a
positivity indicates the p r e s e n c e of Schwann M P N S T a n d m a y also be painful ( e s p e c i a l l y in
 MALIGNANT P E R I P H E R A L NERVE SHEATH T U M O R S 95

Figure 3

The volume of the mass In the popliteal nerve suggests a

malignant peripheral nerve sheath tumor.

Investigation
M a g n e t i c r e s o n a n c e i m a g i n g ( M R I ) d o e s no t reli-
ably distinguish malignant from non-malignant
tissue within a neurofibroma ( F i g . 3). MPNST
a p p e a r s like soft t i s s u e s a r c o m a s : l o w in s i g n a l
o n T 1 - w e i g h t e d i m a g e s a n d h i g h in stir s e q u e n c e s
with i n h o m o g e n e o u s e n h a n c e m e n t of contrast
Figure 2
a g e n t ( S t a r k et al 2001). C o m p u t e d t o m o g r a p h y
( C T ) s c a n is u s e f u l to e v a l u a t e infiltration of b o n e
Patient presenting with neurofibromatosis 1 with a malig-
nant peripheral nerve sheath tumor on her right proximal ( D u c a t m a n et al 1986). P o s i t r o n e m i s s i o n t o m o g -
forearm arising from a plexiform neurofibroma. r a p h y ( P E T ) is p r o b a b l y m o r e r e l i a b l e t h a n CT. In
(18) f l u o r o d e o x y g l u c o s e P E T , a n i n c r e a s e d u p t a k e
is f o u n d to b e c h a r a c t e r i s t i c of M P N S T but t h e r e
is overlap between some benign plexiform
n e u r o f i b r o m a s a n d M P N S T ( F e r n e r e t al 2 0 0 0 ) .
response to t r a u m a ) . M o r e o v e r , the M P N S T m a y E l e c t r o m y o g r a p h y ( E M G ) is u s e f u l to e v a l u a t e t h e
c o n c e r n o n l y a s m a l l p o r t i o n of t h e n e u r o f i b r o m a , neurologic deficit. When the diagnosis of
so that a biopsy could m i s s the malignant d e g e n - M P N S T is c e r t a i n , a n a b d o m i n a l a n d t h o r a c i c C T
e r a t i o n ( K o r f 2000). s c a n is i n d i c a t e d l o o k i n g f o r e i t h e r l u n g o r l i v e r
T h e a s s o c i a t i o n of a p a i n f u l growing mass metastases.
w i t h n e u r o l o g i c deficit is s u g g e s t i v e of t h e p o s -
sibility of a M P N S T . Initially, p a i n is n o c t u r n a l a n d
at t h e l e v e l of t h e m a s s , but m o r e often in t h e
sensory distribution of the nerve. Later, p a i n Treatment
b e c o m e s persistent, with progressive neurologic
deficit. S o m e t i m e s p a i n l i m i t s j o i n t m o v e m e n t . The preoperative diagnosis of MPNST is not
O n e of m y c a s e s presented with a s p o n t a n e o u s a l w a y s c e r t a i n a n d it is e x c e s s i v e to s a c r i f i c e a
flexum of the knee, the MPNST w a s on the functional n e r v e for r e s e c t i o n of a t u m o r which
popliteal n e r v e ( F i g . 3) a n d w a s s t r e t c h e d with a p p e a r s later to b e b e n i g n ! T h u s , it is r e c o m -
knee extension causing violent pain. Pain appears m e n d e d that either a preoperative n e e d l e biopsy
t o b e t h e m o s t r e l i a b l e clinical i n d i c a t o r of m a l i g - (under C T visualization) or a n open oncologic
n a n t d e g e n e r a t i o n ( R o s s e r a n d P a c k e r 2002). biopsy should be performed when, during
96 T U M O R S O F T H E HAND

Figure 4

The same patient as in Figure 2. After

seven local recurrences, the patient has
been amputated. A few months later,
recurrence occurred on the stump extrem-
ity. The patient died 3 years later, after
shoulder amputation with a proximal
tumor infiltrating the brachial plexus and
the thorax.

s u r g e r y , t h e d i a g n o s i s of M P N S T is s u s p e c t e d . It performed (with free margins), the prognosis can

is i m p o r t a n t t o understand that excision of a b e f a v o r a b l e . In a l a r g e s e r i e s o f D u c a t m a n e t a l
MPNST as if it was a schwannoma with open (1986) of 120 patients, only seven patients
intraneurial dissection will lead to dissemination c o r r e s p o n d e d t o t h e s e criteria (MPNST in the
of high-grade MPNST. distal part of a l i m b a n d ' e n b l o c ' r e s e c t i o n w i t h
T h e d i a g n o s i s of M P N S T b e i n g c e r t a i n , a w i d e f r e e m a r g i n s ) . T w o ( o u t of s e v e n ) d i e d of m e t a s -
'en b l o c ' p r o c e d u r e s h o u l d b e p e r f o r m e d w i t h at t a s e s , o n e d i e d t u m o r - f r e e after 14.5 y e a r s , a n d
least 5-cm r e s e c t i o n of t h e n e r v e o n e a c h s i d e of four were alive and well after an average
t h e t u m o r . T h e p e r o p e r a t i v e frozen s e c t i o n e x a m i - f o l l o w - u p of 5.7 y e a r s . T h e s u r v i v a l rate at 5 y e a r s
n a t i o n is not sufficiently reliable to limit t h e n e r v e in M P N S T w i t h o u t N F 1 w a s 53 p e r c e n t in t h e
r e s e c t i o n to t h e t u m o r itself a n d g i v e n t h e p o s s i - s e r i e s of D u c a t m a n et al (1986) (58 c a s e s ) , a n d 50
bility of i n t r a n e u r i a l s p r e a d , s e c u r i t y m a r g i n s (of per cent in the s e r i e s of Birch et al (1998)
at least 5 c m ) a r e i m p e r a t i v e . S o m e t i m e s , a m p u - (26 c a s e s ) .
t a t i o n is n e e d e d . F o r t h e s a m e r e a s o n , local a d j u - W h e n M P N S T is p r e s e n t in p a t i e n t s suffering
v a n t irradiation at a total d o s e of at least 60 G y f r o m a N F 1 , p r o g n o s i s is w o r s e w i t h a 5 - y e a r s u r -
including brachytherapy and intraoperative elec- v i v a l rate of 16-30 p e r c e n t ( D u c a t m a n et al 1986,
t r o n irradiation a r e often u n d e r t a k e n to improve B i r c h e t al 1 9 9 8 ) . T h e r e a s o n is p r o b a b l y multifac-
local c o n t r o l of t h e d i s e a s e ( W o n g et al 1998). t o r i a l : in N F 1 a s s o c i a t e d M P N S T , t h e l o c a t i o n is
T h e difficulty of d i a g n o s i s , i n t e r p r e t a t i o n of m o r e often c e n t r a l (57 per c e n t v e r s u s 36 per c e n t ) ,
preoperative investigations ( M R I , P E T ) , and c o m - and the tumor is l a r g e r (57 p e r c e n t > 5 c m in
plexity of t r e a t m e n t ( e x c i s i o n ' e n b l o c ' o n c o l o g i c , p a t i e n t s w i t h N F 1 v e r s u s 38 p e r c e n t in t h o s e
postoperative radiotherapy, reconstruction) w i t h o u t N F 1 ) ( D u c a t m a n et al 1986).
require a multidisciplinary approach with a t e a m L o c a l r e c u r r e n c e o c c u r s in 42 p e r c e n t of c a s e s ,
including s u r g e o n , radiologist, oncologist, and and more frequently (45 p e r c e n t ) in patients
radiotherapist. with N F 1 than in those with solitary MPNST
(38 p e r c e n t ) ( F i g . 4 ) . W h e n e x c i s i o n i s i n c o m -
plete, recurrence o c c u r s w i t h i n a f e w m o n t h s w i t h
ultimately distant m e t a s t a s e s resulting in d e a t h
Results, recurrence, ( D u c a t m a n et al 1986). D i s t a n t metastases are
and metastasis c o m m o n ( 4 0 - 8 0 p e r c e n t ) ( D u c a t m a n e t al 1986,
S t a r k et al 2 0 0 1 , R o s s e r a n d P a c k e r 2002). T h e y
W h e n t h e M P N S T a r i s e s in t h e distal part of t h e are most frequently found in t h e l u n g , lymph
e x t r e m i t y a n d w h e n a n 'en b l o c ' r e s e c t i o n c a n b e n o d e s , a n d liver.
 MALIGNANT P E R I P H E R A L NERVE SHEATH T U M O R S 97

Negative prognostic factors of statistically Ducatman B S , Scheithauer B W , Piepgras DG et al (1986)

significant v a l u e a r e p r e s e n c e of N F 1 disease Malignant peripheral nerve sheath tumors, Cancer
( p < 0.001), tumor size > 5 c m (p< 0.005), and 57:2006-21.
extent of resection (p<0.01). As might be
expected, patients with N F 1 disease and large Ferner R E , Lucas J D , O'Doherty M J et al (2000)
Evaluation of (18)fluorodeoxyglucose positron emission
t u m o r s h a d a w o r s e p r o g n o s i s : 5 - y e a r s u r v i v a l of
tomography in the detection of malignant peripheral
9 p e r c e n t ( D u c a t m a n e t a l 1986). P a t i e n t s w h o
nerve sheath tumours arising from within plexiform
h a d u n d e r g o n e total e x c i s i o n of a s m a l l tumor neurofibromas in neurofibromatosis 1 , J Neurol Neuro-
h a d a statistically s i g n i f i c a n t improved survival surg Psychiatry 68:353-7.
( D u c a t m a n e t a l 1986).
Kleihues P, C a v e n e e W K (2000) Pathology and Genetics
of Tumours of the Nerve System. IAARC Press: Lyon.

Conclusions Korf BR (2000) Malignancy in neurofibromatosis type 1 ,

Oncologist 5:477-85.
T h e h a n d s u r g e o n w i l l b e v e r y rarely c o n c e r n e d
w i t h a M P N S T A painful e n l a r g i n g m a s s w i t h n o c - Reed N, Gutmann DH (2001) Tumorigenesis in neuro-
turnal pain (like in c a n a l a r s y n d r o m e s ) s u g g e s t s a fibromatosis: new insights and potential therapies,
M P N S T . T h e probability i n c r e a s e s w h e n t h e r e is a Trends Mol Med 7:157-62.
neurologic deficit or if t h e patient h a s clinical
s i g n s of N F 1 including 'café-au-lait' m a c u l e s , ( s u b ) - Rosser T, Packer R J (2002) Neurofibromas in children
c u t a n e o u s n e u r o f i b r o m a s , a n d f a m i l y history. with neurofibromatosis 1 , J Child Neurol 17:585-91.
W h e n a M P N S T is s u s p e c t e d , it is i m p e r a t i v e
to confirm the d i a g n o s i s preoperatively either by Scheithauer B W , Woodruff J M , Erlandson RA (1999)
Tumors of the Peripheral Nervous System. Armed
needle biopsy or o p e n biopsy because a w e l l -
Forces Institute of Pathology: Washington, DC: 303-72.
p l a n n e d ' e n b l o c ' e x c i s i o n is t h e o n l y c h a n c e f o r
l o n g - t e r m s u r v i v a l . Partial e x c i s i o n o r p e r o p e r a -
Stark A M , Buhl R, Hugo HH, Mehdorn H M (2001)
tive dissemination increases the risk of recur-
Malignant peripheral nerve sheath tumours, Acta
rence a n d metastases.
Neurochir 143:357-64.
If t h e diagnosis of MPNST is e v o k e d only
d u r i n g s u r g e r y , t h e b e s t attitude is to s t o p d i s s e c - Waggoner D J , Towbin J , Gottesman G , Gutmann DH
tion (avoid intraneurial or intratumoral dissec- (2000) Clinic-based study of plexiform neurofibromas in
tion), do a v e r y sharp oncologic biopsy, and await neurofibromatosis 1 , Am J Med Genet 92:132-5.
c o n f i r m a t i o n of t h e m a l i g n a n c y . A w i d e l o c a l ' e n
b l o c ' e x c i s i o n w i l l b e p e r f o r m e d later. W o n g W W , Hirose T, Scheithauer B W et al (1998)
Malignant peripheral nerve sheath tumor: analysis of
treatment outcome, Int J Radiat Oncol Biol Phys
42:351-60.
References
Woodruff J M (1999) Pathology of tumors of the peri-
Birch R, Bonney G , W y n n Parry C (1998) Surgical Dis- pheral nerve sheath in type 1 neurofibromatosis, Am J
orders of Peripheral Nerves. Churchill Livingstone, Med Genet 89:23-30.
London: 358-68.
 11
Vascular tumors: classification
and surgery
Antoine Lavie, Régis Legre and Guy Magalon

History Terminology
T h e h i s t o r y of v a s c u l a r a n o m a l i e s of t h e h a n d Terminology in the m a n a g e m e n t of vascular
b e g a n in 460 BC w h e n H i p p o c r a t e s d e s c r i b e d t h e t u m o r s of t h e h a n d is a m a j o r p r o b l e m for m o s t
first arterial a n e u r y s m of t h e h a n d . G u a t t a n i , in physicians facing these pathologies. Historic
1772, d e s c r i b e d t h e first c a s e of u l n a r a n e u r y s m t e r m i n o l o g y r e s u l t e d in t h e u s e of t h e i m p r e c i s e
after r e p e a t e d t r a u m a s . A m a j o r a d v a n c e in t h e word 'angiomas' which should no longer be
understanding of vascular tumors came from used. T h e term angioma included most vascular
S u c q u e t a n d H o y e r w h o d e s c r i b e d , in 1862, t h e t u m o r s that w e r e i m p r o p e r l y d i a g n o s e d : h e m a n -
anatomy of an a r t e r i o v e n o u s fistula. Virchow giomas, vascular malformations, lymphan-
d e s c r i b e d t h e a r c h i t e c t u r e a n d o r g a n i z a t i o n of all g i o m a s , telangiectasias, etc.
vascular birthmarks a n d categorized t h e m as Co-operating w i t h a c o m m i t t e e of physicians
' a n g i o m a s ' in 1863. K l i p p e l a n d T r e n a u n a y , a n d i n t e r e s t e d a n d i n v o l v e d in v a s c u l a r t u m o r s of t h e
Parkes and W e b e r described s y n d r o m e s including h a n d , M u l l i k e n a n d G l o w a c k i e s t a b l i s h e d , in 1982,
vascular tumors in 1900 and 1907. W e i s m a n , a classification based on biological, physical,
b a s e d o n e t i o p a t h o g e n i c criteria, e s t a b l i s h e d t h e h i s t o l o g i c a l , a n d pattern of g r o w t h of v a s c u l a r
first c l a s s i f i c a t i o n of t u m o r s of t h e h a n d in 1959. a n o m a l i e s . M u l l i k e n a n d G l o w a c k i w e r e t h e first
T h e s e q u e n c e of t h e s e e v e n t s led t o t h e c o m p l e t e to initiate a n i n t e r n a t i o n a l w o r k s h o p o n v a s c u l a r
k n o w l e d g e of v a s c u l a r a n o m a l i e s of t h e h a n d in a n o m a l i e s , w h i c h w a s a n a r e a of g r o w i n g interest
t h e e a r l y 1980s. M a n y v a r i o u s t r e a t m e n t s w e r e for m a n y p h y s i c i a n s f r o m s e v e r a l m e d i c a l d i s c i -
t r i e d a n d p e r f o r m e d w i t h v a r i a t i o n s in s u c c e s s p l i n e s w o r l d w i d e . T h i s led t o t h e c r e a t i o n of t h e
(Courbier 1988). T u r n e r , in his 1714 treatise, International Society for Study of Vascular
f a v o r e d surgical resection w i t h legation of super- A n o m a l i e s ( I S S V A ) in 1992.
ficial angiomas. Many physicians, such as U s i n g t h i s c l a s s i f i c a t i o n a s a m a j o r r e f e r e n c e in
Wardrop, Gross, K i n g s t o n , etc., t r i e d artificial handling and managing vascular anomalies
u l c e r a t i o n w i t h v a r i o u s c a u s t i c s at t h e b e g i n n i n g h e l p e d u s to d i s t i n g u i s h clearly 'true vascular
of t h e 19th c e n t u r y . T h e n e l e c t r o l y s i s , t h e r m o - tumors' from 'vascular malformations' which
c a u t e r y , s c l e r o s a n t t h e r a p y , a n d r a d i a t i o n led to differ in m a n y f u n d a m e n t a l c h a r a c t e r i s t i c s . T h u s ,
d e f e a t o r i n c o m p l e t e r e s u l t s in t h e t r e a t m e n t of talking a b o u t v a s c u l a r t u m o r s w i t h o u t t a l k i n g of
vascular tumors. C o h e r e n t a n d efficient treat- vascular malformations w o u l d be an incomplete
m e n t s w e r e first p e r f o r m e d in t h e 1970s w i t h t h e a p p r o a c h to v a s c u l a r a n o m a l i e s of t h e h a n d . ' T r u e
understanding of development of vascular v a s c u l a r t u m o r s ' a r e u n d e r s t o o d to b e ' h e m a n -
t u m o r s , a n d k n o w l e d g e of e v o l u t i o n of different g i o m a s ' w h i c h possess the proper characteristics
vascular tumors. a n d criteria d e f i n i n g a t u m o r . T h e c l a s s i f i c a t i o n of
100 T U M O R S O F T H E HAND

the International S o c i e t y for S t u d y of V a s c u l a r w e r e f i n a l l y a b l e to e s t a b l i s h t h e s a m e d i a g n o s i s

A n o m a l i e s in 1996 at t h e J u n e w o r k s h o p in R o m e , and speak the s a m e language.
Italy was declined as follow (Enjolras and I m p o r t a n t criteria in t h e d i a g n o s i s of v a s c u l a r
M u l l i k e n 1998): a n o m a l i e s a r e i n c l u d e d in t h i s c l a s s i f i c a t i o n f r o m
microscopic to macroscopic features. First,
(1) Hemangiomas:
microscopic features such as mitosis r a t e , cell
(a) Superficial (tuberous); n a t u r e a n d a r c h i t e c t u r e , a n d s e c r e t i o n of f a c t o r s
(b) Subcutaneous; a r e u s e d to d i s t i n g u i s h t u m o r s f r o m a n o m a l i e s .
(c) Combined forms. T u m o r s a r e d e f i n e d b y a n e l e v a t e d m i t o s i s rate
( p r o l i f e r a t i o n or h y p e r p l a s i a ) , w h i l e v a s c u l a r m a l -
(2) Malformations: formations present a normal or slightly elevated
(a) Capillary; mitosis rate ( d y s p l a s i a ) . A n u m b e r of f e a t u r e s
(b) Venous; such a s physical examination, symptoms, and
(c) Lymphatic; e v o l u t i o n a r e a l s o i n c l u d e d in t h e d i a g n o s i s . T h e
(d) Arterials; c l a s s i f i c a t i o n r e f e r s to t h e r e s u l t s of c o m p l e m e n -

(e) Arteriovenous; tary explorations to establish the diagnosis.

(f) C o m p l e x or c o m b i n e d forms. T w o major explorations, ultrasonography with

color Doppler and magnetic resonance imaging
A l l of t h e s e v a s c u l a r a n o m a l i e s a r e c o n s i d e r e d t o ( M R I ) ( w i t h or w i t h o u t i n j e c t i o n ) h a v e b e c o m e a

b e b e n i g n b e c a u s e of t h e i r e v o l u t i o n a n d n o n - significant part of the diagnosis of vascular

lethal characteristics. H o w e v e r , m a n y different malformations. Internal blood circulation is a

f o r m s of m a l i g n a n t v a s c u l a r t u m o r s exist, w i t h a m a j o r c h a r a c t e r i s t i c of v a s c u l a r a n o m a l i e s . U l t r a -
l o w e r i n c i d e n c e . V a s c u l a r a n o m a l i e s of t h e h a n d sonography with Doppler techniques enables
will be mentioned as benign vascular tumors a n o m a l i e s with 'slow-' a n d 'fast-flow' circulations

(hemangiomas), vascular malformations (includ- to b e d i s t i n g u i s h e d . M R I is u s e d in t h e d i a g n o s i s

ing c o m b i n e d or c o m p l e x f o r m s ) , a n d m a l i g n a n t of v a s c u l a r a n o m a l i e s w h e n it is n e c e s s a r y t o
v a s c u l a r t u m o r s of t h e h a n d . explore local or regional extension. M R I angio-
T h e localization of v a s c u l a r a n o m a l i e s to t h e g r a p h y w i t h injection of g a d o l i n i u m e n a b l e s m o r e
h a n d is not f r e q u e n t c o m p a r e d to h e a d , neck, o r detailed i m a g e s to be obtained, w i t h vasculariza-
trunk. In 1974, M a g a l o n d e s c r i b e d a r e v i e w of t i o n a r c h i t e c t u r e of t h e a n o m a l y .
1000 v a s c u l a r a n o m a l i e s i n c l u d i n g 135 localized Arteriography or phlebography is n o longer
to the upper limb, w h i c h represented approxi- performed routinely for the diagnosis of
mately 10 p e r c e n t . T h i s statistic is commonly v a s c u l a r a n o m a l i e s . It m a y o n l y b e i n d i c a t e d in
a d m i t t e d in t h e i n c i d e n c e of v a s c u l a r a n o m a l i e s t h e p r e o p e r a t i v e m a n a g e m e n t of s p e c i f i c v a s c u -
of t h e h a n d . It r e p r e s e n t s a p p r o x i m a t e l y 10 p e r lar malformations (mostly arteriovenous). The
c e n t of all v a s c u l a r a n o m a l i e s , a n d t h e life i m p a c t classification using ultrasonography and color
of t h e s e a n o m a l i e s is m a j o r b e c a u s e of t h e h a n d - D o p p l e r u l t r a s o n o g r a p h y is a s s h o w n in T a b l e 1
icap they often create. Moreover, the major ( H u t c h i n s o n 1993).
i n c i d e n c e of v a s c u l a r a n o m a l i e s of t h e h a n d in Malignant vascular anomalies cannot be
childhood complicates the management. i n c l u d e d in t h i s c l a s s i f i c a t i o n b e c a u s e of a t y p i c a l
and non-specific characteristics.

Classification
Hemangiomas
The classification of vascular anomalies w a s
officially a d o p t e d in 1996 at t h e I S S V A round H e m a n g i o m a s a r e k n o w n a s 'capillary', 'cellular'
table. This enabled coherent communication or ' h y p e r t r o p h i c ' angiomas. Hemangiomas are
b e t w e e n different m e d i c a l s p e c i a l i s t s o n t h e d i a g - a l s o k n o w n a s ' i m m a t u r e a n g i o m a s ' o p p o s e d to
n o s i s of v a s c u l a r a n o m a l i e s w h i c h w a s a m a j o r 'mature a n g i o m a s ' as represented by vascular
improvement. Plastic s u r g e o n s , pediatric physi- malformations. Hemangiomas are the most
cians, anatomopathologists, or dermatologists common tumors during infancy. Hemangioma
 VASCULAR T U M O R S : CLASSIFICATION AND S U R G E R Y 101

Table 1 Classification based on ultrasonography and color plastic s u r g e o n t o e s t a b l i s h a n e a r l y a n d r e g u l a r

Doppler ultrasonography follow-up of t h e t u m o r . D u r i n g t h e first y e a r of
life, h e m a n g i o m a will represent a threatening
Hemangiomas
e v e n t for p a r e n t s b e c a u s e of t h e rapid s p e e d of
Slow flow (most often)
e x t e n s i o n of t h e t u m o r e v e n if t h e s u r g e o n tries
Fast flow (very active forms)
to r e a s s u r e t h e m .
Malformations T h e g r o w t h of a h e m a n g i o m a c a n b e s e p a r a t e d
Capillary: slow flow into t h r e e p h a s e s : proliferative p h a s e , ' s t e a d y - s t a t e '
Venous: slow flow
p h a s e , a n d t h e n involutive p h a s e . T h e proliferative
Lymphatic: no flow
p h a s e lasts f r o m birth to t h e e n d of t h e first y e a r of
Arterial: fast flow
Arteriovenous: fast flow life. H e m a n g i o m a h a s a rapid a n d e x t e n s i v e g r o w t h
Combined or complex forms: slow or fast flow to reach a m a x i m u m a r o u n d 12 m o n t h s . T h i s p h a s e
is c o n s i d e r e d to b e characteristic of h e m a n g i o m a .
B i o l o g i c a l s t u d y s h o w s that t h e proliferative p h a s e
is d e f i n e d by a proliferation of endothelial cells w i t h

c a n b e localized in a n y part of t h e b o d y , h o w e v e r , virtual l u m e n s joining into capillaries a s a solid

h a n d or u p p e r l i m b localization r e q u i r e a c o m p l e t e m a s s . T h e cells present a n important endocrinal

u n d e r s t a n d i n g of v a s c u l a r a n o m a l i e s to a v o i d a n activity with s e c r e t i o n of von Willbrand factor

incorrect d i a g n o s i s . S u p p o r t e d b y t h e I S S V A c l a s - ( v W F ) , basic fibroblast g r o w t h factor ( b F G F ) , v a s c u -

sification, the diagnosis of cavernous heman- lar endothelial growth factor (VEGF), urokinase,

gioma should disappear from the scientific a n d proliferative cell nuclear a n t i g e n ( P C N A ) ( P a s y k

l a n g u a g e . T h i s classification avoids the u s e of 1987, G o n z a l e s - C r u s s i a n d Reyes-Mugica 1991,

t e r m s that c o n f u s e t h e d i a g n o s i s of h e m a n g i o m a s Takahashi et al 1994).The mitosis rate in endothelial

with vascular malformations. Hemangiomas cells is highly e l e v a t e d during this p h a s e . T h e pro-

n e v e r a p p e a r in a d u l t s . T h e s e t u m o r s s h o w a 3 : 1 liferative p h a s e is t h e m o s t delicate p h a s e b e c a u s e

f e m a l e to m a l e ratio. of potential c o m p l i c a t i o n s . M o s t h e m a n g i o m a s d o

Hemangiomas, tumors of infancy are often not have complications, however, ulceration,

n o t i c e d first in n u r s e r y but a r e not a l w a y s c o n - b l e e d i n g , or c o m p r e s s i o n a r e p o s s i b l e .

genital. H e m a n g i o m a s are precisely defined by a A t t h e e n d of t h e first y e a r of life, w h e n a

predictable natural c o u r s e . hemangioma h a s r e a c h e d its most expressive
o b v i o u s a p p e a r a n c e , t h e ' s t e a d y state', a n i n t e r m e -
d i a r y p h a s e b e g i n s ; this c a n last until 3 y e a r s of
a g e . D u r i n g this p h a s e , a h e m a n g i o m a d o e s not
p r e s e n t s e r i o u s m o d i f i c a t i o n s a n d retains its e n d -
Evolution and growth of-first-phase appearance. Studies have demon-
of hemangioma strated that this s t e a d y state p h a s e is, in fact,
a n e a r l y involution of t h e t u m o r w i t h cell c o u n t
W h a t e v e r appearance a hemangioma presents, d e c r e a s e a n d architectural m o d i f i c a t i o n s . T h e p r e s -
the natural pattern of growth remains almost e n c e of m a s t c e l l s , a s i m p l i c a t e d in f i b r o u s e v o l u -
c o n s t a n t . T h i s c r i t e r i o n is v e r y u s e f u l in t h e c a s e tion, is not y e t totally u n d e r s t o o d ( P a s y k et al 1984).
of a t y p i c a l a p p e a r a n c e of a h e m a n g i o m a . The involutive p h a s e , the third part of the
H e m a n g i o m a m o s t often a p p e a r s a f e w w e e k s t y p i c a l e v o l u t i o n of h e m a n g i o m a , really b e g i n s
( s e c o n d to f o u r t h w e e k ) after birth a s a s m a l l e r y - at t h e a g e of 3 o r 4. C l i n i c a l a s p e c t is d e f i n e d b y
t h e m a t o u s m a c u l a r p a t c h , a b l a n c h e d s p o t , or a d e c r e a s e of t h e size a n d v o l u m e of t h e tumor,
localized t e l a n g i e c t a s i a ( M u l l i k e n a n d Y o u n g 1988). s h o w i n g p a l e c o l o r s . T h e d e c r e a s e in c o m p l i c a -
Congenital h e m a n g i o m a represents one-third of t i o n s s u c h a s b l e e d i n g or u l c e r a t i o n is a p o s i t i v e
c a s e s . N o t all h e m a n g i o m a s a r e n o t i c e d at birth; sign of involution. Some authors explain this
m a n y s e r i e s report d e l a y s in a p p e a r a n c e but t h e n a t u r a l r e g r e s s i o n a s t h e result of m u l t i p l e t h r o m -
first t o f o u r t h w e e k s e e m s t h e m o s t c o m m o n . bosis and infarctions (MacCollum and Martin
When d i a g n o s e d by pediatric physicians or 1956, M a t t h e w s 1968).
o b s t e t r i c i a n s , h e m a n g i o m a localized to t h e h a n d T h e involution p h a s e lasts until a g e 7 w h e n
m u s t b e quickly r e p o r t e d to a h a n d s u r g e o n or m a x i m a l r e g r e s s i o n is r e a c h e d . In a f e w p a t i e n t s ,
102 T U M O R S O F T H E HAND

a b c

Figure 1

Progressive involution of a superficial hemangioma of the forearm.

tumors only partially disappear, however, in T h e I S S V A classification distinguishes b e t w e e n

g e n e r a l , total r e g r e s s i o n is t h e n o r m . D e f o r m a - t h e t h r e e m a i n f o r m s of h e m a n g i o m a s :
tion c a n persist, especially for c o m b i n e d forms
(1) S u p e r f i c i a l : localized in d e r m i s ;
(superficial plus s u b c u t a n e o u s c o m p o n e n t ) a s a
(2) S u b c u t a n e o u s : localized in h y p o d e r m i s ;
s m o o t h d e p r e s s i o n . T h e m o s t f r e q u e n t s e q u e l a is
(3) Mixed form: localized b o t h in d e r m i s and
a persistent fibrous tissue with pale color as a
hypodermis.
' t a t t o o ' o f t h e h e m a n g i o m a ( F i g . 1).
F o l l o w - u p is f u n d a m e n t a l in t h e m a n a g e m e n t of Superficial h e m a n g i o m a , k n o w n also a s 'tuber-
h e m a n g i o m a b e c a u s e of t h e predictable natural o u s ' , ' s t r a w b e r r y ' , or ' c a p i l l a r y ' h e m a n g i o m a is
c o u r s e of g r o w t h . A n y c h a n g e o r m o d i f i c a t i o n in t h e m o s t c o m m o n f o r m . It p r e s e n t s w i t h a s p e -
t h e pattern of g r o w t h w i l l alert t h e p h y s i c i a n to cific appearance: vivid red or purple well-
re-evaluate the diagnosis. G o o d knowledge of circumscribed, finely b o s s e d like a s t r a w b e r r y ,
hemangiomas allows aggressive therapy or flexible, m o b i l e , a n d painless ( F i g . 2).
s u r g e r y to b e a v o i d e d in m o r e t h a n 90 p e r c e n t Localization t o t h e h a n d is h a z a r d o u s , w i t h o u t
of c a s e s . A c t u a l l y , s u r g e r y is o n l y i n d i c a t e d for topographic p r e f e r e n c e s b e c a u s e of t h e lack of
non-typical f o r m s with complications or partial correlation w i t h t h e v a s c u l a r i z a t i o n . C o m p l e m e n -
r e g r e s s i o n , a s w i l l b e s e e n later. t a r y e x p l o r a t i o n s a r e u s u a l l y not n e c e s s a r y for t h e
diagnosis.
S u b c u t a n e o u s h e m a n g i o m a is localized in t h e
Clinical examination h y p o d e r m i s . Its a s p e c t is a c y s t i c t u m o r c o v e r e d
w i t h n o r m a l s k i n . S k i n c a n s o m e t i m e s b e lightly
A n a s c e n t h e m a n g i o m a a p p e a r s like a p u n c t i - blue. S u b c u t a n e o u s h e m a n g i o m a s are usually
f o r m , r e d d i s h m a c u l a m o s t of t h e t i m e . It m a y painless except when compressing vascular
sometimes appear as a pale halo around a tissue ( R a y n a u d s y n d r o m e or ischemia) rarely or
blanched lesion. Rapid growth during the neona- collateral n e r v e s (paresthesia) ( F i g . 3).
tal p e r i o d w i l l l e a d to a w e l l - c i r c u m s c r i b e d l e s i o n D i a g n o s i s of a s u b c u t a n e o u s f o r m c a n b e m o r e
w i t h a vivid red color within a f e w w e e k s . difficult a n d s o m e t i m e s r e q u i r e s u l t r a s o n o g r a p h y
 VASCULAR T U M O R S : CLASSIFICATION AND S U R G E R Y 103

Figure 2 Figure 3

Tuberous hemangioma of the dorsum on baby. Subcutaneous hemangioma of the palmar face of the hand
on a child.

with color Doppler and M R I . Ultrasound will p r o v i d e s t r u e d i a g n o s i s , but s h o u l d b e a v o i d e d

d e m o n s t r a t e a s l o w - f l o w lesion w i t h c a p i l l a r y p r e - w h e n e v e r possible.
d o m i n a n c e . M R I in this f o r m is helpful to deter-
m i n e the d e e p extension a m o n g hand structures
in r a r e c a s e s of p r e o p e r a t i v e p l a n n i n g . T h o s e c o m - Complications
p l e m e n t a r y e x p l o r a t i o n s a r e a l s o helpful in c a s e of
doubt with a venous malformation, which may C o m p l i c a t i o n s o c c u r in l e s s t h a n 5 p e r c e n t of
have sometimes-similar aspect. hemangiomas (Margileth and Museles 1965).
Mixed forms (both d e r m i c a n d hypodermic) T h e i r o c c u r r e n c e is m o s t c o m m o n in t h e prolifer-
can be confused with vascular malformations a t i v e p h a s e . A n u m b e r of different c o m p l i c a t i o n s
b e c a u s e of t h e v a r i o u s a s p e c t s w h i c h a r e c o m m o n are possible.
to s u p e r f i c i a l a n d s u b c u t a n e o u s h e m a n g i o m a s . U l c e r a t i o n is t h e m a i n c o m p l i c a t i o n ; it r e s u l t s
P h y s i c a l e x a m i n a t i o n is t h e m a i n t o o l for d i a g - f r o m e p i d e r m i s e r o s i o n in r e g a r d of t h e tumor
n o s i s of h e m a n g i o m a s , a n d to a l l o w p h y s i c i a n s a n d is often t h e c o n s e q u e n c e of a c h r o n i c a b r a -
to avoid complementary explorations in most s i o n ( f i n g e r s a n d d o r s u m of t h e h a n d ) . R a r e l y
cases. Ultrasonography with color Doppler and does ulceration l e a d to b l e e d i n g or s e c o n d a r y
M R I c a n b e v e r y h e l p f u l in u n c e r t a i n o r c o m p l e x infection. T h e ultimate evolution of ulceration
forms. Histological examination from biopsy is n e c r o s i s but t h i s should b e r a r e if nursing
104 T U M O R S OF THE HAND

Figure 5

Bleeding superficial hemangioma of the wrist.

persistent coloration (pale red). Veins can persist

a r o u n d t h e site of t h e h e m a n g i o m a .
In s u m m a r y , c o m p l i c a t i o n s of h e m a n g i o m a s
of t h e h a n d a r e r a r e but c a n l e a d t o modified
follow-up m a n a g e m e n t and non-routine surgery
being undertaken.
Figure 4

Ulcerated superficial hemangioma of the wrist.

Surgical management
Historic treatments for h e m a n g i o m a s are n u m e r -
m a n a g e m e n t is i m p l e m e n t e d quickly. S p o n t a n e o u s ous. F r o m early or repeated excision to any
healing c a n l e a v e s c a r o r fibrosis t i s s u e ( F i g . 4). method of s c l e r o s i s , e l e c t r o l y s i s , l i g a t i o n , etc.,
S p o n t a n e o u s bleeding or secondary bleeding m a n y procedures w e r e tried before the natural
f r o m p r i m a r y u l c e r a t i o n is a n o t h e r p o s s i b l e c o m - c o u r s e of h e m a n g i o m a w a s f u l l y u n d e r s t o o d . In
plication. This represents a major life-threatening f a c t , t h e ' t u m o r a l ' c h a r a c t e r i s t i c of h e m a n g i o m a
e v e n t f r o m t h e p a r e n t ' s p e r s p e c t i v e , but rarely often c o m p l i c a t e s its management because of
l e a d s to c o n s i d e r a b l e h e m o r r a g i a . L o c a l t h e r m o - strong parental involvement. Attentive attitude
c o a g u l a t i o n is r a r e l y p e r f o r m e d a n d s i m p l e c o m - a n d regular non-acting follow-up often surprise
pression stops the bleeding ( F i g . 5). p a r e n t s . T h e p r e d i c t a b l e c o u r s e of h e m a n g i o m a s
Skeletal distortion c a n result f r o m a v o l u m i - c o n f i r m s a n o n - s u r g i c a l a t t i t u d e m o s t of t h e t i m e .
n o u s h e m a n g i o m a producing a local a n d chronic Total r e g r e s s i o n is t h e r u l e . T h e r a r e i n d i c a t i o n s
m a s s effect o n a g r o w i n g bone, especially the for h e m a n g i o m a surgery are:
phalanges creating minor deviation (Mulliken and
Y o u n g 1988). (1) C o m p r e s s i o n : collateral n e r v e c o m p r e s s i o n
Fibrous residuum is a p o t e n t i a l sequela of or collateral v a s c u l a r pedicles o n finger local-
a n y h e m a n g i o m a , w i t h l o s s of skin elasticity a n d ization a r e v e r y r a r e b e c a u s e of t h e soft a n d
 VASCULAR T U M O R S : CLASSIFICATION AND S U R G E R Y 105

smooth characteristics of hemangiomas.

Difficulty in e x a m i n i n g v e r y y o u n g children
m a k e s this diagnosis v e r y rare.
(2) S e q u e l a e o r s c a r s : retraction o r l o s s of e l a s -
ticity of t h e skin o n t h e h a n d c a n b e a c a u s e
for surgical management but this should
not b e p e r f o r m e d b e f o r e a g e 8 o r 9. P l a s t i c
surgery techniques are useful (Z plasty,
grafts, local flaps, etc.).
(3) D o u b t in d i a g n o s i s : n o n - t y p i c a l h e m a n g i o m a
with malignant tumor or vascular malforma-
t i o n a s p e c t s h o u l d l e a d to s u r g e r y w i t h e a r l y
total e x c i s i o n a n d h i s t o l o g i c a l a n a l y s i s .

H e m a n g i o m a is a p a t h o l o g y of e a r l y i n f a n c y a n d
m o s t of t h e t i m e it is n o n - c o n g e n i t a l . T h e c l a s s i c
physical examination and a constant predictable
evolution make the diagnosis easier. Regular
follow-up should be undertaken to avoid unnec-
essary complementary explorations. Efficient
echography and M R I a r e a v a i l a b l e in c a s e of
d o u b t . H a n d localization r e m a i n s pretty c o m m o n
a n d rarely h a s c o n s e q u e n c e s o n normal d e v e l o p-
m e n t . S u r g e r y s h o u l d b e r e s e r v e d for c o m p l e x o r
c o m p l i c a t e d f o r m s b e c a u s e of t h e total natural Figure 6
r e g r e s s i o n of t h e t u m o r .
Capillary malformations of the dorsum on teenage female.

Vascular malformations
i m p r o p e r l y c a l l e d capillary hemangioma, leading
Vascular malformations represent the second to confusion w i t h v a s c u l a r t u m o r s . Histological
c a t e g o r y of v a s c u l a r a n o m a l i e s in t h e h a n d of characteristics are a non-proliferative mass of
the 1996 ISSVA classification. T h e y cannot be e c s t a t i c v e s s e l s in t h e u p p e r d e r m i s . Capillary
c o n s i d e r e d a s ' r e a l t u m o r s ' b e c a u s e of t h e i r n o n - malformations are present at birth a n d never
p r o l i f e r a t i v e c h a r a c t e r i s t i c . In fact, t h e e n d o t h e l i a l involute. T h e mal e and f e m a l e ratio is e q u a l .
c e l l t u r n o v e r o c c u r s a t a n o r m a l r a t e a s it d o e s in P h y s i c a l e x a m i n a t i o n s h o w s a flat m a c u l a of v i v i d
n o r m a l t i s s u e s d e f i n i n g dysplasia. The abnormal red color generally g r o w i n g with the develop-
development of these malformations can be m e n t of t h e h a n d ( F i g . 6). P o r t - w i n e s t a i n s t u r n to
explained by a slightly elevated mitosis rate. hypertrophic p u r p l e painful patches w h e n adult
A s a m a j o r differential d i a g n o s i s of v a s c u l a r (Fig. 7). I n d i c a t i o n s for treatment are mainly
t u m o r s , r e v i e w i n g the m a i n features of v a s c u l a r e s t h e t i c c o n s i d e r a t i o n s . L a s e r is t h e t r e a t m e n t of
malformations is n e c e s s a r y t o m a n a g e p r o p e r l y c h o i c e but results a r e poor.
t h e d i a g n o s i s of v a s c u l a r a n o m a l i e s . A s r e f e r r e d
to in t h e c l a s s i f i c a t i o n , v a s c u l a r malformations
exist in different f o r m s .
Venous malformations
Venous malformations represent the most fre-
Capillary malformations quent v a s c u l a r malformations. Incorrectly called
'cavernous hemangioma', venous malformations
Known as 'port-wine stains' or 'telangiectasic d o not h a v e t h e c h a r a c t e r i s t i c s of a t u m o r but a r e
stains', capillary malformations are often c o n f u s i n g . V e n o u s m a l f o r m a t i o n s a r e , in g e n e r a l ,
106 T U M O R S OF THE HAND

Figure 7

Giant capillary malformation of the upper limb on adult

male.

congenital, and follow hand development;

h o w e v e r , t h e y m a y d e v e l o p in a d u l t s .
P h y s i c a l e x a m i n a t i o n r e v e a l s a b l u e or p u r p l e Figure 8
m a s s , w i t h painful s w e l l i n g w h e n f a c i n g d o w n -
Spongy venous malformation of the palmar and dorsal
w a r d s . Giant v e n o u s malformation called spongy
zone.
venous malformation causes problems because
of its size a s a c o m p r e s s i o n f a c t o r ( M u l l i k e n a n d
Y o u n g 1988). C i r c u m s t a n c e s s u c h a s p r e g n a n c y ,
hormonal turnover, and menstruation can modify Venous malformations are sometimes associ-
t h e size, s y m p t o m s , a n d e v o l u t i o n of t h e m a l f o r - ated with s y n d r o m e s such a s the blue rubber
m a t i o n ( F i g . 8). bleb n e v u s s y n d r o m e : multiple v e n o u s malfor-
Histological analysis s h o w s a packed, d e n s e mations of t h e skin and gastrointestinal tract.
m a s s of v e n o u s e c s t a t i c v e s s e l s w i t h a n a r c h i c Phlebectasia, multiple intraosseous malforma-
o r g a n i z a t i o n , a n d n o r m a l m i t o s i s rate. T h e c a v - tions h a v e also been demonstrated.
e r n o u s f o r m is d e f i n e d b y v i r t u a l c a v i t i e s b e t w e e n S u r g e r y is n e c e s s a r y w h e n symptomatology
vessels. Phlebolitis a n d thrombosis are responsi- b e c o m e s u n b e a r a b l e . S e v e r e p a i n is t h e major
b l e for p a i n . s y m p t o m especially w h e n thromboses and phle-
Diagnosis can be performed by physical e x a m - b o l i t h s a r e n u m e r o u s ( F i g . 10).
ination but further e x p l o r a t i o n s a r e often u n a v o i d - T h e e n o r m o u s v o l u m e of v e n o u s malforma-
a b l e . E v a l u a t i o n of t h e e x t e n s i o n among hand tions creates a considerable handicap w h e n local-
s t r u c t u r e s is f u n d a m e n t a l . U l t r a s o n o g r a p h y w i t h ized to the hand, and may lead to surgery.
Doppler techniques is t h e most efficient tech- H o w e v e r , primary t r e a t m e n t for v e n o u s malfor-
n i q u e , s h o w i n g a s l o w - f l o w l e s i o n w i t h phlebolitis m a t i o n s is n o n - s u r g i c a l . I n t e r v e n t i o n a l radiology
a n d t h r o m b o s i s (Trop et al 1999). M R I p e r m i t s t h e with alcohol embolization provides a very effi-
limits of extension of t h e malformation to be c i e n t t h e r a p y , w i t h a c o n s i d e r a b l e r e d u c t i o n in t h e
established. T1 and T2 sequences with injected malformation volume. W h e n cutaneous troubles
g a d o l i u m p e r m i t e v a l u a t i o n of t h e s p e e d of b l o o d ( e r o s i o n ) o c c u r , s u r g i c a l e x c i s i o n is t h e s o l u t i o n .
f l o w ( F i g . 9). V e n o g r a p h y is o n l y u n d e r t a k e n w h e n D e e p e x t e n s i o n of m a l f o r m a t i o n s into h a n d s t r u c -
p r e o p e r a t i v e m a n a g e m e n t is n e c e s s a r y . tures such a s vascular pedicles, tendons, and
 VASCULAR T U M O R S : CLASSIFICATION AND S U R G E R Y 107

Figure 9 Figure 10

Magnetic resonance imaging (MRI) aspect of a venous Peroperative aspect of venous malformations with
malformation of finger. phlebolitis.

n e r v e s limit e x c i s i o n in m o s t c a s e s . F u r t h e r m o r e , millions of connections between intercalated

p e r o p e r a t i v e h e m o r r h a g e risk s h o u l d b e c o n s i d - normal tissues.
ered before surgery. Frequent recurrence after Physical examination s h o w s a red, beating,
s u r g e r y l e a d s t o p o o r results a n d m o s t often r a d i - whistling m a s s w i t h local t e m p e r a t u r e . P a i n is
o l o g i c t r e a t m e n t is p r e f e r r e d . c o m m o n . Compared with venous malformations
there is no modification with hand position.
H o r m o n a l f a c t o r s p l a y a s p e c i f i c role in t h e m e d -
ical t r e a t m e n t a n d g r o w t h ( F i g . 11).
Arteriovenous malformations
Complementary explorations
Arteriovenous malformations are the most
serious vascular malformations.They correspond The efficiency of ultrasonography with color
to a b n o r m a l v a s c u l a r c o m m u n i c a t i o n s between D o p p l e r a n d M R I h a s led to m a j o r i m p r o v e m e n t s
artery a n d v e i n . T h e y c a n be congenital or post- in t h e d i a g n o s i s of a r t e r i o v e n o u s malformations.
traumatic such as the hypothenar hammer U l t r a s o n o g r a p h y d e m o n s t r a t e s a b e a t i n g fast-flow
syndrome. Arteriovenous malformations are lesion w i t h fistulas. Color Doppler d e m o n s t r a t e s
r e s p o n s i b l e f o r h e m o d y n a m i c p r o b l e m s in c a s e s a s h u n t i n g a c t i v i t y w i t h arterial f l o w . It a n a l y z e s
of m u l t i p l e f o r m s . L o c a l i z e d f o r m s c a u s e f e w e r vessel density a s central, peripheral, or both.
problems. On the upper limb, arteriovenous H y p e r e c h o g e n i c i t y is f o u n d c o m p a r e d to normal
malformations d e v e l o p in a diffuse form with t i s s u e s (Paltiel et al 2000).
108 T U M O R S OF THE HAND

Figure 12

Arteriography of an arteriovenous malformation of the

hand.
Figure 11

Giant arteriovenous malformation of the dorsum hand.

tendinous invasion create major difficulties.
M o r e o v e r , in a r t e r i o v e n o u s m a l f o r m a t i o n s of t h e
finger base, blood supply c a n be c o m p r o m i s e d
for the rest of t h e f i n g e r ( U p t o n et al 1999).
Sometimes hematologic complications with
M R I , a n d e s p e c i a l l y M R I a n g i o g r a p h y , is t h e
m i c r o t h r o m b o s i s in a d j a c e n t v e s s e l s c a n l e a d to
m e t h o d of c h o i c e for d e t e c t i o n of arterial f l o w ,
e x t e n s i o n of t h e m a l f o r m a t i o n a m o n g soft t i s s u e s , l o s s of f i n g e r e x t r e m i t y . W h e n all t r e a t m e n t s f a i l ,
a n d v e s s e l c a l i b e r ( R i n k e r et al 2003). T h e u s e of amputation m a y be the only solution with impor-
a r t e r i o g r a p h y h a s r e d u c e d c o n s i d e r a b l y e x c e p t in t a n t m a r g i n s to a v o i d r e c u r r e n c e .
p r e o p e r a t i v e p l a n n i n g ( F i g . 12). L i g a t i o n of v e s s e l s c a n b e i n d i c a t e d w h e n t h e
m a l f o r m a t i o n is d i f f u s e , but r a d i o l o g i c e m b o l i z a -
t i o n is p r e f e r r e d . T h i s s o l u t i o n c a n b e r e q u i r e d in
Surgical management
r e c u r r e n t m a l f o r m a t i o n s after e x c i s i o n . S u r g i c a l
A r t e r i o v e n o u s m a l f o r m a t i o n s a r e t h e m o s t diffi- complications are c o m m o n and include bleeding,
cult t o c u r e . C o n s e r v a t i v e t r e a t m e n t is t h e p r o - peroperative hemorrhage, wound breakdown,
cedure of c h o i c e in t h e m a n a g e m e n t of this h e m a t o m a , infection, or necrosis. S e v e r e pain
p a t h o l o g y b e c a u s e of t h e i m p o r t a n t risk, s o m e - often o c c u r s after s u r g e r y .
times life-threatening, of peroperative hemor-
rhage. The poor results of surgery limit
indications.
H o w e v e r , v e r y aggressive f o r m s m a y require Lymphatic malformations
radical excision that only surgery can provide. and combined forms
Complete excision on a carcinologic m o d e w h e n
possible, for localized malformations, is the Lymphatic malformations and combined
m e t h o d of c h o i c e . A l t h o u g h i m p r e c i s e l i m i t s of forms are known as lymphangioma or angi-
the malformation, with neural, vascular, and o l y m p h a n g i o m a ; t h e s e m a l f o r m a t i o n s are often
 VASCULAR T U M O R S : CLASSIFICATION AND S U R G E R Y 109

( R i n k e r et al 2003). M a p p i n g of t h e infiltration is
v e r y u s e f u l in p r e o p e r a t i v e p l a n n i n g .
C o n s e r v a t i v e t r e a t m e n t is t h e r u l e u n l e s s size
or v o l u m e b e c o m e unacceptable. C o m p r e s s i o n
and elevation provide significant results with
regard to the non-agressive nature of these
techniques. Surgical debulking is sometimes
n e c e s s a r y in m a j o r f o r m s a n d is p e r f o r m e d for
the p u r p o s e of r e d u c t i o n in t h e size a n d v o l u m e .
E x c i s i o n of t h e m a x i m u m q u a n t i t y o f pathologic
tissue is recommended with preservation of
noble organs (nerves, tendons).
Better results are a c h i e v e d o n the dorsal side
of t h e h a n d t h a n p a l m a r f a c e o r digit. C o m b i n e d
lymphatic and v e n o u s forms display the s a m e
symptoms as lymphatic or venous malforma-
tions. These malformations associated with
gigantism and hypertrophic limb define the
Klippel-Trénaunay syndrome.

Malignant tumors
M a n y different t y p e s of m a l i g n a n t t u m o r exist but
their i n c i d e n c e is far l o w e r t h a n t h a t of benign
Figure 13 vascular tumors.

Giant lymphangioma of a baby's hand.

Angiosarcoma
defined by their voluminous and edematous Angiosarcoma can occur during any period of
a p p e a r a n c e . T h e y c a n i n v o l v e a n y part of the life, h o w e v e r , it is c o m p a r a t i v e l y r a r e a n d l o c a l -
h a n d (digit, d o r s u m ) , a n d s o m e t i m e s i n v a d e the ization to t h e h a n d is e x c e p t i o n a l (Glicenstein
e n t i r e h a n d , o r f o r e a r m . L y m p h a t i c c h a n n e l s of et al 1988).
all s i z e s f o r m i n g microscopic or macroscopic Its clinical a p p e a r a n c e is of a n e r y t h e m a t o u s
cysts c o m p o s e the m a l f o r m a t i o n s . T h e clinical infiltrated n o d e o r p a i n f u l a n d irregular patch.
appearance of voluminous limb with edema, C o n f u s i o n w i t h h e m a n g i o m a is p o s s i b l e , but t h e
n o r m a l s k i n c o l o r , a n d b u l k y c y s t a s p e c t is t y p i - rapid g r o w t h , a n d r a p i d a p p e a r a n c e of d i s s e m i -
c a l ( F i g . 13). A g e of a p p e a r a n c e v a r i e s but t h e nated pathologic m e t a s t a s e s at lymph nodes,
malformations are often congenital. T h e freaky m a k e s the difference. G e n e r a l dissemination hap-
a p p e a r a n c e of m a l f o r m a t i o n s is a m a j o r s o u r c e p e n s later.
of a n x i e t y f o r p a r e n t s w h e n t h e y o c c u r d u r i n g Histologically, it is a m a l i g n a n t proliferation of
infancy. e n d o t h e l i a l cells f o r m i n g dilated v e s s e l s w i t h a n a r -
In d o u b t f u l d i a g n o s e s , e c h o g r a p h y w i t h c o l o r chic organization ( G i r a r d et al 1970). T h e p r e s e n c e
Doppler will detect a low-flow lesion w i t h dilated of n u m e r o u s a t y p i c a l cells s u p p o r t s t h e d i a g n o s i s .
lymphatic c h a n n e l s a n d static f l u i d . Micro- or T r e a t m e n t is l i m i t e d ; a m p u t a t i o n w i t h c a r c i n o -
macrocystic aspects are possible. M R I detects a logic limits associated with radiotherapy and
heterogeneous signal with hyposignal on T1 chemotherapy gives poor results (Glicenstein
sequence and hypersignal on T2 sequence et al 1988).
110 T U M O R S OF THE HAND

Lymphangiosarcoma Conclusions
Lymphangiosarcoma occurs as a sequela of V a s c u l a r t u m o r s o f t h e h a n d i n c l u d e m a n y different
chronic lymphedema (Glicenstein et al 1988). f o r m s that are clearly defined by a precise a n d
C l i n i c a l a p p e a r a n c e s e e m s to b e K a p o s i - l i k e w i t h structured classification based upon physical
b l u e n o d e s a n d l y m p h e d e m a . T r e a t m e n t is v e r y examination, s y m p t o m s , histology, a n d e p i d e m i -
l i m i t e d a n d p r o g n o s i s v e r y poor. S u r v i v a l is g e n - o l o g i c f e a t u r e s . M a n a g e m e n t of t h e s e patholo-
erally under 6 months. gies demands a complete knowledge of this
classification to a v o i d incorrect d i a g n o s e s .
Vascular malformations and benign vascular
tumors are the most frequent. Incidence in
Hemangioendothelioma infancy is a major characteristic. Learning to
distinguish these two different categories is
Hemangioendothelioma can be classified a s a m o s t i m p o r t a n t to e n s u r e c o n s i s t e n c y . R e g u l a r
b o r d e r l i n e t u m o r . H i s t o l o g y is d e f i n e d b y prolifer- f o l l o w - u p is t h e s t r o n g e s t t o o l in t h e m a n a g e -
ative endothelial cells forming dilated vessels m e n t of t h e s e p a t h o l o g i e s . M o r e o v e r , m u l t i d i s c i -
with thrombosis ( A c h a r y a et al 1980). A t y p i c a l plinary consultations with radiologists, especially
cells a r e n u m e r o u s . A red or purple voluminous in p e d i a t r i c c a s e s w i l l p r o v i d e a m o r e efficient
n o d e infiltrating t h e normal t i s s u e s is present perspective in t h e handling of these tumors,
on clinical examination. Benign forms of this w h e n localization t o t h e h a n d c r e a t e s a p a r t i c u l a r
t u m o r h a v e b e e n r e p o r t e d ( S t o u t 1943). S u r g e r y p r o b l e m b e c a u s e of t h e e s t h e t i c s a n d functional
should be performed a s soon as possible with consequences involved.
amputation. S u r g i c a l m a n a g e m e n t c a n be t h e solution, but
r e g u l a r f o l l o w - u p of t h e t u m o r evolution very
often e n a b l e s radical a n d devastating excision,
t h e r e s u l t s of w h i c h a r e v a r i o u s , t o b e a v o i d e d .

Hemangiopericytoma
H e m a n g i o p e r i c y t o m a is a b o r d e r l i n e t u m o r with References
benign and malignant forms. Slow extension,
w i t h local infiltration of c a p i l l a r y p r o l i f e r a t i o n a n d Acharya S , Merrit W H , Teogarag S D (1980) Hemangio-
pericyte cells, m a k e s the diagnosis confusing endotheliomas of the hand: case report, J Hand Surg
5:181-2.
( G l i c e n s t e i n et al 1988). C o n f u s i o n w i t h v e n o u s
malformations c a n c r e a t e p r o b l e m s . Its clinical
Courbier R (1988) Tumeurs vasculaires bénignes de la
a p p e a r a n c e is of a r e d d i s h , e l a s t i c , h y p o d e r m i c
main. A propos de 92 cas. Thèse. Faculté de medecine
n o d e w i t h potential ulceration. Long-term recur-
de Marseille: Marseille: 2-3.
rences have been observed. Strategic m a n a g e -
m e n t is e a r l y e x c i s i o n w i t h c a r c i n o l o g i c m a r g i n s Enjolras O, Mulliken J B (1998) Vascular tumors and
and regular follow-up. vascular malformations (new issues), Adv Dermatol
13:375-422.

Girard C, J o h n s o n W C , Graham J H (1970) Cutaneous

angiosarcoma, Cancer 26:868-84.
Angioleiomyoma
Glicenstein J , Ohana J , Leclercq C (1988) Tumeurs de la
Angioleiomyoma is a b o r d e r l i n e tumor, which Main. Springer-Verlag: 163-6.
d e v e l o p s f r o m t h e v e n o u s v e s s e l w a l l s . It c a n
o c c u r o n t h e h a n d at a n y a g e o r in e i t h e r s e x Gonzales-Crussi F, Reyes-Mugica M (1991) Cellular
( G l i c e n s t e i n et al 1988). Its clinical a p p e a r a n c e is hemangiomas ('hemangioendotheliomas') in infants.
of a pink n o d e s e n s i t i v e t o p r e s s u r e a n d c o l d . Light microscopic, Immunohistochemical and ultra-
T r e a t m e n t is e x c i s i o n . structural observations, Am J Surg Pathol 15:769-78.
 VASCULAR T U M O R S : CLASSIFICATION AND S U R G E R Y 111

Hutchinson DT (1993) Color duplex imaging. Pasyk KA, Cherry G W , Grabb W C , Sasaki G H (1984)
Applications to upper extremity and microvascular Quantitative evaluation of mast cells in cellularly
surgery, Hand Clin 9:47-57. dynamic and adynamic vascular malformations, Plast
Reconstr Surg 73:69.
MacCollum DW, Martin LW (1956) Hemangiomas in
infancy and childhood: a report based on 6479 cases, Pasyk KA (1987) Classification and clinical and
Surg Clin North Am 36:1647. histopathological features of haemangioma and other
vascular malformations. In: Ryan T J , Cherry G W , eds.
Magalon G (1974) Etude analytique de 1000 angiomes Vascular Birthmarks. Oxford University Press: 1-54.
cutanéo-muqueux de l'enfant: deduction thérapeutique.
Thèse. Faculté de medecine de Marseille: Marseille: Rinker B, Karp N S , Margiotta M et al (2003) The role of
13-15. magnetic resonance imaging in the management of
vascular malformations of the trunk and extremities,
Margileth A M , Museles M (1965) Cutaneous heman- Plast Reconstr Surg 112:504-10.
giomas in children: diagnosis and conservative man-
agement, J Am Med Assoc 194:523. Stout A P (1943) Hemangioendothelioma: a tumor of
blood vessels, featuring vascular endothelial cells, Ann
Matthews DN (1968) Hemangiomata, Plast Reconstr Surg 118:445-64.
S u r g 41:528.
Takahashi K, Mulliken J B , Kozakewich H et al (1994)
Mulliken J B , Glowacki J (1982) Hemangiomas and vas- Cellular markers that distinguish the phases of heman-
cular malformations in infants and children: a classifica- gioma during infancy and childhood, J Clin Invest
tion based on endothelial characteristics, Plast Reconstr 93:2357-64.
Surg 69:412-20.
Trop I, Dubois J , Guibaud L et al (1999) Soft-tissue
Mulliken J B , Young A E (1988) Vascular Birthmarks: venous malformations in pediatric and young adult
Hemangiomas and Malformations. W B Saunders: patients: diagnosis with doppler U S , Radiology
Philadelphia: 24-37. 212:841-5.

Paltiel H J , Burrows P E , Kozakewich HP et al (2000) Soft- Upton J , Coombs C J , Mulliken J B et al (1999) Vascular
tissue vascular anomalies: utility of U S for diagnosis, malformations of the upper limb: a review of 270
Radiology 214:747-54. patients, J Hand Surg Am 24:1019-35.
 12
Radiologic treatment of
vascular 'tumors'
Philippe Petit, Jean M Bartoli, Karen Lambot, Guy Moulin
and Philippe Devred

Introduction General considerations

V a s c u l a r ' t u m o r s ' localized t o t h e e x t r e m i t i e s a r e I n d i c a t i o n s for t r e a t m e n t a r e d i s c u s s e d o n a c a s e -
r a r e . T h e i r m a n a g e m e n t is t h e r e f o r e difficult a n d b y - c a s e b a s i s . P a i n , p r o g r e s s i v e e n l a r g e m e n t of
t h e e x p e r t i s e of a t r a i n e d m u l t i d i s c i p l i n a r y t e a m the lesion, neurologic c o m p r o m i s e , diminished
including dermatologists, radiologists, plastic function, recurrent ulceration, hemorrhage,
a n d v a s c u l a r s u r g e o n s , a n d p a t h o l o g i s t s is n e c e s - i s c h e m i a , o r a c o m b i n a t i o n of t h e s e a r e t h e m o s t
s a r y for o p t i m a l d i a g n o s i s a n d t r e a t m e n t . Initial c o m m o n indications. A s y m p t o m a t i c patients do
imaging work-up of t h e s e l e s i o n s c o n s i s t s of not n e e d t o b e t r e a t e d .
ultrasound Doppler examination and magnetic G e n e r a l a n e s t h e s i a is r e q u i r e d f o r t h e t r e a t -
resonance imaging (MRI). m e n t of c h i l d r e n and/or w h e n a complex and
T h e m o r p h o l o g i c a p p e a r a n c e is e a s i l y o b t a i n e d p o t e n t i a l l y p a i n f u l p r o c e d u r e is p r o p o s e d ( a l c o -
o n u l t r a s o u n d ; pattern ( c y s t i c o r s o l i d , p r e s e n c e h o l injection is u s u a l l y v e r y p a i n f u l ) .
of p h l e b o l i t h s ) , limits ( s h a r p or ill-defined), c o n - Post-procedural pain a n d swelling are usually
s i s t e n c y ( h a r d o r soft), a n d v e s s e l s ( n u m b e r a n d w e l l c o n t r o l l e d w i t h s t e r o i d s a n d p a i n killers.
size) a r e t h e m o s t i m p o r t a n t f i n d i n g s . T h e f l o w
p a t t e r n of t h e l e s i o n is a n a l y z e d b y c o l o r a n d
pulsed
high-flow
Doppler, a n d allows the separation
lesions ( h e m a n g i o m a , arteriovenous
of
Radiologic treatment
malformation (AVM)) from low- or no-flow of specific lesions
lesions ( v e n o u s malformation, cystic l y m p h a n -
gioma, cyst). Malformations
The role of M R I is to define the size a n d
e x t e n s i o n o f t h e l e s i o n into a d j a c e n t s t r u c t u r e s .
Venous malformations
T 2 - s e q u e n c e s a r e sufficient t o e x p l o r e v e n o u s o r Venous malformations ( V M ) are the most c o m -
lymphatic malformations correctly. All other m o n v a s c u l a r m a l f o r m a t i o n s ( D u b o i s e t al 2001).
l e s i o n s s h o u l d b e e x p l o r e d after c o n t r a s t injec- R i s k s of V M i n c l u d e i s c h e m i a , p h l e b i t i s , rarely
tion; contrast material-enhanced M R angiography consumptive coagulopathy, and bleeding at
is u s e f u l to identify a c c u r a t e l y t h e arterial p e d i - s u r g e r y . M R I is t h e c o r n e r s t o n e of e v a l u a t i o n of
c l e s i n v o l v e d in A V M ( C o n n e l l et al 2002). such a disease and has recently b e e n proposed as
For s y m p t o m a t i c A V M a detailed arteriography a n efficient p r e d i c t o r of t r e a t m e n t o u t c o m e . G o o d
m a y b e a d d e d to d e f i n e t h e a n g i o a r c h i t e c t u r e of correlation b e t w e e n the a p p e a r a n c e o n M R I and
t h e l e s i o n . It c a n b e p e r f o r m e d p r i o r to t h e r a p e u - the result of percutaneous sclerotherapy has
tic d e c i s i o n - m a k i n g o r at t h e t i m e of e m b o l i z a t i o n . b e e n r e p o r t e d b y G o y a l et al (2002) (Table 1).
114 T U M O R S OF THE HAND

Table 1 Correlation between magnetic resonance imaging s o d i u m amidotrizoate, and propyleneglycol.

appearance and result of percutaneous sclerotherapy T h i s thick p r o d u c t i s difficult t o i n j e c t t h r o u g h
s m a l l - d i a m e t e r n e e d l e s . Its p r i n c i p a l risks a r e
Grade 1 : well-defined V M < 5 cm
of u l c e r a t i o n s a n d e x t r u s i o n t h r o u g h t h e skin
Grade 2A: well-defined V M > 5 cm
f o r d a y s a f f e c t i n g t h e initial o u t c o m e .
Grade 2B: ill-defined V M < 5 cm
Grade 3: ill-defined V M > 5 cm (4) P o l i d o c a n o l m i c r o f o a m is a s c l e r o s i n g a g e n t
t h a t h a s b e e n e m p l o y e d w i t h s u c c e s s in V M
( C a b r e r a et al 2003).

O n e or several punctures c a n be performed dur-

T h e infiltrating n a t u r e o f t h e s e b e n i g n l e s i o n s
ing t h e s a m e s e s s i o n . N e e d l e s a r e left in p l a c e for
a n d the potential i n v o l v e m e n t of e s s e n t i a l ner-
10 m i n b e f o r e r e m o v a l . A t o u r n i q u e t c a n b e u s e d
v o u s or v a s c u l a r s t r u c t u r e s m a k e s u r g e r y at h i g h
to s t o p v e n o u s d r a i n a g e a n d m a x i m i z e t h e effect
risk f o r c o m p l i c a t i o n s . Partial r e s e c t i o n m a y l e a d
of t h e p r o d u c t i n j e c t e d ( F i g . 1). C a r e s h o u l d b e
t o e n l a r g e m e n t of u n r e s e c t e d v e i n . L a s e r p h o t o -
t a k e n a s t h e r e is a risk o f m i g r a t i o n of t h e s c l e -
c o a g u l a t i o n , s t e r o i d a d m i n i s t r a t i o n , a n d arterial
r o s i n g a g e n t into t h e s y s t e m i c c i r c u l a t i o n after
embolization h a v e limited success. P e r c u t a n e o u s
r e m o v a l of t h e c o m p r e s s i n g d e v i c e s . T w o o r t h r e e
e m b o l i z a t i o n is n o w c o n s i d e r e d t o b e t h e t r e a t -
sessions m a y be required to obtain a significant
m e n t of choice.
result. A d e l a y o f 2 m o n t h s b e t w e e n e a c h s c l e -
Direct p h l e b o g r a p h y performed with 21-25-
rotherapy s e s s i o n is r e c o m m e n d e d .
g a u g e b u t t e r f l y c a t h e t e r s is t h e first s t e p b e f o r e
T h e rate o f m i n o r c o m p l i c a t i o n s v a r i e s f r o m
percutaneous sclerotherapy. It is needed to
4 t o 25 p e r c e n t . S e r i o u s c o m p l i c a t i o n s a r e e x c e p -
a s s e s s t h e v o l u m e of c o n t r a s t w h i c h fills t h e V M
t i o n a l . S y m p t o m a t i c relief ( d e c r e a s e in size o r d i s -
and to analyze, under fluoroscopic control, the
a p p e a r a n c e of t h e l e s i o n ) is o b t a i n e d in 6 4 - 1 0 0
v a s c u l a r p a t t e r n of t h e m a l f o r m a t i o n w h i c h c a n
per cent of patients; better results occur in
b e c a v i t a r y w i t h late v e n o u s d r a i n a g e into a nor-
patients with smaller and well-limited lesions
mal vein, spongy with small h o n e y c o m b cavities
( S h i r e m a n et al 1997, G e l b e r t et al 2 0 0 0 , C a b r e r a
a n d late v e n o u s d r a i n a g e , or d y s m o r p h i c with
et al 2003). R e c u r r e n c e is m o r e c o m m o n w i t h t h e
v e i n s r a p i d l y o p a c i f i e d ( D u b o i s et al 2001).
d y s m o r p h i c v e i n pattern.
S c l e r o s i s m a y b e o b t a i n e d u s i n g a n u m b e r of
C l i n i c a l f o l l o w - u p is u s u a l l y sufficient t o e v a l u -
different p r o d u c t s :
a t e r e s u l t s of s c l e r o s i s . W h e n r e q u i r e d M R c o n t r o l
m u s t not b e p e r f o r m e d before 6 months after
(1) S o d i u m t e t r a d e c y l s u l f a t e ( O ' D o n o v a n et al treatment d u e to t h e important chronic tissue
1997) h a s b e e n r e p o r t e d t o b e l e s s e f f e c t i v e inflammation produced by sclerosis.
than alcohol.
(2) Absolute alcohol affects protein denatura-
t i o n , w h i c h is r e s p o n s i b l e for clumping of
Lymphatic malformation
blood cells a n d vessel-wall necrosis w h i c h Lymphangioma most commonly o c c u r s in the
result in t h r o m b o s i s ( M a s o n et al 2000). T h e head and neck, a n d rarely develops in the
m a x i m u m r e c o m m e n d e d v o l u m e of a l c o h o l extremities. Similar imaging assessment and
is 1 m l / k g . P a t i e n t s w h o r e c e i v e u p to this t r e a t m e n t t o t h o s e for V M c a n b e a p p l i e d t o t h i s
dose m a y have elevated s e r u m ethanol levels l e s i o n . H o w e v e r , s p o n t a n e o u s r e g r e s s i o n of up
and b e e x p o s e d to the potential risks of to 15 p e r cent has been reported (Alqahtani
respiratory depression, cardiac arrhythmia, e t a l 1999). M a c r o c y s t i c f o r m s w i t h large a n e -
seizure, rhabdomyolisis, a n d hypoglycemia choic a r e a s a r e m o r e often treated by the per-
( M a s o n et a l 2000). L o c a l risks c o u l d a l s o b e cutaneous approach than are microcystic or
significant with skin and nerve ischemia, m i x e d f o r m s w h i c h a l l o w l e s s d i f f u s i o n of the
scarring, blistering, and temporary post- sclerotic agent.
procedural pain. A s p i r a t i o n a l o n e h a s not p r o v e d to b e efficient.
(3) E t h i b l o c is a 60 per c e n t a l c o h o l i c s o l u t i o n of
®
Multiple sclerosing a g e n t s h a v e b e e n u s e d to
c o r n protein zein m i x e d w i t h o l e u m p a p a v e r i s , treat t h i s m a l f o r m a t i o n :
 RAD 10LOGIC TREATMENT OF VASCULAR ' T U M O R S ' 115

(1) B l e o m y c i n e sulfate is an antibiotic with

antitumoral activity that provided significant
results but is n o l o n g e r u s e d o w i n g to its risk
of p u l m o n a r y f i b r o s i s ( Z h o n g et al 1998).
(2) OK-432, which is a l o w - v i r u l e n c e strain of
group A streptococcus incubated with peni-
cillin G , i n d u c e d a n i m m u n o l o g i c regulation
of c y t o k i n e s b y a n t i g e n i c s t i m u l u s i n d u c e d b y
s t r e p t o c o c c i . T h e r e is a risk of a n a p h y l a c t i c
s h o c k in c a s e s of a l l e r g y to p e n i c i l l i n . T h i s
product has gained a large enthusiasm in
t h e literature d u e to its a b s e n c e of toxicity
(Ishida a n d H o s h i n o 1985) a n d o v e r a l l effi-
c a c y of 66 p e r c e n t ( G i g u e r e et al 2002). It is
not c u r r e n t l y a v a i l a b l e in F r a n c e .
(3) Ethibloc has a similar success rate and
has the previously described potential side-
effects ( H e r b r e t e a u e t a l 1993).
(4) T r e a t m e n t w i t h a l c o h o l , 50 p e r c e n t d e x t r o s e ,
a n d T i s s u c o l ® (a fibrin s e a l a n t ) h a v e been
described sporadically.

Arteriovenous malformation
This vascular malformation has an unpredictable
outcome. Precipitating factors are not well
known, although puberty, pregnancy, trauma,
i l l - a d v i s e d s u r g e r y , or p r o x i m a l embolization or
ligature h a v e b e e n a d v o c a t e d .
D o p p l e r u l t r a s o u n d a l l o w s a correct d i a g n o s i s
of A V M to b e m a d e ( F i g . 2), but is insufficient to
a n a l y z e afferent a n d efferent v e s s e l s . M R a n g i o -
g r a p h y ( C o n n e l l et al 2002) c a n v i s u a l i z e t h e s e
v e s s e l s but is, f r o m o u r point of v i e w , not suffi-
ciently a c c u r a t e to p l a n t h e r a p y . A n g i o g r a p h y w i t h
superselective injections is t h e o n l y m e a n s to
a s s e s s t h e m a p p i n g of A V M correctly. H o w e v e r ,
this i n v e s t i g a t i o n is r e q u i r e d o n l y w h e n t r e a t m e n t
is c o n s i d e r e d . In s u c h c a s e s d i a g n o s t i c angio-
graphy m a y or may not be c o m b i n e d to the
e m b o l i z a t i o n . T h e latter c o u l d b e p e r f o r m e d a l o n e
o r in c o m b i n a t i o n w i t h partial or radical s u r g e r y
c
which must be performed w i t h i n a short time
Figure 1 ( 1 - 4 d a y s ) after e m b o l i z a t i o n in o r d e r to limit b l o o d
loss, a n d t h u s p r e v e n t t h e A V M recurring t h r o u g h
Venous malformation of the right hand of a 15-year-old boy.
collateral f e e d i n g arteries ( M e r l a n d et al 1992).
(a) Coronal T2-weighted image shows a well-limited high-
Asymptomatic patients do not require any
signal intensity lesion corresponding to low-flow venous
vessels developed in the thenar muscles. (b) Direct phlebo- treatment. Pain, functional impairment, and,
gram performed with a 25-gauge butterfly needle demon- rarely, c a r d i a c i n s u f f i c i e n c y a r e r e c o g n i z e d i n d i c a -
strates a well-limited lesion with only one small normal t i o n s for t r e a t m e n t . T h e g o a l of w h i c h is e i t h e r to
draining vein. (c) Embolotherapy was performed with alco- reduce the flow of the A V M to stabilize the
hol and tourniquet inflated on the draining vein. i n c r e a s e in v o l u m e of t h e l e s i o n , o r prior t o r a d i c a l
116 T U M O R S OF THE HAND

Figure 2

Arteriovenous malformation (AVM) of the third finger of the

right hand of a 24-year-old woman. (a) Transverse color
Doppler ultrasound of the second phalange of the third
digit, shows enlarged tubular structures corresponding to
feeding arteries and draining veins of the A V M . Pulsed
Doppler reveals increased turbulent flow with low resis-
tance index and spectral broadening corresponding to the
nidus of the A V M . (b) Pulsed Doppler demonstrated an
increased flow with high diastolic component in the medial
proper palmar digital artery of the third finger; compare
(b) with the normal slow flow and high resistance index of
the medial proper palmar digital artery of the second digit (c).
c

surgery to cure the lesion. Eradication m u s t be a v o i d a c c i d e n t a l diffusion to u n d e s i r a b l e r e g i o n s ,

t h e final g o a l of s u r g e r y but it is often difficult t o h a s b e e n p r o p o s e d ( S l a b a et al 1998).
avoid functional impairment and reconstruction Direct puncture (18-20-gauge needle) or
problems. c a t h e t e r i z a t i o n of t h e v e n o u s p o r t i o n of t h e A V M
w h e n t h e d r a i n i n g v e i n is c l o s e t o t h e n i d u s h a s
Methods of opacification ofAVM b e e n d e s c r i b e d o n l y rarely. In c a s e s of m u l t i p l e
feeding arteries, or impossible access to the
A n g i o g r a p h y is c u r r e n t l y p e r f o r m e d v i a a f e m o r a l
n i d u s , or w h e n it is t o o c l o s e t o i m p o r t a n t normal
approach; selective angiography with a 5-Fr
arterial b r a n c h e s t h i s t e c h n i q u e m u s t b e c o n s i d -
c a t h e t e r i n t r o d u c e d into t h e h u m e r a l a r t e r y a n d
e r e d ( J a c k s o n e t al 1996, G o r r i z G o m e z E 1998).
c o l l a t e r a l a r t e r i e s g i v e s a n o v e r v i e w of t h e u p p e r
limb vascularization. Microcatheters (3-Fr) are
t h e n a d v a n c e d c o a x i a l l y t h r o u g h t h e 5-Fr c a t h e t e r
Embolotherapy
for m o r e h y p e r s e l e c t i v e c a t h e t e r i z a t i o n , a s c l o s e
a s p o s s i b l e t o t h e n i d u s , a n d distal injections T h e simple rules that no ligation or proximal
( F i g . 3). O t h e r t e c h n i q u e s o f e m b o l i z a t i o n have embolization must be performed since they are
been reported to be successful. i n e f f e c t i v e m u s t b e f o l l o w e d . I n d e e d , after t r a n -
Direct p u n c t u r e of t h e m a i n f e e d e r arterial v e s s e l sient shrinkage the multiple small feeder v e s s e l s
a s s o c i a t e d w i t h a n a n n u l a r c o m p r e s s i o n d e v i c e , to are recruited, enlarged, a n d replace the initial
 RADIOLOGIC TREATMENT OF VASCULAR T U M O R S ' 117

malformation; the nidus is t h e n much more

c o m p l e x to a c c e s s . Distal s u p r a s e l e c t i v e e m b o l i z a -
tion is t h e t e c h n i q u e of c h o i c e . M u l t i p l e products
h a v e b e e n u s e d for t h i s p u r p o s e :

(1) L a r g e r - c a l i b e r c o i l s a r e not a p p l i c a b l e d u e to
t h e size of t h e v e s s e l to b e o c c l u d e d .
(2) P a r t i c l e s a r e u s u a l l y u s e d w h e n s u r g e r y is
p l a n n e d r a p i d l y after e m b o l i z a t i o n . T h e risk
of recanalization after particulate material
(150-600 µm) is w e l l k n o w n ( M e r l a n d e t a l
1992).
(3) Liquid polymerizing agent A/-butyl-2-
c y a n o a c r y l a t e (Histoacryl®) is w i d e l y r e c o m -
m e n d e d ( M e r l a n d et al 1992, S l a b a et al 1998).
R e c a n a l i z a t i o n h a s b e e n d e s c r i b e d to b e faster
w i t h particle t h a n p o l y m e r i z i n g a g e n t s (3 to
6 m o n t h s a n d 1 to 9 y e a r s , r e s p e c t i v e l y ) ( S l a b a
et al 1998). Histoacryl i n d u c e d rapid t h r o m b o -
sis o n c o n t a c t w i t h b l o o d . L i p i o d o l ® (a m i x t u r e
of oil a n d c o n t r a s t m e d i u m ) is currently a d d e d
to this product at a dilution w h i c h is d e p e n -
d e n t o n t h e s p e e d of A V M . T h e faster is t h e
AVM the lower the volume of Lipiodol is
i n j e c t e d . T h i s g l u e is not v e r y e a s y to m a n i p u -
late a n d t h e risk of g l u i n g t h e c a t h e t e r w i t h i n
t h e artery s h o u l d b e a p p r e c i a t e d . A n a m o u n t
of 0.1-1.5 m l of H i s t o a c r y l is u s u a l l y sufficient
to fill t h e n i d u s a n d a v o i d large opacification
of t h e d r a i n i n g v e i n s .
(4) Liquid sclerosing a g e n t s including S o t r a d e c o l ®
a n d p u r e a l c o h o l ( m a x i m u m d o s e 1.0 ml/kg
b o d y w e i g h t ) (Yakes et al 1996) directly injected
by p e r c u t a n e o u s puncture or through micro-
catheters h a v e also b e e n p r o p o s e d .

Results

O w i n g to t h e rarity of t h i s l e s i o n at this site o n l y

a f e w s e r i e s of A V M t r e a t e d b y embolotherapy
b a l o n e o r in c o m b i n a t i o n w i t h s u r g e r y h a v e b e e n
p u b l i s h e d ( G o m e s 1994, Y a k e s e t al 1996, E n j o l r a s
Figure 3 et al 2000, W h i t e et al 2000, S o f o c l e o u s et al
2001, R o c k m a n et al 2003). S o f o c l e o u s et al
Arteriovenous malformation (AVM) of the right hand of a
(2001) report o n a 15-year r e t r o s p e c t i v e study
28-year-old woman. (a) Coronal T1-weighted image shows
of 21 c a s e s of A V M ; d e s p i t e immediate good
multiple large signal void vessels (high-flow vessels)
developed throughout the wrist and hand. (b) Pre-emboliza- results t h e m e a n s y m p t o m - f r e e p e r i o d w a s o n l y
tion elective angiography demonstrated the extension 30 months. However, others authors (Gomes
of the AVM which has already necessitated multiple 1994, Y a k e s et al 1996, E n j o l r a s et al 2000, W h i t e
amputations. et al 2000, R o c k m a n et al 2003) r e p o r t e d much
118 T U M O R S OF THE HAND

better r e s u l t s in a n e q u i v a l e n t n u m b e r of p a t i e n t s w h e n s y s t e m i c or i n t r a l e s i o n a l s t e r o i d t r e a t m e n t ,
explored during the s a m e period of t i m e . F o r a s w e l l a s i n t e r f e r o n α2a o r α2b, h a v e not b e e n
e x a m p l e , W h i t e et al (2000) r e p o r t e d long-term sufficient. W h e n l o c a t e d to t h e e x t r e m i t i e s t h e s e
relief of s y m p t o m s ( m e a n f o l l o w - u p 7.5 y e a r s ) in t r e a t m e n t s a r e u s u a l l y not r e q u i r e d .
11 p a t i e n t s e i t h e r w i t h e m b o l i z a t i o n a l o n e o r in
c o m b i n a t i o n w i t h s u r g i c a l e x c i s i o n of t h e A V M . Glomus tumor
N o extremity amputations w e r e required (White
This benign tumor r e p r e s e n t s 1-5 per cent of
et al 2000).
h a n d t u m o r s . T h e t r e a t m e n t is s u r g i c a l a n d t h e r e
T h e c o m p l i c a t i o n r a t e r e p o r t e d in t h e literature
is n o n e e d for i n t e r v e n t i o n a l r a d i o l o g y ( P o n n e l l e
v a r i e d f r o m 10 to 30 p e r c e n t w i t h a l a r g e m a j o r -
et al 1999).
ity of m i n o r f o r m s ( G o m e s 1 9 9 4 , Y a k e s et al 1996).
Complications included:

Malignant tumors
(1) Arterial spasm which may occur during
angiography or after embolization and
S o f t t i s s u e m a l i g n a n t t u m o r s localized to t h e h a n d
r e q u i r e s g r e a t c a r e if it is t o b e a v o i d e d , but
are unusual. T h e following tumors are discovered
is r e v e r s i b l e w i t h i n j e c t i o n of v a s o d i l a t o r ;
occasionally: angiosarcoma, hemangiopericytoma,
(2) Deep venous thrombosis; hemangioendothelioma, lymphangiosarcoma,
(3) Transient n e r v e c o m p r e s s i o n s e c o n d a r y to angioleiomyoma, synovialosarcoma, and primi-
the swelling - s e v e r e n e r v e injury s e e m s to t i v e n e u r o e c t o d e r m a l t u m o r . T h e role of i n t e r v e n -
b e m o r e f r e q u e n t after a b s o l u t e a l c o h o l injec- tional r a d i o l o g y m a y b e l i m i t e d t o embolization
t i o n ( G o m e s 1994); prior to b i o p s y or s u r g e r y .
(4) Tissue and skin necrosis may need skin
grafting;
(5) Pulmonary embolism;
(6) In a s e r i e s of 50 p a t i e n t s t r e a t e d for e x t r e m i t y
v a s c u l a r m a l f o r m a t i o n o n e c a s e (2 p e r c e n t )
Conclusion
of limb loss w a s reported ( R o c k m a n et al
V a s c u l a r m a l f o r m a t i o n s of t h e h a n d a r e difficult
2003);
to eradicate completely with embolization,
(7) Cardiopulmonary collapse secondary to
surgery, or both. Recurrences are frequent.
o v e r d o s e ethanol injection.
Management with a multidisciplinary team is
mandatory and long-term follow-up is n e e d e d .
Follow-up T h e s e malformations are far m o r e c o m m o n than
Clinical and Doppler examinations must be malignant t u m o r s . H o w e v e r , careful clinical a n d
p e r f o r m e d r e g u l a r l y f o r a n u m b e r of y e a r s . T h e r e imaging evaluation, a n d expert decision-making
is n o d e f i n i t e s i g n of i l l n e s s a n d late r e c u r r e n c e are crucial before proposing embolotherapy or
is a l w a y s p o s s i b l e . surgical treatment.

Vascular tumors References

Alqahtani A , Nguyen LT, Flageole H et al (1999) 25 years'
Benign tumors experience with lymphangiomas in children, J Pediatr
Surg 34:1164-8.
Hemangioma
Cabrera J , Cabrera J J r , Garcia-Olmedo M A , Redondo P
T h e v a s t m a j o r i t y of t h i s pediatric t u m o r d o not
(2003) Treatment of venous malformations with scle-
require a n y treatment a n d involute with a g e . Less rosant in microfoam form, Arch Dermatol 139:1409-16.
than 15 p e r c e n t of h e m a n g i o m a s n e e d to be
treated either because of high-output cardiac Connell DA, Koulouris G , Thorn DA, Potter HG (2002)
failure o r insults to v i s i o n o r b r e a t h i n g . R a d i o l o g i c Contrast-enhanced M R angiography of the hand,
i n t e r v e n t i o n (i.e. e m b o l i z a t i o n ) c o u l d b e n e c e s s a r y Radiographics 22:583-99.
 RADIOLOGIC TREATMENT OF VASCULAR T U M O R S ' 119

Dubois J , Soulez G , Oliva VL et al (2001) Soft-tissue Merland J J , Marache P, Herbreteau D et al (1992)

venous malformations in adult patients: imaging and Superficial and peripheral vascular malformations. Role
therapeutic issues, Radiographics 21:1519-31. of interventional radiology and of embolization, J Mal
Vasc 17:44-9.
Enjolras O, Logeait I, Gelbert F et al (2000)
Arteriovenous malformations: a study of 200 cases, Ann O'Donovan J C , Donaldson J S , Morello FP et al (1997)
Dermatol Venerol 127:17-22. Symptomatic hemangiomas and venous malformations
in infants, children, and young adults: treatment with
Gelbert F, Enjolras O, Deffrenne D et al (2000) percutaneous injection of sodium tetradecyl sulfate, Am
Percutaneous sclerotherapy for venous malformation of J Roentgenol 169:723-9.
the lips: a retrospective study of 23 patients,
Neuroradiology 42:692-6. PonnelleT, Gouny P, Boudghene F et al (1999) Glomus
tumor of the extremities, J Mal Vasc 24:364-7.
Giguere C M , Bauman N M , Smith R J (2002) New treat-
ment options for lymphangioma in infants and children, Rockman C B , Rosen R J , Jacobowitz GR et al (2003)
Ann Otol Rhinol Laryngol 111:1066-75. Transcatheter embolization of extremity vascular
malformations: the long-term success of multiple inter-
Gomes A S (1994) Embolization therapy of congenital ventions, Ann Vasc Surg 17:417-23.
arteriovenous malformations: use of alternate approa-
ches, Radiology 190:191-8. Shireman PK, McCarthy W J , Y a o JS,Vogelzang RL (1997)
Treatment of venous malformations by direct injection
Gorriz Gomez E, Carreira Villamor J M , Reyes Perez R with ethanol, J Vasc Surg 26:838-44.
et al (1998) Percutaneous treatment of peripheral vascu-
lar malformations, Rev Clin Esp 198:565-70. Slaba S , Herbreteau D, J h a v e r i H S et al (1998)
Therapeutic approach to arteriovenous malformations
Goyal M , Causer PA, Armstrong D (2002) Venous vascu- of the tongue, Eur Radiol 8:280-5.
lar malformations in pediatric patients: comparison of
results of alcohol sclerotherapy with proposed MR Sofocleous CT, Rosen R J , Raskin K et al (2001)
imaging classification, Radiology 223:639-44. Congenital vascular malformations in the hand and
forearm, J EndovascTher8:484-94.
Herbreteau D, Riche M C , Enjolras O et al (1993)
Percutaneous embolization with Ethibloc of lymphatic White RI J r , Pollak J , Persing J et al (2000) Long-term
cystic malformations with a review of the experience in outcome of embolotherapy and surgery for high-flow
70 patients, IntAngiol 12:34-9. extremity arteriovenous malformations, J Vasc Interv
Radiol 11:1285-95.
Ishida N, HoshinoT (1985) A streptococcal preparation
as a potent biological modifier OK - 432. Amsterdam, Yakes W F , Rossi P, Odink H (1996) H o w I do it.
The Netherlands, Excerpta Medica 1-70. Arteriovenous malformation management, Cardiovasc
intervent Radiol 19:65-71.
Jackson J E , Mansfield AO, Allison D J (1996) Treatment
of high-flow vascular malformations by v e n o u s Zhong PQ, Zhi F X , Li R et al (1998) Long-term results of
embolization aided by flow occlusion techniques, intratumorous bleomycin-A5 injection for head and
Cardiovasc Intervent Radiol 19:323-8. neck lymphangioma, Oral Surg Oral Med Oral Pathol
Oral Radiol Endod 86:139-44.
Mason KP, Michna E, Zurakowski D et al (2000) Serum
ethanol levels in children and adults after ethanol
embolization or sclerotherapy for vascular anomalies,
Radiology 217:127-32.
 13
What can the hand surgeon
do when facing a patient
with a melanonychia?
Christian Dumontier and Philippe Abimelec

Introduction surgery, untreated subungual

p o t e n t i a l l y lethal d i s e a s e .
melanoma is a

Longitudinal melanonychia ( L M ) presents a s a T h e s u r g e o n f a c e d w i t h a patient w i t h a longitu-

t a n , b r o w n i s h , o r black l o n g i t u d i n a l s t r i p e of t h e d i n a l dark b a n d m u s t a l w a y s think first of s u b -
nail p l a t e , w h i c h results f r o m i n c r e a s e d m e l a n i n ungual m e l a n o m a and perform an adequate biopsy
d e p o s i t i o n . M e l a n o c y t e s a r e p r e s e n t in t h e nail to e n s u r e t h e correct d i a g n o s i s . A nail b i o p s y is a n
m a t r i x a n d nail b e d , w h e r e t h e y r e m a i n f u n c t i o n - e a s y p r o c e d u r e that d o e s not l e a v e permanent
ally quite i n a c t i v e . L M m a y b e t h e result of d a m a g e t o t h e nail if it is p e r f o r m e d a d e q u a t e l y .
increased melanin synthesis by normally non- Optimal care should be given by a team, which
functional m e l a n o c y t e s that m a y b e n o r m a l o r s h o u l d include a d e r m a t o l o g i s t and a dermato-
t u m o r a l ( b e n i g n o r m a l i g n a n t ) . S i m u l a t o r s of L M pathologist w h o a r e e x p e r i e n c e d in this field.
( p s e u d o L M ) result f r o m a nail b e d d y s c h r o m i a
a n d m a y m i m i c t h i s c o n d i t i o n ( i . e . splinter h e m -
orrhages, onychomycosis, foreign bodies) (Figs. 1
a n d 2). M o s t L M a r e n o t t u m o r a l ( s e e T a b l e 1), Epidemiologic studies
t h e y a r e t h e result of p o s t i n f l a m m a t o r y o r p o s t -
t r a u m a t i c nail p l a t e h y p e r m e l a n i n o s i s t h a t m a y L M s a r e c o m m o n in d a r k l y p i g m e n t e d p e r s o n s ,
b e s e c o n d a r y t o m i c r o t r a u m a ( n a i l b i t i n g , picking, a n d o c c u r in t h r e e - q u a r t e r s of A f r i c a n - A m e r i c a n s
nail t r a u m a s in ill-fitting s h o e s ) , i n f e c t i o n s ( o n y - o v e r 20 y e a r s of a g e a n d in a l m o s t 100 p e r c e n t
c h o m y c o s i s or bacterial infection), drug ingestion o v e r 50 y e a r s . T h e t h u m b a n d i n d e x f i n g e r a r e
(antibiotics, cytotoxic drugs, etc.), systemic m o s t f r e q u e n t l y i n v o l v e d . L M o c c u r s in 10-20 p e r
d i s e a s e ( A d d i s o n ' s d i s e a s e , h e m o s i d e r o s i s , etc.), cent of J a p a n e s e and the thumb, index a n d
or d e r m a t o l o g i c disease (lichen planus, idiopathic m i d d l e f i n g e r s a r e m o s t f r e q u e n t l y i n v o l v e d . It is
lentiginosis, B o w e n ' s disease) ( F i g . 3). Tumoral a l s o r a t h e r f r e q u e n t in H i s p a n i c a n d o t h e r dark-
c a u s e s of L M m a y b e b e n i g n , s u c h a s lentigo s i m - skinned people but unusual a m o n g w h i t e people.
plex, m e l a n o c y t i c n e v u s , or malignant, such a s Approximately 3 per cent of malignant
m e l a n o m a . A l l c a u s e s of L M a r e m o r e f r e q u e n t in melanomas in w h i t e persons are subungual
dark-skinned p e r s o n s . M o s t L M a r e benign but, m e l a n o m a . In J a p a n e s e , s u b u n g u a l m e l a n o m a s
w h e n it is u n i q u e , t h e m o s t e x p e r i e n c e d c l i n i c i a n r e p r e s e n t 3-10 p e r c e n t of all m e l a n o m a s , w h i l e
c a n n o t differentiate a b e n i g n b a n d f r o m a n e a r l y accounting for 15-20 per cent in African-
m e l a n o m a . W h i l e e a r l y - e v o l v i n g m e l a n o m a is a Americans. A s the incidence of melanoma
d i s e a s e that is definitively c u r e d b y non-mutilating d e c r e a s e s in d a r k - s k i n n e d r a c e s , t h e total n u m b e r
122 T U M O R S OF THE HAND

Figure 1 Figure 2

Simulator of longitudinal melanonychia. Patient presented Simulator of longitudinal melanonychia. Patient presented
here had a foreign body under his nail plate. here had mycotic infection.

of s u b u n g u a l m e l a n o m a is n o t g r e a t e r in t h e s e
c o m m u n i t i e s . T h e r e are s o m e reports of s u b u n -
g u a l m e l a n o m a i n c i d e n c e r a t e s of 17 p e r c e n t in
C h i n e s e a n d 33 p e r c e n t in A m e r i c a n I n d i a n s .

Diagnosis
L M h a s n u m e r o u s e t i o l o g i e s t h a t m a y often be
r e c o g n i z e d b y a c a r e f u l m e d i c a l history, clinical
examination, a n d selected diagnostic tests. T h e
best clinical tool for t h e h a n d s u r g e o n is t h e help
of a n e x p e r i e n c e d d e r m a t o l o g i s t . First, to help
eliminate non-melanotic causes w h e n facing a
L M ( s e e T a b l e 1).
Because L M h a s b e e n recognized to be an
early sign of melanoma a biopsy should be
p e r f o r m e d w h e n its c a u s e is n o t a p p a r e n t ( B a r a n
and K e c h i j i a n 1989). F o r m e l a n o t i c d i s e a s e , f e w
investigations, other than biopsies are necessary,
h o w e v e r , c e n t e r e d , plain X - r a y s a r e n e e d e d t o
eliminate any bony involvement.
W h e n t h e L M is u n i q u e , e v e n t h e m o s t e x p e r i -
e n c e d d e r m a t o l o g i s t h a s f e w w a y s to distinguish
clearly the subgroup of s u b u n g u a l melanomas
f r o m t h e larger g r o u p of ' n o n - s p e c i f i c ' L M ( F i g . 4 ) .
T h e d i s t r i b u t i o n of L M a n d s u b u n g u a l m e l a n o m a
b is v e r y similar. In t h e h a n d , b o t h o c c u r in t h e
t h u m b o r i n d e x finger. In a l l , 4 5 - 6 0 p e r c e n t of s u b -
Figure 3
ungual m e l a n o m a s develop on the h a n d , while
Simulator of longitudinal melanonychia. Patient presented 4 0 - 5 5 p e r c e n t o c c u r o n t h e g r e a t t o e . T h e r e is
here had an onychopapilloma (a). Inspection of the nail plate e v i d e n c e s u g g e s t i n g t h a t L M is a p r e c u r s o r l e s i o n
(b) revealed the tumor underneath. of a c t u a l m e l a n o m a . S u b u n g u a l m e l a n o m a s a r e
 WHAT CAN THE HAND S U R G E O N DO W H E N FACING A PATIENT WITH A MELANONYCHIA? 123

Table 1 Causes of longitudinal melanonychia (LM) usually asymptomatic, pain and bleeding are
v e r y late s i g n s for w h i c h nobody should wait.
Polydactylous LM
The surgeon should suspect a melanoma and
Drugs and chemicals
offer a b i o p s y for a n y u n e x p l a i n e d m o n o d a c t y l o u s
Antibiotics: cyclines, sulfonamide
L M o r a n y p r e v i o u s l y k n o w n L M that h a s c h a n g e d
Antimalarials
Antineoplastic drugs significantly.
Beta-blocking agents: timolol
Heavy metals: arsenic, gold, mercury
Ketoconazole
Phenothiazine
Nail unit melanoma
Psoralen
Zydovuridine
N a i l unit m e l a n o m a ( N U M ) is a m a l i g n a n t n e o -
Endocrine
plasm derived from melanocytes. N U M s repre-
ACTH therapy
ACTH, MSH-producing tumors s e n t 2 - 3 p e r c e n t of all m e l a n o m a s in C a u c a s i a n s
Addison's disease a n d a r o u n d 20 p e r c e n t in i n d i v i d u a l s of dark-skin
Hyperthyroidism r a c e s . N U M is m o r e f r e q u e n t l y d i a g n o s e d in t h e
Pregnancy sixth d e c a d e but is not r a r e in y o u n g e r i n d i v i d u -
Ethnic als; w o m e n are m o r e frequently affected than
Dark-skin individuals
m e n ( B l e s s i n g et al 1991, P a r k et al 1992, F i n l e y
Genetic
et al 1994, K a t o et al 1996). T h u m b a n d g r e a t t o e
Peutz-Jeghers syndrome
a r e m o r e often i n v o l v e d . R e c e n t l y , m u c h attention
Infectious
Onychomycosis h a s b e e n d r a w n to t h e e a r l y d e t e c t i o n of N U M .
Inflammatory Clinically, m e l a n o m a in situ of t h e nail a p p a r a t u s
Lichen planus p r e s e n t s a s a l a r g e L M (> 6 m m ) o r a nail t h a t is
Lichen striatus t o t a l l y black. T h e d i a g n o s i s is r a r e l y m a d e w h e n
Metabolic and nutritional t h e b a n d is t h i n but N U M often b e g i n s a s a v e r y
Hemochromatosis thin band that should raise suspicion.
Hemosiderosis
P r o g r e s s i v e w i d e n i n g of t h e b a n d is a n a l m o s t
Kwashiorkor
c o n s t a n t f e a t u r e . A p r o x i m a l w i d t h of t h e b a n d
Vitamin B12 deficiency
Miscellaneous that is s u p e r i o r to t h e distal w i d t h indicates a
Laugier-Hunziker syndrome rapid g r o w t h rate. L i g h t b r o w n bands may be
Carpal tunnel syndrome s e e n , but t h e c o l o r of t h e b a n d is m o r e often dark,
Radiation with variegated colors and multiple fine linear
Traumas s t r e a k s of d e n s e r h y p e r p i g m e n t a t i o n (Blessing
Acute et al 1991). B r o w n / b l a c k m a c u l a r p i g m e n t a t i o n of
Chronic: self-inflicted
t h e nail m a y i n d i c a t e m e l a n o m a of t h e nail b e d .
Onychomania
L a t e s i g n s m a y i n c l u d e : d e s t r u c t i o n of t h e nail
Monodactylous LM plate or f i s s u r e ; t u m o r ; g r a n u l a t i o n t i s s u e , w h i c h
Neoplastic m a y or m a y not b e , p i g m e n t e d ; u l c e r a t i o n of t h e
Melanocytic nail b e d a s s o c i a t e d w i t h o n y c h o l y s i s ; d e s t r u c t i o n
Melanoma of the nail plate; infection; b l e e d i n g ; or pain
Melanocytic hyperplasia
( B l e s s i n g et al 1991).
Nevus
P i g m e n t a t i o n of t h e p e r i u n g u a l t i s s u e , s o c a l l e d
Non-melanocytic
Spinous cell carcinoma H u t c h i n s o n ' s s i g n , is a later, but v e r y s u g g e s t i v e ,
Myxoid cyst s i g n that is i n c o n s i s t e n t a n d not pathognomonic
Wart ( B a r a n a n d K e c h i j i a n 1996). H u t c h i n s o n ' s s i g n w a s
d e s c r i b e d a s a p e r i u n g u a l s p r e a d of p i g m e n t a t i o n
PseudoLM to the proximal a n d lateral nail f o l d s ( F i g . 5).
Hemorrhage
P s e u d o - H u t c h i n s o n ' s s i g n is d u e to t h e relative
Foreign body
t r a n s p a r e n c y of t h e cuticle a n d p r o x i m a l nail f o l d
Onychomycosis
(Table 2). T h e b i o p s y of t h e p e r i u n g u a l p i g m e n t a -
ACTH, adrenocorticotropic hormone; M S H , melanocyte t i o n m a y offer a n e a s y t e c h n i q u e of s a m p l i n g t h e
stimulating hormone lesion to c o n f i r m t h e m a l i g n a n c y , but a n e g a t i v e
124 T U M O R S OF THE HAND

a b

Figure 4

Longitudinal melanonychia. It is almost impossible, even for an experienced pathologist to differentiate a subungual
melanoma (a) from a lentigo (b).

result will nonetheless often necessitate an a s this w i l l not c h a n g e t h e t r e a t m e n t o r p r o g n o s i s .

excisional biopsy. However, an experienced pathologist is very
A m e l a n o t i c m e l a n o m a s a c c o u n t for 1 5 - 2 5 p e r i m p o r t a n t , e s p e c i a l l y in t h e l e s s s e v e r e f o r m , to
c e n t of N U M . W e lack i n f o r m a t i o n c o n c e r n i n g t h e differentiate a b e n i g n h y p e r p l a s t i c l e s i o n f r o m a n
e a r l y clinical p r e s e n t a t i o n of a m e l a n o t i c nail unit in situ m e l a n o m a .
m e l a n o m a s ( A N U M ) t h a t a r e m o r e often d i s c o v - The prognosis of subungual melanomas is
e r e d at a v e r y late s t a g e . P e r i u n g u a l A N U M m a y e s s e n t i a l l y r e l a t e d to their B r e s l o w t u m o r thick-
present as a pyogenic granuloma-like tumor n e s s a n d Clark's l e v e l of invasion. D u e to the
w h e r e a s a s u b u n g u a l lesion m a y begin a s a red u s u a l d e l a y in t h e d i a g n o s i s , t u m o r s a r e u s u a l l y
m a c u l a r d y s c h r o m i a t h a t e v o l v e s to o n y c h o l y s i s thick at p r e s e n t a t i o n a n d , a s a c o n s e q u e n c e , m o r -
and a pyogenic granuloma-like lesion. tality is high. Early diagnosis and surgical
Atypical melanocytic hyperplasia should be r e m o v a l of N U M is n e c e s s a r y t o i m p r o v e cur-
considered in situ melanoma. Differentiation rently poor survival r a t e s . Initial a s s e s s m e n t ,
between acrolentiginous melanoma (at least staging, and follow-up are similar to t h o s e of
80 per cent of m e l a n o m a c a s e s ) , superficial s p r e a d - m e l a n o m a s of o t h e r skin s i t e s . W i d e local s u r g i c a l
ing melanoma (rare), and nodular melanoma excision of t h e lesion is r e c o m m e n d e d . T h e r e
( d e b a t a b l e ) is of little interest for t h e h a n d s u r g e o n , are no clear surgical guidelines. For m e l a n o m a
 WHAT CAN THE HAND S U R G E O N DO W H E N FACING A PATIENT WITH A MELANONYCHIA? 125

in s i t u , w e r e c o m m e n d c o m p l e t e e x c i s i o n of t h e t o e s ) . S e n t i n e l n o d e b i o p s y is e x p e r i m e n t a l but
nail a p p a r a t u s t o t h e u n d e r l y i n g b o n e f o l l o w e d b y often p r a c t i c e d at s e l e c t e d r e s e a r c h c e n t e r s f o r
a full t h i c k n e s s graft ( M o e r h l e et al 2003, Lazar, high-risk l e s i o n s . T h e r a p e u t i c lymphadenectomy
submitted for publication) ( F i g . 6). F o r i n v a s i v e is a d v i s e d w h e n there is clinical evidence of
m e l a n o m a a m p u t a t i o n of t h e digit/toe is r e q u i r e d , m e t a s t a t i c d i s e a s e in r e g i o n a l l y m p h n o d e s .
s t u d i e s h a v e s h o w n n o b e n e f i t of p r o x i m a l o v e r
distal a m p u t a t i o n s ( S l i n g l u ff et al 1990, Park et al
1992, F i n l e y et a l 1994, Q u i n n et al 1996, B r o c h e z
Benign melanotic lesions:
et al 2000, O ' L e a r y et al 2000, W a g n e r et al 2000).
P r o v i d e d t h a t a d e q u a t e e x c i s i o n of t h e l e s i o n is melanocytic nevus and
p e r f o r m e d , t h e l e v e l of a m p u t a t i o n is c h o s e n t o lentigo of the nail unit
o b t a i n t h e best f u n c t i o n a l o u t c o m e (distal inter-
p h a l a n g e a l joint for t h e f i n g e r s , p r o x i m a l inter- Melanocytic n e v u s of the nail apparatus is a
p h a l a n g e a l or m e t a t a r s o p h a l a n g e a l joint for the benign neoplasm derived from melanocytes.
J u n c t i o n a l n e v u s s h o w s m e l a n o c y t e s a r r a n g e d in
n e s t s at t h e d e r m o e p i d e r m a l j u n c t i o n (Tosti et al
1996). Lentigo shows melanocyte hyperplasia,
t h e r e is a n i n c r e a s e in t h e n u m b e r of m e l a n o c y t e s
a m o n g b a s a l o n y c h o c y t e s (Tosti et al 1996). T h e
clinical a s p e c t s of nail m a t r i x n e v i ( N M N ) a r e
often i n d i s t i n g u i s h a b l e f r o m t h o s e o f m e l a n o m a s
(Tosti et al 1996). N M N m a y b e c o n g e n i t a l or
a c q u i r e d . A m o n g a d u l t s , N M N a p p e a r in t h e t h i r d
decade of life. T h e t h u m b and great toe are
affected in a l m o s t t w o - t h i r d s of c a s e s . N M N p r e -
s e n t s a s a m o n o d a c t y l o u s L M t h a t is m o s t often
w i d e ( m e a n w i d t h 4 m m , t o t a l l y black nail in 15
p e r c e n t of patients ) ( B a r a n a n d K e c h i j i a n 1989,
Tosti et al 1996). T h e c o l o r is dark b r o w n in t w o -
t h i r d s o f c a s e s s o m e t i m e s w i t h l i n e a r s t r e a k s of
h y p e r p i g m e n t a t i o n , w h i l e a light b r o w n c o l o r is
n o t e d in o n e - t h i r d of p a t i e n t s (Tosti et al 1996).
Figure 5
Nail plate alterations, Hutchinson's sign, gradual
Early-evolving melanoma with pseudo-Hutchinson's sign e n l a r g e m e n t , or f a d i n g a r e m o r e r a r e ly reported
(periungual spread of pigmentation to the proximal nail fold) (Tosti e t a l 1996).The clinical differential d i a g n o s i s
in a patient with subungual melanoma. of N M N is t h e s a m e a s that of L M . B e c a u s e it is

Table 2 Other possible etiologies for Hutchinson's sign (from Baran and Dawber 1995)

Laugier-Hunziker syndrome L M is associated with macular pigmentation of lips and mouth

Congenital nevus After biopsy
X-ray therapy X-ray therapy for finger dermatitis has been reported to be responsible for L M and
periungual pigmentation
Malnutrition Some drugs have also been reported
Drug ingestant Minocycline, zidovudine
Peutz-Jeghers syndrome
AIDS L M and pigmentation of distal pulp have been reported in an AIDS patient, even
before treatment
'Childhood melanosis' Observed in Japanese children, may undergo spontaneous regression
Trauma induced Friction and pressure have been reported in the nails of a boxer

L M , longitudinal melanonychia
 126 T U M O R S OF THE HAND

Figure 6

In situ subungual melanoma treated by excision of the nail

unit (a) and full-thickness skin grafting (b). Excellent func-
tional and acceptable cosmetic results at 2 years follow-up
c (c) without recurrence at 5 years.

not possible to differentiate adequately NMM b y c u l t u r e , self-inflicted injury), w e recommend

f r o m e a r l y - e v o l v i n g N U M o n clinical g r o u n d s o r regular follow-up, a n d w e biopsy a n y L M that
o n a partial b i o p s y , t h e d i a g n o s t i c s t e p s p r o p o s e d c h a n g e s . A n u m b e r of m e t h o d s h a v e b e e n r e c -
in t h e L M d i a g n o s i s s e c t i o n s h o u l d b e f o l l o w e d o m m e n d e d t o b i o p s y L M , o n l y s o m e of w h i c h w i l l
in o r d e r to a s c e r t a i n t h a t L M is p r o d u c e d b y a n be discussed here.
i n c r e a s e in t h e n u m b e r of m e l a n o c y t e s . W h e n
h i s t o l o g y of t h e p u n c h b i o p s y s h o w s m e l a n o c y t e
h y p e r p l a s i a or a n e v u s , a c o m p l e t e surgical e x c i -
s i o n of s u s p e c t e d N M N is g e n e r a l l y r e c o m m e n d e d Punch biopsy
in a d u l t s (Tosti et al 1996).
To p e r f o r m a p u n c h b i o p s y , t h e p r o x i m a l nail w a l l
is b e n t b a c k w a r d s a w a y f r o m t h e u n d e r l y i n g nail
p l a t e . T h e p u n c h b i o p s y is c a r r i e d o u t a t t h e prox-
Biopsy techniques i m a l part of t h e p i g m e n t e d nail s t r e a k t h r o u g h
t h e nail plate a n d m a t r i x t o t h e u n d e r l y i n g b o n e .
W e r e c o m m e n d t h e b i o p s y of all s o l i t a r y L M in However, punch biopsies, which are usually
a d u l t s t h a t h a v e n o c l e a r l y identified c a u s e , e v e n 2 - 3 m m l a r g e , a r e difficult to interpret a n d it is not
if t h e e t i o l o g y is o b v i o u s ( o n y c h o m y c o s i s p r o v e n p o s s i b l e to rule out complete excision of the
 WHAT CAN THE HAND S U R G E O N DO W H E N FACING A PATIENT WITH A MELANONYCHIA? 127

a b

Figure 7

Lentigo of the nail unit presenting as a longitudinal melanonychia (a). Removal of only the proximal half of the nail plate allows
for precise localization of the lesion (b). Longitudinal excision and closure by undermining the matrix follows.

lesion e v e n with the modification of B a r a n a n d p e r f o r m e d . T h i s w i l l a l l o w e x c i s i o n of probably

K e c h i j i a n (1989). F o r t h i s r e a s o n s o m e a u t h o r s t h e m o s t i m p o r t a n t part of t h e m e l a n o n y c h i a a n d
prefer larger L M s a m p l i n g . H o w e v e r , this tech- c l o s u r e of t h e m a t r i x w i t h l i m i t e d s c a r r i n g .
nique is s i m p l e and a d e q u a t e to rule out a
melanocytic lesion. Excisional biopsies
Excisional biopsy technique depends on the
Surgical biopsies w i d t h a n d l o c a t i o n of t h e p i g m e n t e d streak. W h e n
t h e r e is a h i g h s u s p i c i o n i n d e x for a m e l a n o m a
Limited surgical biopsies ( a g e > 50, l a r g e b a n d > 5 m m , H u t c h i n s o n ' s s i g n ,
r e c e n t e n l a r g e m e n t , or m o d i f i c a t i o n ) a c o m p l e t e
It is better t o r e m o v e o n l y t h e p r o x i m a l half of t h e
s u r g i c a l e x c i s i o n o r ' e n b l o c ' e x c i s i o n of t h e l e s i o n
nail p l a t e , l e a v i n g t h e distal half a t t a c h e d to t h e
is m a n d a t o r y .
nail b e d ( F i g . 7 a ) . T h i s t e c h n i q u e h a s t w o a d v a n -
t a g e s : first, it l e a v e s a c o l o r e d l a n d m a r k which
Lateral longitudinal melanonychia
h e l p s t o d e l i n e a t e better t h e p r o x i m a l site of t h e
L M ( F i g . 7 b ) ; a n d , s e c o n d , it p r o t e c t s t h e patient In lateral L M l e s i o n s , it is s i m p l e r to r e m o v e all
f r o m p a i n d u r i n g d r e s s i n g , a n d t h e nail b e d f r o m the tissues 'en bloc', leaving the nail plate
a u s e l e s s t r a u m a . E x c i s i o n of t h e L M c a n t h e n b e a t t a c h e d to t h e u n d e r l y i n g t i s s u e s in o r d e r to
performed two w a y s . facilitate t h e p a t h o l o g i s t ' s i n t e r p r e t a t i o n . Incision
is m a d e w i t h a n o . 15 s c a l p e l b l a d e o n t o t h e nail
Longitudinal excision plate, with at least a 1-mm margin (Fig. 8a).
B e c a u s e of t h e h a r d n e s s of t h e nail p l a t e , a rock-
E x c i s i o n is p e r f o r m e d f r o m t h e proximal matrix to
ing m o t i o n is n e c e s s a r y to g e t t h r o u g h t h e nail
t h e distal part of t h e lunula. U n d e r m i n i n g of t h e tis-
plate. The incision is prolonged to bone.
s u e s is n e c e s s a r y to c l o s e t h e defect but a lesion
P r o x i m a l l y , t h e i n c i s i o n m u s t i n c l u d e all t h e nail
w i d e r t h a n 3 m m c a n n o t b e c l o s e d using this t e c h -
m a t r i x , i.e. it s h o u l d extend up to the dorsal
n i q u e . T h e proximal half of t h e nail plate is replaced
r i d g e s of t h e i n t e r p h a l a n g e a l j o i n t but r e s p e c t t h e
at t h e e n d of t h e p r o c e d u r e to protect t h e repair.
extensor t e n d o n . Laterally, incisions follow the
lateral w a l l , w i t h a 1-mm margin, however, the
Horizontal e x c i s i o n
nail h o r n m u s t b e r e m o v e d p r o x i m a l l y a n d v e n -
To limit t h e risk of nail r i d g i n g , in larger l e s i o n s , a t r a l l y to a v o i d r e g r o w t h of nail s p i k e s ( F i g . 8 b ) .
horizontal e x c i s i o n of t h e p r o x i m a l b a n d c a n b e Once the excisional-biopsy specimen has been
128 T U M O R S O F T H E HAND

c d

Figure 8

Lateral longitudinal melanonychia that proved to be a nevus. Excisional biopsy with 1 mm margin (a). Care should be taken to
remove totally the proximal part, and especially the proximal horn (b). Reconstruction is performed by mobilization of the lat-
eral part of the pulp as described by Dubois (1974) (c), which gave an excellent functional and esthetical result at 1 year (d).

removed, reconstruction of t h e lateral w a l l is t h a t is n e c e s s a r y . It is t h e n p o s s i b l e t o l e a v e t h e

m a d e e a s i e r b y m o b i l i z a t i o n of t h e lateral p u l p a s t w o half nail p l a t e s a n d fix t h e m u s i n g t h e nail
d e s c r i b e d b y D u b o i s (1974) ( F i g . 8 c a n d d ) . s u t u r e t e c h n i q u e of F o u c h e r et al (1984) ( F i g . 9).
W i t h g r e a t e r w i d t h s , t h e u n d e r m i n i n g of t h e
matrix a n d nail b e d f r o m t h e u n d e r l y i n g bone
Central longitudinal melanonychia,
s h o u l d e x t e n d t o t h e f i b r o f a t t y t i s s u e of t h e lateral
less than 3 m m w i d e
nail f o l d . N a i l p l a t e r e m o v a l is m a n d a t o r y . T h i s
I n c i s i o n s m u s t b e m a d e o n both s i d e s of t h e prox- a l l o w s mobilization of t w o m a t r i x a n d nail b e d
i m a l nail w a l l to b e n d it b a c k w a r d s a n d a l l o w f l a p s , a s d e s c r i b e d b y J o h n s o n (1971). C l o s u r e is
c l e a r v i s u a l i z a t i o n of t h e l o n g i t u d i n a l m e l a n o n y - m a d e w i t h 6-0 a b s o r b a b l e s u t u r e s . T h e m o s t criti-
c h i a . T h e p r i n c i p l e s a n d t e c h n i q u e of e x c i s i o n a r e cal s u t u r e p l a c e m e n t is in t h e m o s t p r o x i m a l nail
s i m i l a r but o n c e e x c i s i o n h a s b e e n d o n e , r e c o n - m a t r i x . C a r e f u l c l o s u r e of t h e nail m a t r i x , espe-
s t r u c t i o n of t h e m a t r i x s h o u l d b e p e r f o r m e d c a r e - cially t h e m o s t p r o x i m a l part, a f f o r d s t h e b e s t
f u l l y t o limit t h e p o t e n t i a l f o r r i d g i n g o r splitting o p p o r t u n i t y t o a v o i d a split nail o r s i g n i f i c a n t nail
n a i l . T h e s i d e s of e x c i s i o n m u s t b e m o b i l i z e d to d y s t r o p h y . T h e l a r g e r of t h e t w o half nail p l a t e s i s
c l o s e t h e d e f e c t . If t h e w i d t h of e x c i s i o n is n a r r o w , replaced with a b s o r b a b l e s u t u r e s to mold the
t h e n a f e w m i l l i m e t e r s of u n d e r m i n i n g m a y b e all repair, a v o i d t h e c r e a t i o n of a p t e r y g i u m , and
 WHAT CAN THE HAND S U R G E O N DO W H E N FACING A PATIENT WITH A MELANONYCHIA? 129

Figure 9

Central longitudinal melanonychia. Closure using absorbable 6-0 sutures on the proximal matrix, and nail suture according to
Foucher on the nail bed.

a b

Figure 10

Large central longitudinal melanonychia. The proximal half of the nail plate was removed (a) and shaving of the lesion was
performed using the technique described by Haneke (1999, 2003} (b). The specimen can be seen placed on a thick and rigid
structure to facilitate its analysis.

p r o t e c t b o t h t h e p a t i e n t a n d t h e repair. L a t e r a l t e c h n i q u e ( H a n e k e 1999, 2003) for medium-sized

p r o x i m a l w a l l i n c i s i o n s a r e c l o s e d w i t h 4-0 o r 5-0 l e s i o n s that w e s u s p e c t to b e b e n i g n or t h a t m a y
absorbable sutures. r e p r e s e n t a n e a r l y - e v o l v i n g m e l a n o m a ( F i g . 10).
This technique is delicate and necessitates
Central longitudinal melanonychia,
adequate training. Proximal avulsion exposes the
more than 3 m m
m a t r i x , p r o x i m a l nail f o l d retraction is n e c e s s a r y
U s u a l excisional biopsy will l e a v e the patient with for proximal band, a magnifying g l a s s is v e r y
a p e r m a n e n t , dystrophic nail. W e use the s h a v e h e l p f u l , A n o . 11 b l a d e is u s e d to m a k e a v e r y
130 T U M O R S O F T H E HAND

s h a l l o w vertical incision circumscribing the lesion t h e f a c t t h a t d i a g n o s e s a r e m a d e at later s t a g e s .

to be excised. The blade is t h e n tangentially Prognosis of subungual melanoma depends
o r i e n t e d a n d t h e l e s i o n is s u p e r f i c i a l l y s h a v e d off. m o s t l y o n its t h i c k n e s s ; p r e v i o u s s e r i e s r e p o r t e d
T h e t h i n n e s t s l i c e t h a t is p o s s i b l e w i t h a h a n d - s u r v i v a l r a t e s of 16-60 p e r c e n t a t 5 y e a r s , h o w -
held scalpel reaches the l e v e l of t h e reticular ever, B r e s l o w ' s index w a s higher than 4 m m in
d e r m i s . M e d i u m - d e p t h excisions of this t y p e heal most series. Conversely, early-evolving melano-
with minimal scarring. m a s m a y be definitely c u r e d after s i m p l e n o n -
m u t i l a t i n g s u r g e r y . F o r t h a t r e a s o n , all s u s p i c i o u s
Very large band and black nail L M m u s t benefit f r o m a n a d e q u a t e biopsy to rule
out subungual m e l a n o m a .
H i g h l y s u s p i c i o u s l e s i o n s t h o u g h t to b e i n v a s i v e
m a l i g n a n t m e l a n o m a m a y benefit f r o m a total nail
unit e x c i s i o n f r o m t h e o n s e t . T h a t d e c i s i o n s h o u l d
b e m a d e in c o l l a b o r a t i o n w i t h a d e r m a t o l o g i s t or
Bibliography
s u r g e o n e x p e r i e n c e d in nail d i s e a s e .
Abimelec P, Grussendorf-Conen El (2001) Hair and nail
tumors. In: Hordinsky S , Scher R, eds. Hair and Nail
Diseases. Appleton & Lange: N e w York.
Conclusion
Baran R, Dawber RPR (1995) Diseases of the Nail and
In s u m m a r y , t h e d i a g n o s i s of L M r e q u i r e s t h e their Management, 2nd edn. Blackwell: Oxford: 513.
following steps:
Dumontier C (2000) L'ongle. Elsevier: Paris: 248.
(1) Careful m e d i c a l history, clinical e x a m i n a t i o n ,
and adequate diagnostic t e s t s to exclude Krull E A , Zook E G , Baran R, Haneke E (2001) Nail
medical causes known to produce LM or Surgery. AText and Atlas. Lippincott Williams & W i l k i n s :
pseudo-LM. Philadelphia: 415.
(2) B i o p s y of t h e m a t r i c a l o r i g i n of a n y adult
m o n o d a c t y l o u s m e l a n o n y c h i a of w h i c h t h e r e
is not a d e q u a t e e x p l a n a t i o n .
(3) W h e n t h e r e is a h i g h s u s p i c i o n i n d e x for a
References
melanoma ( a g e > 50, large band > 5 m m ,
Baran R, Kechijian P (1989) Longitudinal melanonychia
Hutchinson's sign, recent e n l a r g e m e n t , or (melanonychia striata): diagnosis and management,
m o d i f i c a t i o n ) a c o m p l e t e s u r g i c a l e x c i s i o n of J Am Acad Dermatol 21:1165-75.
t h e l e s i o n is r e c o m m e n d e d .
(4) For polydactylous melanonychia or children Baran R, Kechijian P (1996) Hutchinson's sign: a reap-
with monodactylous melanonychia, follow- praisal, J Am Acad Dermatol 34:87-90.
u p a n d p h o t o g r a p h at r e g u l a r i n t e r v a l s a n y
single b a n d that m a y be clinically suspected Blessing K, Kernohan N M , Park K G (1991) Subungual
t o b e m e l a n o m a w i t h b i o p s y in c a s e of c o l o r malignant melanoma: clinicopathological features of
modifications or gradual enlargement. 100 cases, Histopathology 19:425-9.

(5) C o m p l e t e surgical excision if t h e b i o p s y s h o w s

Brochez L, Verhaeghe E, Sales F et al (2000) Current
a n i n c r e a s e in t h e n u m b e r of m e l a n o c y t e s .
guidelines in melanoma treatment, Dermatology
L o n g i t u d i n a l m e l a n o n y c h i a is a f r e q u e n t l e s i o n , 200:160-6.
i n c i d e n c e of w h i c h is u n d e r e v a l u a t e d ; it is n o w
r e c o g n i z e d a s b e i n g a n e a r l y s i g n of m e l a n o m a . Dubois J P (1974) Un traitement de l'ongle incarné, Nouv
Presse Med 3:1938.
The hand surgeon should always have a high
suspicion index for a s u b u n g u a l m e l a n o m a a n d
Finley R, Driscoll DL, Blumenson L E , Karakousis C P
p e r f o r m a b i o p s y o n all i s o l a t e d L M w h e r e t h e
(1994) Subungual melanoma: an eighteen-year review,
d i a g n o s i s is not c l e a r l y p r o v e n . It is k n o w n t h a t Surgery 116:96-100.
late d i a g n o s i s is m o s t l y d u e to d o c t o r m i s d i a g n o -
sis. T h e prognosis of malignant melanoma is Foucher G , Merle M , Van Genechten F, Schuind F (1984)
r e p u t e d t o b e v e r y s e v e r e , this is m o s t l y o w i n g to La synthèse unguéale, Ann Chir Main 3:168-9.
 WHAT CAN THE HAND S U R G E O N DO W H E N FACING A PATIENT W I T H A MELANONYCHIA? 131

Haneke E (1999) Operative therapie akraler und Park K G , Blessing K, Kernohan N M (1992) Surgical
subungualer Melanome. In: Rompel J , Petres J , eds. aspects of subungual malignant m e l a n o m a s . T h e
Operative onkologische Dermatologie. 15. Springer: Scottish Melanoma Group, Ann Surg 216:692-5.
Heidelberg: 210-14.
Quinn M J , Thompson J E , Crotty K et al (1996) Sub-
Haneke E (2003) The shave biopsy. Presented at ungual melanoma of the hand, J Hand Surg Am 2 1 :
Sixth Meeting of the European Nail Society, Barcelona, 506-11.
October.
Slingluff C L , Vollmer R, Seigier HF (1990) Acral
J o h n s o n R K (1971) Nailplasty, Plast Reconstr Surg melanoma: a review of 185 patients with identification
47:275-6. of prognostic variables, J Surg Oncol 45:91-8.

KatoT, SuetakeT, S u g i y a m a Y et al (1996) Epidemiology Tosti A , Baran R, Piraccini B M et al (1996) Nail matrix
and prognosis of subungual melanoma in 34 Japanese nevi: a clinical and histopathologic study of twenty-two
patients, Br J Dermatol 134:383-7. patients, J Am Acad Dermatol 34:765-71.

Moerhle M , Metzger S , Schippert W et al (2003) Wagner J D , Gordon M S , Chuang T-Y, Coleman J lllrd
'Functional surgery' in subungual melanoma, Dermatol (2000) Current therapy of cutaneous melanoma, Plast
Surg 29:366-74. Reconstr Surg 105:1774-99.

O'Leary J A , Berend K R , J o h n s o n J L et al (2000)

Subungual melanoma. A review of 93 cases with identi-
fication of prognostic variables, Clin Orthop 378:206-12.
 14
Malignant melanoma
Margita Flügel

Incidence of melanoma in g r o w t h of c e r t a i n parts of t h e l e s i o n l e a d to

a s y m m e t r y of s h a p e . ' B ' is for b o r d e r : in c o n t r a s t
M e l a n o m a is t h e m o s t r a p i d l y i n c r e a s i n g c a n c e r to d y s p l a s t i c n e v u s , t h e b o r d e r of m e l a n o m a is
in W e s t e r n p o p u l a t i o n s . It is i n c r e a s i n g at a n often w e l l d e f i n e d (not f a d i n g ) a n d j a g g e d o r w i t h
a l a r m i n g rate. O n e in e v e r y 75 E u r o p e a n s w i l l b e t o n g u e - s h a p e d e d g e s (not rounded) w i t h s o m e
at risk of d e v e l o p i n g m a l i g n a n t m e l a n o m a ( W o l f p r o m o n t o r i e s . 'C' is for color: l a r g e d i f f e r e n c e s in
e t a l 2001). c o l o r or c h a n g e s of c o l o r w i t h i n t h e l e s i o n a r e
U p t o 20 p e r c e n t o f m e l a n o m a s o c c u r o n t h e suspicious. S o m e t i m e s a narrow red halo occurs
u p p e r l i m b (Table 1). In c a s e s of localization on around the edge. ' D ' is for diameter: lesions
t h e h a n d , p a t i e n t s w i l l , for t h e m o s t part, p r e s e n t greater than 6 mm and recent growth of the
to a s u r g e o n f o r t h e i r initial e v a l u a t i o n , w h e n l e s i o n r e q u i r e i n v e s t i g a t i o n . ' E ' is for e l e v a t i o n :
seeking medical attention for suspicious skin d i a g n o s i s s h o u l d b e m a d e b e f o r e distinct e l e v a -
lesions. For this reason a n d b e c a u s e only h a n d t i o n of t h e t u m o r is o b v i o u s .
s u r g e o n s a r e a b l e to p r e s e r v e a s m u c h function O o z i n g a n d c h a n g e s in s e n s a t i o n a r e m i n o r or
as adequate tumor treatment a l l o w s they must be late f e a t u r e s ( R o b e r t s et al 2002).
f a m i l i a r w i t h t h e n a t u r e of t h e t u m o r . A l l c l i n i c i a n s a n d p a t i e n t s w i t h a s p e c i f i c risk
should be trained in t h e recognition of early
m e l a n o m a . A g o o d clinical history is important
a n d s h o u l d i n c l u d e a n y c h a n g e s in t h e l e s i o n ,
Clinical diagnosis of melanoma history of skin lesions, and family history of
m e l a n o m a . E x a m i n a t i o n s h o u l d b e u n d e r t a k e n in
T h e A B C D E s y s t e m o f d i a g n o s i s of melanoma g o o d lighting u s i n g m a g n i f i c a t i o n . T r a i n i n g and
e m p h a s i z e s t h e h i s t o r y of c h a n g e in size, s h a p e , experience with skin-surface microscopy is
a n d c o l o r of a pre-existing p i g m e n t e d l e s i o n . It is mandatory for all t h o s e who are particularly
r e c o m m e n d e d for u s e in b o t h patient a n d p h y s i - i n v o l v e d in t r e a t m e n t of skin t u m o r s .
c i a n e d u c a t i o n (Clinical P r a c t i c e G u i d e l i n e s 1999, P a t i e n t s w i t h high-risk are considered to be
G Ö h l e t al 2000). 'A' is f o r a s y m m e t r y : d i f f e r e n c e s t h o s e w i t h e i t h e r a p r e v i o u s m e l a n o m a or l a r g e

Table 1 Incidence of malignant melanoma from 1978 to 2002 at the Department of Surgery and the Department of
Dermatology University of Erlangen (Hohenberger and Schuler)

Localization 1978-1982 1983-1987 1988-1992 1993-1997 1998-2002

Upper extremity 93 123 115 139 202

Lower extremity 248 285 239 180 294
Head, body, occult 166 304 346 344 517

Total 507 712 700 663 1013

134 T U M O R S OF THE HAND

n u m b e r s of m o l e s , s o m e of w h i c h a r e c l i n i c a l l y S u r g e r y for melanoma of any thickness is to

atypical. Patients with a giant congenital pig- ensure complete excision confirmed by histo-
m e n t e d h a i r y n e v u s a r e at g r e a t l y i n c r e a s e d risk logically negative m a r g i n s . Recent studies h a v e
(100 times that of the general population). s h o w n t h a t s u r g i c a l m a r g i n s of u p t o 5 c m - a s
I n d i v i d u a l s w i t h a f a m i l y h i s t o r y of t h r e e o r m o r e formerly recommended - have no statistically
c a s e s of m e l a n o m a a r e a l s o c o n s i d e r e d to b e of s i g n i f i c a n t effect o n s u r v i v a l o r r e c u r r e n c e .
h i g h risk for m e l a n o m a ( R o b e r t s e t al 2002). The histological assessment follows two
T h e p r o p h y l a c t i c e x c i s i o n of s m a l l c o n g e n i t a l i m p o r t a n t criteria. A c c o r d i n g to C l a r k t h e l e v e l of
n e v i is not r e c o m m e n d e d . i n v a s i o n to the anatomic stratum of t h e skin
should b e m e a s u r e d (Clark l e v e l ) (Clark et al
1969). B r e s l o w (1970) p r o p o s e d t h a t t h e m a x i m a l
v e r t i c a l g r o w t h s h o u l d b e m e a s u r e d in millime-
Histological diagnosis ters ( B r e s l o w thickness).These m e a s u r e m e n t s are
t h e b a s i s for p T c l a s s i f i c a t i o n of t h e T N M ( t u m o r ,
A n y lesion that c a n be fully excised including an nodes, metastasis) staging system (American
a d e q u a t e ( G Ö h l et al 2000) p o r t i o n of u n d e r l y i n g Academy of Dermatology Association 2001,
s u b c u t a n e o u s tissue should be r e m o v e d totally R o b e r t s et al 2 0 0 2 , W e e d o n 2002) ( T a b l e 2).
w i t h a 5 - m m clinical m a r g i n of n o r m a l skin later-
ally a n d w i t h a sufficiently l a r g e cuff of s u b d e r m a l
t i s s u e s ( W o l f e t al 2001 ( . T h i s a l l o w s c o n f i r m a t i o n
of t h e d i a g n o s i s , s u c h t h a t s u b s e q u e n t d e f i n i t i v e Lentigo maligna
treatment c a n be b a s e d on B r e s l o w thickness.
S h a v e a n d punch biopsies, laser excision, or C h a n g i n g flat p i g m e n t e d l e s i o n s o n t h e d o r s u m
c u r e t t a g e a r e not r e c o m m e n d e d b e c a u s e t h e y , at of t h e h a n d or o n t h e f a c e m a y r e p r e s e n t lentigo
t h e v e r y l e a s t , m a k e t h e p a t h o l o g i c s t a g i n g of t h e m a l i g n a . H i s t o l o g i c a l l y c o n f i r m e d lentigo m a l i g n a
lesion impossible. is b e s t t r e a t e d b y c o m p l e t e e x c i s i o n b e c a u s e of
Incisional biopsy is o c c a s i o n a l l y a c c e p t a b l e , t h e risk of i n v a s i v e c h a n g e . O n t h e d o r s u m of t h e
for example, in the differential diagnosis of h a n d the defect can usually be closed by flap and
lentigo m a l i g n a o n t h e f a c e o r of a c r a l m e l a n o m a skin t r a n s p l a n t a t i o n p r o c e d u r e s . N o f u r t h e r treat-
if t o t a l e x c i s i o n w o u l d i m p a i r f u n c t i o n . T h e r e is m e n t is t h e n r e q u i r e d .
little e v i d e n c e that incisional biopsies of m e l a n o m a A l t h o u g h r e c u r r e n c e of o t h e r t y p e s of in situ
affect t h e p r o g n o s i s . There is no place for inci- m e l a n o m a is r a r e , r e c u r r e n c e of l e n t i g o maligna
sional biopsy in primary care. is c o m m o n a n d is u s u a l l y a t t r i b u t e d to a ' f i e l d
B i o p s i e s of possible subungual melanomas effect', whereby atypical melanocytes extend
(differential d i a g n o s i s : s u b u n g u a l n e v u s ) s h o u l d laterally a l o n g t h e e p i d e r m i s b u t a r e not c l i n i c a l l y
b e p e r f o r m e d o n l y b y h a n d s u r g e o n s . T h e nail detectable.
should be r e m o v e d a n d clinically o b v i o u s tumor, Different t y p e s of m a l i g n a n t m e l a n o m a c a n b e
or in t h e a b s e n c e of a m a s s t h e nail matrix, distinguished:
should be adequately sampled. W h e n e v e r possi-
b l e t h e nail b e d s h o u l d b e r e c o n s t r u c t e d micro- (1) Superficial spreading melanoma s h o w s nests
s u r g i c a l l y a n d t h e nail itself p u t back in place, of t u m o r c e l l s in all e p i d e r m a l l a y e r s , c h a r a c -
p r o v i d e d t h a t m e l a n o m a d i a g n o s i s h a s not b e e n terized b y c h a n g e s in size a n d pleomorphic
confirmed. nuclei.
I n d i v i d u a l m e l a n o m a c e l l s h a v e t h e c a p a c i t y to (2) Nodular melanoma s h o w s vertical growth
migrate locally away from the tumor origin. over the w h o l e tumor.
M e l a n o m a m a y e x t e n d w i d e r o r d e e p e r t h a n is (3) Acral lentiginous melanoma is c h a r a c t e r i z e d
visible to the e y e . T h e r e f o r e , the task force rec- b y l e n t i g i n o u s g r o w t h a l o n g t h e rete r i d g e s ,
ommends that a margin of clinically normal- but all t y p e s of melanoma can occur on
a p p e a r i n g skin s h o u l d b e r e m o v e d a r o u n d the h a n d s a n d f e e t ( S m i t h et al 1993).
tumor site to e n s u r e c o m p l e t e r e m o v a l of the (4) Lentigo maligna melanoma appears to
t u m o r ( M e y e r et al 2000). T h e v e r t i c a l d i m e n s i o n e x t e n d laterally v e r y s l o w l y o v e r a p e r i o d of
of e x c i s i o n h a s a l s o b e e n p r o v e n to b e of p r o g - m a n y y e a r s . It u s u a l l y o c c u r s in skin w i t h
n o s t i c v a l u e f o r t h e patient ( W o l f et al 2001). actinic damage. The tumor cells may be
 MALIGNANT M E L A N O M A 135

Table 2 pT classification

Breslow thickness (vertical)

pT-Category thickness of the tumor (mm) Clark's level of dermal invasion

pTI < 0.75 II: invasion of the papillae

pT2 < 0.75-1.5 III: involvement of superficial vascular plexus
pT3 > 1.5-4.0 IV: invasion of reticular dermis
pT3a > 1.5-3.0
pT3b > 3.0-4.0
pT4 > 4.0 V: involvement of subcutaneous tissue
pT4a > 4.0 and/or Clark level V
pT4b

Table 3 Histological type of melanoma of the upper extremity. (Data from the Department of Surgery and the Department
of Dermatology University of Erlangen (Hohenberger and Schuler))

1978-1982 1983-1987 1988-1992 1993-1997 1998-2002

Nodular melanoma 24 36 22 30 43
Lentigo maligna melanoma (LMM) 4 3 2 4 4
Superficial spreading melanoma (SSM) 48 67 68 87 125
Acral lentiginous melanoma (ALM) 7 6 11 9 13
Amelanotic melanoma - 2 6 2 4
Rare types, unknown 10 9 6 7 13

Total 93 123 115 139 202

arranged vertically a m o n g the basal kerato- (3) A n a t o m i c site of t h e l e s i o n ;

c y t e s ( G ö h l e t al 2000). (4) A full description of the macroscopic
(5) Amelanotic melanoma m a y b e light or pink a p p e a r a n c e of t h e t u m o r (possibly photo
s o it is i m p o r t a n t t o r e m e m b e r t h a t not all d o c u m e n t a t i o n ) , a n d t h e d i m e n s i o n s of t h e
m e l a n o m a s a r e of dark b r o w n - b l a c k color. s p e c i m e n in m i l l i m e t e r s ;
(5) Type of surgical procedure (excision,
A l l t y p e s of m a l i g n a n t m e l a n o m a m a y o c c u r in re-excision, incision biopsy);
the upper extremity, although in t h e p a l m a n d (6) W h e n p o s s i b l e , a s t a t e m e n t of w h e t h e r t h e
subungual region acral lentiginous melanoma lesion is primary, locally recurrent, or
t y p e is m o s t c o m m o n (Clark et al 1979) ( T a b l e 3). m e t a s t a t i c to t h e site;
(7) G r o s s and microscopic description of t h e
specimen;

Summary of (8)
(9)
W h e t h e r u l c e r a t i o n is p r e s e n t ;
Tumor thickness in millimeters Breslow's
diagnostic procedures l e v e l , m e a s u r e d f r o m t h e g r a n u l a r l a y e r of
t h e e p i d e r m i s to t h e b a s e of t h e t u m o r , to t h e
According to the guidelines for primary cuta- n e a r e s t 0.1 m m . U l c e r a t e d t u m o r s s h o u l d b e
n e o u s m e l a n o m a of the A m e r i c a n A c a d e m y of m e a s u r e d f r o m t h e b a s e of t h e ulcer to t h e
D e r m a t o l o g y A s s o c i a t i o n (2001) at t h e e n d of t h e b a s e of t h e t u m o r . T u m o r f o r m i n g a s h e a t h
diagnostic procedure the following should have around appendages should be excluded
been documented: w h e n m a k i n g m e a s u r e m e n t s (Table 2);
(10) Clark's l e v e l , i.e. t h e d e p t h of p e n e t r a t i o n of
(1) Patient's a g e a n d gender; the dermis m a y also be stated, although
(2) C l i n i c a l history, s p e c i a l risks; t h i s is a l e s s r e l i a b l e i n d i c a t o r of p r o g n o s i s
136 T U M O R S OF THE HAND

t h a n B r e s l o w thickness in m o s t c i r c u m s t a n c e s t u m o r s < 1.5 m m , in 15 per cent of tumors

(Table 2); measuring 1.5-3 m m , and 23 per c e n t of the
(11) M a r g i n i n v o l v e m e n t for s u r g i c a l e x c i s i o n s ; t u m o r s > 3 m m ( G Ö h l et al 2 0 0 0 ) .
(12) Growth phase; Real progress has been m a d e with the devel-
(13) T u m o r infiltrating l y m p h o c y t e s ; o p m e n t of s e l e c t i v e l y m p h n o d e b i o p s y d u r i n g
(14) M i t o t i c rate a n d r e g r e s s i o n ; recent years. Scintigraphic m e t h o d s a n d color
(15) A n g i o l y m p h a t i c invasion, microsatellitosis, marking allow the selective identification and
neurotropism, histological subtype; e x c i s i o n of t h e s o - c a l l e d s e n t i n e l n o d e w i t h h i g h
(16) W h e t h e r e x c i s i o n is c o m p l e t e a n d t h e m i n i - accuracy regarding occult early node involve-
mum m a r g i n of e x c i s i o n t o p e r i p h e r a l or m e n t w h i c h is of g r e a t r e l e v a n c e r e g a r d i n g p r o g -
d e e p s u r g i c a l m a r g i n m e a s u r e d in m i l l i m e - nosis and therapeutic decisions. Clinically
t e r s . If e x c i s i o n is not c o m p l e t e , t h e r e s i d u a l manifest regional lymph node metastases
d i s e a s e s h o u l d b e i d e n t i f i e d a s in situ o r r e m a i n an u n e q u i v o c a l indication for radical n o d e
invasive; dissection.
(17) Pathologic staging (pTNM classification
s y s t e m a c c o r d i n g to A m e r i c a n J o i n t C o m m i t -
tee o n Cancer ( A J C C ) / U n i o n Internationale
C o n t r e le C a n c e r ( U I C Q ) ( T a b l e 2 ) . Concepts for follow-up
M o r e t h a n 80 p e r c e n t of all r e c u r r e n c e s a r e pri-
marily detected during follow-up examinations
w h e n a s t r u c t u r e d s c h e d u l e is a p p l i e d ( T a b l e 5).
Treatment P h y s i c a l e x a m i n a t i o n r e v e a l s m o s t of t h e r e c u r -
r e n c e s ( G a r b e e t al 2003). L y m p h n o d e u l t r a s o u n d
The r e c o m m e n d e d surgical margins are those is a s e n s i t i v e e x a m i n a t i o n m e t h o d w h i c h e n a b l e s
m e a s u r e d c l i n i c a l l y at t h e t i m e of s u r g e r y , r a t h e r e a r l y d e t e c t i o n of l o c o - r e g i o n a l r e l a p s e s b e f o r e
than the histopathologic margins measured they b e c o m e palpable.
microscopically. T h e margins suggested may
n e e d to b e a d j u s t e d f o r f u n c t i o n a l or cosmetic
r e a s o n s e s p e c i a l l y in t h e h a n d . I n c i s i o n a l b i o p s y
is s o m e t i m e s n e c e s s a r y t o b e c e r t a i n of t h e d i a g - Table 4 Recommended margins for excision
nosis before for e x a m p l e doing a n amputation
w h i c h is n e c e s s a r y t o e x c i s e t h e t u m o r w i t h t h e Tumor thickness Clinical excision margins
recommended margins.
In situ 0.5 cm (no further treatment necessary)
In t h e past up to 80 p e r c e n t of lympha-
< 2 mm 1 cm
denectomies w e r e unnecessary because metas-
> 2 mm 2 cm
tases could be s h o w n in only 6 per cent of

Table 5 Follow-up schedule in cutaneous melanoma (Garbe et al 2003)

Frequency of
Frequency of Frequency of Frequency of Frequency of imaging techniques
physical physical lymph node blood tests (LDH, AP, (abdominal ultrasound,
Thickness/stage examination examination ultrasound protein S-100 β X-ray, CT MRI, or PET)
of the tumor years 1-5 years 6-10 years 1-5 years 1-5 years 1-5

I < 1 mm 6 months 12 months —

I + II > 1 mm 3 months 6-12 months 6 months 6 months
III 3 months 6 months 3-6 months 3-6 months h months
IV Individual

LDH, lactate dehydrogenase; AP, alkaline phosphatase; CT, computed tomography; MRI, magnetic resonance imaging;
PET positron emission tomography
 MALIGNANT M E L A N O M A 137

Systemic treatment of Breslow A (1970) Thickness, cross-sectioned area and

depth of invasion in prognosis of cutaneous melanoma,
metastatic melanoma Ann Surg 172:902-7.

O n t h e b a s i s of a n u m b e r of r a n d o m i z e d clinical tri- Clark W H J r , From L, Bernardino E A (1969) T h e

a l s Keilholz e t al (2003) c o n c l u d e d that e v i d e n c e - histogenesis and biologic behaviour of primary human
b a s e d a s s e s s m e n t of a v a i l a b l e c o m p o u n d s is n o w malignant melanoma of the skin, Cancer Res 29:705-11.
possible. T h e agents dacarbazine, vindesine, cis-
platin, a n d i n t e r f e r o n - αa r e l i c e n s e d . In s o m e c o u n - Clark W H , Bernardino E A , Reed R J , Kopf A W (1979)
tries, f o t e m u s t i n e a n d the cytokine interleukin-2 Acral lentiginous melanomas including of the mucous
are also licensed.The treatment recommendations membrane. In: Clark W H , Goldman LI, Mastrangelo M J ,
eds. Human Malignant Melanoma. Grune & Stratton:
a r e for s i n g l e - a g e n t t r e a t m e n t b e c a u s e c o m b i n a -
N e w York, S a n Francisco, London.
t i o n t r e a t m e n t h a s not b e e n p r o v e n t o p r o v i d e a
s u r v i v a l benefit. I m m u n o l o g i c a l a p p r o a c h e s s u c h
Garbe C, Paul A , Kohler-Späth H et al (2003) Prospective
a s t u m o r v a c c i n e s a n d c o m b i n a t i o n of h i s t a m i n e
evaluation of a follow-up schedule in cutaneous
a n d interleukin-2 a r e u n d e r i n v e s t i g a t i o n .
melanoma patients. Recommendations for an effective
In a d d i t i o n t o m e d i c a l t r e a t m e n t , r e s e c t i o n of follow-up strategy, J Clin Oncol 21:520-9.
m e t a s t a s e s is i n d i c a t e d to b e a p p r o p r i a t e w h e n -
ever this is possible without high morbidity. Göhl J , Driesch P, M e y e r T (2000) Z e i t g e m ä ß e
Radiation treatment m a y b e i n d i c a t e d for local Diagnostik beim malignen M e l a n o m m , Chir Praxis
t u m o r c o n t r o l if t h e localization a l l o w s a sufficient 57:391-402.
d o s e to be administered.
Particularly in melanoma of the extremities Keilholz U,Tilgen W , Hohenberger W (2003) Sytemische
t h e r e a r e additional t h e r a p e u t i c o p t i o n s u s i n g per- Therapie des metastasierenden M e l a n o m s , Dtsch
f u s i o n of t h e t i s s u e s w i t h s u b s t a n c e s far t o o toxic Arztebl 100A: 1054-64 [Heft 16].
for s y s t e m i c application. B y isolated l i m b perfusion
o f extremities w i t h t u m o r n e c r o s i s factor ( T N F ) a n d Meyer T, Driesch P, Grabenbauer G C , Göhl J (2000).
m e l p h a l a n , a l m o s t 100 per c e n t c o m p l e t e r e m i s - Aktuelle therapiekonzepte beim malignen Melanom,
s i o n of local m e t a s t a s e s c o u l d b e a c h i e v e d (Keilholz Chir Praxis 57:579-93.
et al 2003). O t h e r a u t h o r s prefer d r u g s w i t h less
toxicity b e c a u s e of a high rate of s e v e r e c o m - N H M R C (1999) Clinical Practice Guidelines: The man-
plications such as compartment compression agement of cutaneous melanoma. National Health and
Medical Research Council: Canberra, Australia.
s y n d r o m e , tissue especially muscle necrosis s o m e -
times necessitates amputation a n d s o m e systemic
Roberts DLL, Anstey AV, Barlow R J , Cox NH on behalf of
toxicity o c c u r s d u e to l e a k a g e ( A r i y a n 2003).
the British Association of Dermatologists and Newton
If left u n t r e a t e d , t h e s e local in transit lesions
Bishop J A , Corrie P G , Evans J et al (2002) on behalf of the
w i l l l e a d to b u l k y d i s e a s e that w i l l u l c e r a t e , b l e e d , Melanoma Study Group: UK guidelines for the manage-
a n d c a u s e p a i n . T h i s m a k e s e x c i s i o n of m e t a s t a s e s ment of cutaneous melanoma, Br J Dermatol 146:7-17.
a n d o t h e r t r e a t m e n t of m e t a s t a t i c m e l a n o m a d i s -
e a s e i m p o r t a n t e v e n if it p r o b a b l y d o e s not influ- S m i t h A , S m i t h D J r , Robson M , Beatty I (1993)
ence survival time. Melanoma of the upper limb. In: Boggumill GP,
Fleegler E J , eds. Tumors of the Hand and Upper Limb.
Churchill Livingstone: Edinburgh London Madrid

References Melbourne New York and Tokyo.

A A D (2001) Guidelines of Care for Primary Cutaneous Weedon D (2002) Skin Pathology. Churchill Livingstone:
M e l a n o m a , A m e r i c a n A c a d e m y of Dermatology London, Edinburgh, N e w York, Philadelphia, S t Louis,
Association: Washington. Sydney, Toronto.

Ariyan S (2003) Regional isolated perfusion of extremi- Wolf Y, Balicer RD, Amir A , Feinmesser M (2001) The
ties for melanoma: now 26-year experience with drugs vertical dimension in the surgical treatment of cuta-
other than L-phenylalanine mustard, Plast Reconstr neous malignant melanoma - how deep is deep? Eur J
Surg 111:1257-61. Plast Surg 24:74-7.
 15
Melanomas of the hand:
sentinel node biopsy
Vincent R Hentz

Introduction risk of developing a second tumor

lifetime of a patient w i t h a p r i m a r y
over
melanoma
the

M e l a n o m a of t h e h a n d c o n s t i t u t e s t h e t h i r d m o s t ( O l s o n et al 2000).
c o m m o n m a l i g n a n c y of t h e c u t a n e o u s t i s s u e of
t h e h a n d ( F i g . 1). In a s t u d y of 73 c o n s e c u t i v e
patients presenting with primary malignant Management
t u m o r s of t h e h a n d , 3 p e r c e n t h a d a d i a g n o s i s
of malignant melanoma ( K e n d a l l et al 1969). R e g a r d i n g t h e m a n a g e m e n t of patients w i t h c u t a -
A l t h o u g h , o v e r a l l , t h e i n c r e a s e in t h e i n c i d e n c e of n e o u s m e l a n o m a t h e r e is a c o n s i d e r a b l e a m o u n t
m e l a n o m a a p p e a r s to b e greater than for any of c o n s e n s u s . T h e r e is c o n s e n s u s r e g a r d i n g the
o t h e r h u m a n m a l i g n a n t t u m o r ( O l s o n et al 2000). n e e d for a c a r e f u l history particularly i n q u i r y into
H o w e v e r , s p e c i f i c a l l y w i t h r e g a r d to t h e h a n d , t h e s u n e x p o s u r e , history of e v o l u t i o n of t h e t u m o r ,
i n c i d e n c e of o c c u r r e n c e for m e l a n o m a h a s b e e n a n d a c a r e f u l f a m i l y history. T h e r e is c o n s e n s u s
r e l a t i v e l y s t a b l e d u r i n g t h e p a s t 50 y e a r s ( H o v e regarding examination including the need to
a n d A k s l e n 1999). H o v e a n d A k s l e n p o i n t o u t that characterize t h e lesion a c c o r d i n g to t h e A m e r i c a n
whereas there has been a marked increase C a n c e r S o c i e t y ' s ' A B C D ' a p p r o a c h to pigmented
(15-fold) in t h e i n c i d e n c e o f m e l a n o m a in the l e s i o n s . In t h e U K t h e r e is a similar, s e v e n - p o i n t
upper arm and forearm in m e n a n d a similar checklist (McGovern and Litaker 1992). In the
12-fold i n c r e a s e in w o m e n t h i s h a s not b e e n t h e A m e r i c a n C a n c e r S o c i e t y s y s t e m t h e 'A' s t a n d s for
c a s e w i t h r e g a r d to t h e h a n d a r e a . I n c i d e n c e in a s y m m e t r y indicating t h a t t h e l e s i o n , d i v i d e d into
the h a n d a p p e a r s to h a v e been stable o v e r the h a l v e s , results in a m i s m a t c h of t h e t w o h a l v e s .
p a s t 50 y e a r s . T h e letter ' B ' s t a n d s for border, referring t o a n
Fortunately, probably b e c a u s e of a better irregular b o r d e r w h i c h m a y a p p e a r to b e r a g g e d
m e d i c a l l y e d u c a t e d public, it s e e m s that in m a n y o r b l u r r e d . T h e letter 'C' s t a n d s for c o l o r v a r i a t i o n
countries patients are presenting to physicians r a n g i n g f r o m t a n t h r o u g h to black a n d brown,
w i t h earlier stages of m e l a n o m a . T h i s h a s resulted a n d p e r h a p s e v e n containing a r e a s of red, w h i t e ,
in f a s t e r d i a g n o s i s a n d , at least, a n i n c r e a s e d or b l u e . T h e letter ' D ' s t a n d s for d i a m e t e r of t h e
o p p o r t u n i t y to c u r e this d i s e a s e . Unfortunately, a c q u i r e d n e v u s , t h e d a n g e r o u s size for w h i c h is
e v e n after a p p a r e n t c u r e s , m e l a n o m a h a s r e c u r r e d larger t h a n 6 m m or larger t h a n t h e size of a p e n -
in s o m e c a s e s after 5 - a n d 1 0 - y e a r s of follow-up. cil e r a s e r . T h e r e is c o n s e n s u s r e g a r d i n g subtyping
Therefore, it is n e c e s s a r y that patients with melanomas into s u p e r f i c i a l spreading nodular
melanoma be included in l o n g - t e r m follow-up. and acral lentiginous melanomas (Elder and
T h i s is n e c e s s a r y not o n l y t o d i a g n o s e r e c u r r e n c e s M u r p h y 1999). Clark et al (1969) h a v e e m p h a s i z e d
but a l s o b e c a u s e t h e r e is a n e s t i m a t e d 3 - 6 per c e n t that the growth of neoplasms initially occurs
140 T U M O R S OF THE HAND

a b

Figure 1
There are a variety of presentations of melanomas primary to the hand including a subungual melanoma presenting a black
streaking of the nail (a), or a definitive growth under the nail (b), or as a fungating cutaneous tumor (c).

radially. When the characteristics of growth n e e d not i n c l u d e t h e m u s c l e f a s c i a ; t h i s is t h e

c h a n g e to a m o r e vertical g r o w t h p h a s e t h e n t h e a g r e e d m e t h o d of t r e a t m e n t b y m a n y s u r g e o n s
potential for m e t a s t a s e s m a r k e d l y i n c r e a s e s . t o d a y ( N a r a y a n a n d A r i y a n 2 0 0 0 , W a g n e r et al
T h e r e is a l s o c o n s e n s u s r e g a r d i n g t h e l a b o r a - 2 0 0 0 ) . W a g n e r et al (2000) h a v e p o i n t e d o u t t h a t
t o r y e v a l u a t i o n of s u c h patients i n c l u d i n g a c h e s t t h e g o a l f o r s u r g i c a l t r e a t m e n t is to c u r e t h e l o w -
X - r a y a n d , for m e l a n o m a s of t h e h a n d , a n X - r a y of risk m e l a n o m a p a t i e n t b y r e m o v i n g all m a l i g n a n t
t h e h a n d to u n d e r s t a n d t h e c o n d i t i o n of t h e s k e l e - t u m o r cells f r o m the primary site a n d , in the
t o n b e f o r e potentially altering this a r e a surgically. patient w h o h a s a poor outlook for c u r e , b e c a u s e
T h e various staging s y s t e m s that h a v e been of v a r i o u s t u m o r c h a r a c t e r i s t i c s , a t l e a s t t o c o n -
developed are c o m m o n and allow good c o m m u - trol t h e l o c a l t u m o r .
n i c a t i o n a n d c o m p a r i s o n a n d a s s e s s m e n t of o u t - Until recently there w a s considerable debate
c o m e s . T h e r e is a g r e e m e n t u p o n p r i m a r y t u m o r regarding w h a t w e r e appropriate margins around
staging s y s t e m s with accepted s y s t e m s such as t h e t u m o r o r b i o p s y site. W a g n e r , in citing t h e
those of B r e s l o w (1970), w h i c h measures the N a t i o n a l Institute of H e a l t h C o n s e n s u s C o n f e r e n c e
thickest a r e a of t h e l e s i o n w i t h a n o c u l a r m i c r o m - o n m e l a n o m a of 1992 ( N a t i o n a l Institute of H e a l t h
eter, a n d C l a r k ' s l e v e l s (Clark et al 1969). T h e r e C o n s e n s u s C o n f e r e n c e , 1992), p o i n t e d out that
s e e m s to be an e m p h a s i s o n B r e s l o w ' s m e a s u r e - tumors less than 1 mm by B r e s l o w ' s m e a s u r e -
m e n t s but C l a r k ' s l e v e l s a r e a l s o w o r t h w h i l e a s a n ment s y s t e m w i t h r e g a r d to t h i c k n e s s c a n b e
i n d i c a t o r of t h e b e h a v i o r of t h e t u m o r . t r e a t e d w i t h 1 c m m a r g i n s to t h e m u s c l e f a s c i a .
There is c o n s e n s u s o n the b a s i s of recent O t h e r s t u d i e s h a v e clarified m a r g i n s for thicker
s t u d i e s w h i c h h a v e refined t u m o r thickness m e a - melanomas.The S w e d i s h melanoma study group
s u r e m e n t s , s o that t h e u s e f u l n e s s of d e s i g n a t i o n s c o n c l u d e d t h a t t u m o r s g r e a t e r t h a n 0.8 m m a n d
such as 1 m m , 2 m m , a n d 4 m m has been arrived less t h a n 2 m m in t h i c k n e s s c o u l d b e t r e a t e d b y a
at t h r o u g h statistical a n a l y s i s ( B u t t n e r et al 1995). margin of r e s e c t i o n of 2 cm (Cohn-Cedermark
In a d d i t i o n , s i n c e r e g i o n a l l y m p h n o d e s a r e fre- et al 2000). T o d a y , t h e r e is c o n s e n s u s t h a t thick
q u e n t s i t e s of m e t a s t a t i c i n v o l v e m e n t t h e s e n e e d melanomas (i.e. those greater than 4 mm in
t o b e a s s e s s e d a n d i n c l u d e d in t h e s t a g i n g s y s t e m . d e p t h ) c a n p r o b a b l y b e e x c i s e d w i t h m a r g i n s of
T h e r e is c o n s e n s u s r e g a r d i n g t h e t r e a t m e n t of 2-3 c m (Narayan and Ariyan 2000).These recom-
the primary tumor b y w i d e local i n c i s i o n that mendations are based primarily on data from
i n c l u d e s t h e ' e n b l o c ' e x c i s i o n of t h e t u m o r or melanomas of the trunk and extremities.
b i o p s y site a l o n g w i t h s u b c u t a n e o u s t i s s u e s t h a t Melanomas of the hands and feet are less
 M E L A N O M A S O F T H E HAND: SENTINEL NODE BIOPSY 141

common and less w e l l studied. T h e y pose a enabling a risk-adapted, individual indication for
surgical challenge because primary wound r e g i o n a l l y m p h a t i c d i s s e c t i o n . T h i s c o n c e p t , first
c l o s u r e often is difficult, a n d t h e i n c i d e n c e a n d i n t r o d u c e d in 1977 b y C a b a n a s into t h e t r e a t m e n t
m a n a g e m e n t of regional node metastases are of p e n i s c a r c i n o m a , is b a s e d o n t h e e v i d e n c e
u n c l e a r . In r e g a r d s t o m e l a n o m a of t h e digits, of o r d e r l y and predictable lymphatic drainage
t h e r e still e x i s t s c o n t r o v e r s y r e g a r d i n g s u b u n g u a l pathways. Tumor cell progression within the
m e l a n o m a s w h i c h , in m a n y a s u r g e o n ' s m i n d , l y m p h a t i c s y s t e m s e e m s to f o l l o w a s e q u e n t i a l
remain associated with poor outcomes. pattern and lymph node metastases seem to
o c c u r in a n o r d e r l y manner from the primary
tumor. Primary draining lymph nodes possess
t h e structural a n d f u n c t i o n a l c a p a b i l i t y t o retain
Metastases a n d t o fight t u m o r c e l l s efficiently. T h e s e n t i n e l
n o d e is d e f i n e d a s t h e first t u m o r d r a i n i n g filter,
A l l a g r e e that t h e p r o g n o s i s of m a l i g n a n t d i s e a s e
a n d , if u n i n v o l v e d , s h o u l d t h u s a d e q u a t e l y p r e -
is e s s e n t i a l l y d e t e r m i n e d b y t h e m e t a s t a t i c p o t e n -
d i c t t h e n o d a l s t a t u s of t h e d i s e a s e . M a n y s t u d i e s
tial of t h e p r i m a r y t u m o r . W e n o w p o s s e s s k n o w l -
i n d i c a t e t h a t skip m e t a s t a s e s a r e r a r e , i.e. m e t a s -
e d g e of m a n y biological and molecular tumor
t a s i s to s e c o n d - o r t h i r d - o r d e r l y m p h n o d e s in
markers and mechanisms. However, our knowl-
t h e a b s e n c e of m e t a s t a s i s to t h e s e n t i n e l l y m p h
e d g e of t h e m e c h a n i s m s a n d p a t h w a y s of l y m -
node. T h e ' C a b a n a s approach' w a s found to be
p h a t i c t u m o r s p r e a d is rather l i m i t e d , y e t w e a g r e e
s o m e w h a t u n r e l i a b l e a n d l i m i t e d b y its r e l a t i v e l y
t h a t a d e q u a t e s u r g i c a l c l e a r a n c e of t h e r e g i o n a l
p o o r localization t e c h n i q u e , a n d t h e r e f o r e f a i l e d
l y m p h a t i c s i m p r o v e s t r e a t m e n t results of m a n y
to g a i n w i d e s p r e a d a c c e p t a n c e . It is to t h e c r e d i t
t u m o r s a n d it is u n c o n t e s t e d that a n e s t a b l i s h e d
of C o c h r a n et al (2000) t h a t t h e p r o c e d u r e w a s
d i a g n o s i s of r e g i o n a l l y m p h a t i c s p r e a d is p r o g -
reinstituted in m a l i g n a n t melanoma through a
nostically significant and should influence the
d y e injection t e c h n i q u e at t h e p r i m a r y t u m o r s i t e .
i n d i c a t i o n for a d d i t i o n a l t h e r a p y a n d e v e n t u a l l y
T h i s led t o a r a p i d d e v e l o p m e n t a n d r e f i n e m e n t
for a n i n t e n s i v e f o l l o w - u p . F o r m a n y t u m o r s , t h e
of i n t r a o p e r a t i v e l y m p h a t i c m a p p i n g . O n e m a j o r
i n d i c a t i o n for a d j u v a n t c h e m o t h e r a p y d e p e n d s o n
step in t h i s p r o c e s s w a s to use radiolabeled
t h e n o d a l s t a t u s . O n t h e o t h e r h a n d , it is e q u a l l y
colloids in conjunction with gamma-camera
w e l l k n o w n that a g g r e s s i v e l y m p h a t i c d i s s e c t i o n
i m a g i n g or g a m m a p r o b e - g u i d e d d e t e c t i o n of t h e
increases perioperative morbidity and even
sentinel node.
mortality. L o n g - t e r m s e q u e l a e f r o m r e g i o n a l l y m -
A n u m b e r of a u t h o r s h a v e s h o w n that identifi-
p h a t i c d i s s e c t i o n a r e c o m m o n a n d t h e effect o n
c a t i o n of t h e a p p r o p r i a t e l y m p h b a s i n r e q u i r e s
the local, m a y b e e v e n the systemic, immunologic
t h a t t h e p a t i e n t first u n d e r g o l y m p h o s c i n t i g r a p h y
response to the malignant disease, remains
( F i g . 2) ( J o s e p h et al 1999, Y u et al 1999, C o c h r a n
unclear. To incur s u c h risk s e e m s e s p e c i a l l y p r o b -
et al 2000). T h e c u r r e n t t e c h n i q u e i n v o l v e s p r e -
l e m a t i c in t h o s e p a t i e n t s w i t h o u t a n y l y m p h a t i c
operative lymphoscintigraphy c o n d u c t e d in t h e
s p r e a d a t t h e t i m e of t h e p a t h o l o g i s t ' s w o r k - u p .
nuclear medicine department using a total of
T h u s , t h e r e a r e a r e a s of r e m a i n i n g controversy
10-15 M B q (0.27-0.41 m C i ) of technetium 99m
a n d t h e r e is o n g o i n g d e b a t e a b o u t t h e r a t i o n a l e ,
( 9 9 m T c ) r h e n i u m c o l l o i d or filtered sulfur c o l l o i d
value, extent, a d v a n t a g e , or disadvantage of
injected intradermally a r o u n d the biopsy scar.The
r e g i o n a l s u r g i c a l l y m p h n o d e d i s s e c t i o n or e v e n
o b t a i n e d i m a g e s a r e u s e d to localize all d r a i n i n g
r a d i o t h e r a p y of t h e r e g i o n a l l y m p h a t i c d r a i n a g e
n o d a l b a s i n s a n d t h e l o c a t i o n of t h e s e n t i n e l n o d e
a r e a f o r m a n y different t u m o r s . S h o u l d a n e l e c t i v e
is m a r k e d o n t h e s k i n . A t s u r g e r y , i s o s u l f a n b l u e
lymph node dissection be included a s a primary
d y e is i n j e c t e d i n t r a d e r m a l l y a r o u n d t h e b i o p s y
part of t h e initial t r e a t m e n t ?
s c a r ( F i g . 3). I n t r a o p e r a t i v e l y , a h a n d - h e l d g a m m a
p r o b e is u s e d a s a g u i d e t o t h e first d r a i n i n g n o d e
( F i g . 4 ) . B l u e - s t a i n e d l y m p h a t i c c h a n n e l s a i d in
Sentinel node t h e d i s s e c t i o n . T h i s m a k e s it p o s s i b l e t o i d e n t i f y
the sentinel l y m p h n o d e both b y its relatively
T h e sentinel l y m p h n o d e biopsy technique w a s h i g h r a d i o a c t i v i t y a n d b y t h e p r e s e n c e of b l u e d y e
initially proposed to remedy the dilemma, d e m o n s t r a t i n g t h e afferent l y m p h a t i c a n d l y m p h
 142 T U M O R S OF THE HAND

Figure 3
The appearance after injection of a vital blue dye around the
periphery of a melanoma located on the foot. The dye delin-
eates the primary lymphatic channels and their direction of
Figure 2 drainage.

Lymphoscintigraphy demonstrated in a patient with a sup-

raclavicularly located nodular melanoma. Two sentinel
nodes are demonstrated (smaller areas of uptake) along
with large area of uptake at the primary site.

n o d e ( J o s e p h et al 1999, C o c h r a n et al 2000).
O t h e r w o r k e r s h a v e r e f i n e d t h e i d e n t i f i c a t i o n of
positive lymph nodes by adding immunohisto-
c h e m i c a l s t a i n i n g to t h e n o d e s ( F i g . 5). In t h i s w a y
m e t a s t a t i c d e p o s i t s h a v e b e e n f o u n d in a p p r o x i - Figure 4
m a t e l y 12 p e r c e n t of p a t i e n t s , t h e l y m p h n o d e s of
Gamma probe used intraoperatively to detect nodes with
w h o m w o u l d have otherwise been found by rou-
increased uptake of the radioactive colloid.
t i n e h i s t o l o g y t o b e n e g a t i v e ( Y u et al 1999). O t h e r
methods to avoid underestimating involved
l y m p h n o d e s i n c l u d e t h e u s e of p o l y m e r a s e c h a i n
reaction to detect tyrosinase messenger R N A
( m R N A ) ( W a n g et al 1994). S t a n d a r d immuno-
c h e m i c a l s t a i n i n g is d o n e for S 1 0 0 p r o t e i n s a n d
H M B - 4 5 markers.
If t h e sentinel lymph node is positive for
m e l a n o m a then a therapeutic lymphadenectomy
c a n b e c o m p l e t e d . In a s t u d y r e p o r t e d b y R e i n t g e n
et al (1994) it w a s f o u n d that 19 p e r c e n t of
p a t i e n t s , w h o b y o t h e r criteria w e r e d e e m e d to b e
candidates for elective lymph node dissection,
w h e n e v a l u a t e d for their s e n t i n e l n o d e o r n o d e s
h a d e v i d e n c e of i n v o l v e m e n t of o n l y t h e s e n t i n e l
node. T h e r e w a s additional i n v o l v e m e n t in o n l y
o n e patient. T h i s m a k e s it p o s s i b l e to c a r r y o u t
e x c i s i o n of o n l y a s e n t i n e l n o d e w h e n this is f o u n d
Figure 5
to b e n e g a t i v e for t u m o r . T h i s still m a k e s it p o s s i b l e
to a c h i e v e a n i n c r e a s e in 5 - y e a r s u r v i v a l rate of u p Immunohistochemical staining of a micrometastasis within
t o 27 p e r c e n t a c c o r d i n g t o t h e s e a u t h o r s w h e n the sentinel node.
 M E L A N O M A S OF THE HAND: SENTINEL NODE BIOPSY 143

p a t i e n t s u n d e r g o l y m p h a d e n e c t o m y for clinically encountered melanomas. Various authors have

o c c u l t d i s e a s e c o m p a r e d w i t h t h o s e patients w h o found a decreased survival in t h e subungual
w e r e d e l a y e d in o b t a i n i n g their t h e r a p e u t i c l y m p h m e l a n o m a g r o u p a n d t h e y s u s p e c t d e l a y in d i a g -
n o d e d i s s e c t i o n s until t h e r e w a s a p a l p a b l e t u m o r n o s i s a s w e l l a s t h e a g g r e s s i v e n a t u r e of t h e s e
( B a l c h e t al 1981). It h a s a l s o b e e n p o i n t e d o u t that t u m o r s is to b l a m e . T h i s s u g g e s t s a n e e d t o b e
sentinel node evaluation provides valuable prog- e s p e c i a l l y c o n c e r n e d w i t h p i g m e n t e d nail l e s i o n s
n o s t i c i n f o r m a t i o n . E v e n t h o u g h t h e patient m a y ( Q u i n n et al 1996, O l s o n et al 2000). T r e a t m e n t of
a p p e a r to b e in e i t h e r s t a g e 1 or s t a g e 2 of d i s e a s e s u b u n g u a l m e l a n o m a s h a s b e e n t h e s u b j e c t of
t h e f i n d i n g of a p o s i t i v e n o d e is i n d i c a t i v e of s t a g e m u c h d e b a t e . H o w e v e r , a m p u t a t i o n at t h e t h u m b
3 disease a n d this will d e c r e a s e the 5-year survival i n t e r p h a l a n g e a l joint o r just p r o x i m a l to t h e distal
rate b e t w e e n 25 a n d 50 p e r c e n t ( R e i n t g e n a n d i n t e r p h a l a n g e a l joint for f i n g e r s w i t h c a r e f u l histo-
S h i v e r s 1999). logical e v a l u a t i o n of t h e m a r g i n s f o l l o w e d in a s s o -
In a d d i t i o n to t h e risk f a c t o r s m e n t i o n e d a b o v e , c i a t i o n w i t h s e n t i n e l n o d e e v a l u a t i o n a p p e a r s to
such a s ulceration, high mitotic rate, a n g i o l y m - be appropriate. A positive sentinel n o d e w o u l d be
p h a t i c i n v a s i o n , a n d m i c r o s a t e l l i t e s , it h a s b e e n followed by lymphadenectomy.
d e t e r m i n e d t h a t m e l a n o m a s thicker t h a n 1 m m
e v e n w i t h o u t t h e s e risk f a c t o r s h a v e a 14 p e r c e n t
risk f o r occult m e t a s t a t i c d i s e a s e . It is in this
g r o u p t h a t t h e v a l u e of s e n t i n e l l y m p h nodes Clinical experiences
b i o p s y is e m p h a s i z e d . P a t i e n t s w i t h m e l a n o m a s
thicker t h a n 1 m m , w i t h a g r e a t e r t h a n 25 p e r c e n t T e m p l e e t al (2000) r e p o r t e d o n 56 c o n s e c u t i v e
chance of having metastatic d i s e a s e in their patients with American Joint Committee on
lymph nodes, and who a p p e a r to have an C a n c e r s t a g e 1 b o r 2 m e l a n o m a f r o m all l o c a t i o n s
i m p r o v e d 5 - y e a r s u r v i v a l rate w i t h prophylactic (not restricted to hand tumors) seen at the
lymph n o d e dissection form an excellent group London Health Sciences Centre between July
for sentinel node evaluation (Ali-Salaam and 1998 a n d J a n u a r y 2000. S e n t i n e l n o d e localiza-
A r i y a n 2000). t i o n w a s s u c c e s s f u l in 55 of 56 p a t i e n t s , for a n
In a n u m b e r of s t u d i e s of s e n t i n e l n o d e s f r o m o v e r a l l s u c c e s s rate of 98 p e r c e n t . P r e o p e r a t i v e
v a r i o u s c e n t e r s , it h a s b e e n d e t e r m i n e d t h a t t h e lymphoscintigraphy identified a s e n t i n e l n o d e in
s e n t i n e l n o d e c o u l d b e identified in 95-100 per an unpredictable location in 32 per cent of
c e n t of c a s e s b y g a m m a p r o b e . U s i n g vital d y e s patients. On average, 2.3 sentinel nodes per
a l o n e r e s u l t e d in s e n t i n e l n o d e identification of p a t i e n t w e r e identified o n t h e initial s c a n , a n d
b e t w e e n 53 a n d 87 p e r c e n t . A b e r r a n t l y m p h a t i c 2.2 s e n t i n e l n o d e s p e r p a t i e n t w e r e r e c o v e r e d a t
p a t h w a y s w e r e identified in 10 p e r c e n t . In a s t u d y s u r g e r y . B o t h 9 9 m T c r h e n i u m a n d filtered s u l f u r
b y B o n g e r s et al (1999) of E u r o p e a n c e n t e r s , it colloid showed no substantial differences in
w a s d e t e r m i n e d t h a t 48 p e r c e n t of E u r o p e a n c e n - t r a c e r u p t a k e a n d retention in t h e s e n t i n e l n o d e .
ters w e r e performing sentinel n o d e biopsy. T w e l v e patients had a positive sentinel node o n
S e n t i n e l n o d e b i o p s y c a n d e c r e a s e t h e rate of r o u t i n e h i s t o l o g y , a n d 11 p a t i e n t s subsequently
unnecessary diagnostic lymph node dissections underwent complete lymphadenectomy. Two
a n d the associated morbidity. H o w e v e r , sentinel patients experienced r e c u r r e n c e in a regional
node biopsy requires close collaboration b e t w e e n b a s i n after n e g a t i v e p a t h o l o g i c e v a l u a t i o n of t h e
radiologist, s u r g e o n , a n d pathologist. sentinel node. R e v e r s e transcription-polymerase
Sentinel node biopsy m a y play an important c h a i n r e a c t i o n a n a l y s i s of b o t h of t h e s e p a t i e n t s
role in t h e management of subungual mela- was positive. Two patients were alive with
noma. Subungual melanomas are particular m e t a s t a t i c d i s e a s e a n d 54 p a t i e n t s w e r e a l i v e
p r o b l e m s of m e l a n o m a s o f t h e h a n d b e c a u s e w i t h o u t d i s e a s e , at a m e a n follow-up of 1 y e a r
t h e y s e e m to h a v e a p o o r e r p r o g n o s i s c o m p a r e d ( r a n g e 2 - 2 4 m o n t h s ) . C o m p l i c a t i o n s o c c u r r e d at
with other m e l a n o m a s . T h e patient presenting a substantially higher rate (45 p e r c e n t ) after
with a subungual m e l a n o m a is t y p i c a l l y older c o m p l e t e l y m p h a d e n e c t o m y t h a n after sentinel
t h a n p a t i e n t s h a v i n g m e l a n o m a s in o t h e r a r e a s n o d e b i o p s y a l o n e (9 p e r c e n t ) . T h e s e authors
of t h e h a n d a n d a r m . O n h i s t o l o g i c a l e x a m i n a t i o n c o n c l u d e d t h a t s e n t i n e l n o d e b i o p s y is a f e a s i b l e
t h e s e t u m o r s a r e frequently thicker t h a n other t e c h n i q u e w i t h a h i g h s u c c e s s rate (98 p e r c e n t ) ,
144 T U M O R S OF THE HAND

but t h a t it r e q u i r e s a m u l t i d i s c i p l i n a r y approach. p r i m a r y w i t h a 1-cm m a r g i n . T h i c k e r m e l a n o m a s

This study v a l i d a t e s t h e clinical usefulness of a r e a s s o c i a t e d w i t h a > 50 p e r c e n t rate of r e g i o n a l
9 9 m T c r h e n i u m c o l l o i d for lymphoscintigraphy. o r s y s t e m i c failure. In t h e a b s e n c e of m e t a s t a t i c
L a n d i e t al (2000) r e p o r t e d t h e i r e x p e r i e n c e in disease, these individuals should undergo local
425 A m e r i c a n J o i n t Committee on Cancer stage I excision with a 2-cm margin a n d intraoperative
o r II m e l a n o m a p a t i e n t s e v a l u a t e d b y sentinel lymphatic mapping followed by lymphadenec-
node studies. Their technique employed pre- t o m y if t h e s e n t i n e l n o d e is p o s i t i v e .
operative lymphoscintigraphy by intracutaneous
injection of T c 9 9 m - l a b e l e d a l b u m i n nanocolloids
a r o u n d t h e t u m o r o r e x c i s i o n s c a r of t h e t u m o r
c o m b i n e d w i t h t h e i n t r a o p e r a t i v e u s e of a h a n d -
A logical approach
held g a m m a probe and patent blue V mapping to management
t e c h n i q u e . A w i d e e x c i s i o n of t h e p r i m a r y site w a s
t h e n u n d e r t a k e n in all c a s e s . S e n t i n e l l y m p h n o d e s W a g n e r et al (2000) h a v e s u m m a r i z e d a logical
w e r e e v a l u a t e d w i t h histological a n d i m m u n o h i s - a p p r o a c h t h a t c a n b e a p p l i e d t o m e l a n o m a s of
tochemical examination. Patients with pathologic the hand and upper extremity.This includes exci-
e v i d e n c e of m e t a s t a t i c d i s e a s e in sentinel lymph s i o n of in situ l e s i o n s t h a t a r e l e s s t h a n 1 m m in
n o d e r e t u r n e d for r e g i o n a l l y m p h a d e n e c t o m y . d e p t h a s a w i d e l o c a l e x c i s i o n w i t h 1-cm m a r g i n s .
T h e c o m b i n e d u s e of l y m p h o s c i n t i g r a p h y , b l u e If a d d i t i o n a l risk f a c t o r s s u c h a s u l c e r a t i o n o r h i g h
d y e , a n d g a m m a p r o b e identified o n e or more mitotic index are present then sentinel lymph
sentinel lymph nodes in all but two cases node biopsy should be added depending upon
(99.5 per c e n t s u c c e s s rate). In 70 m e l a n o m a s less judgment. Usually lymphatics should be evalu-
t h a n 0.76 m m thick, s e n t i n e l l y m p h n o d e s w e r e ated by physical examination and followed.
n e g a t i v e for m e t a s t a s e s , w h e r e a s in 380 patients L e s i o n s thicker t h a n 1 m m s h o u l d b e m a n a g e d b y
w i t h thicker t u m o r s m i c r o m e t a s t a s e s w e r e d e m o n - w i d e local excision w i t h 2-cm m a r g i n s for lesions
strated in 75 c a s e s (19.7 per c e n t ) . In patients w i t h u p t o 4 m m in t h i c k n e s s a n d w i t h 2 - 3 - c m m a r g i n s
sentinel l y m p h n o d e m e t a s t a s e s w h o underwent for m e l a n o m a s of greater thickness along w i t h
regional l y m p h n o d e dissection, no other m e t a s - s e n t i n e l n o d e b i o p s i e s . If t h e r e is a p o s i t i v e s e n -
t a s e s w e r e f o u n d t h r e e t i m e s out of four. After a tinel l y m p h n o d e t h e n l y m p h a d e n e c t o m y s h o u l d
m e d i a n f o l l o w - u p p e r i o d of 18 m o n t h s t h e rate of be a d d e d to treatment. W h e n negative sentinel
r e c u r r e n c e of t h e d i s e a s e in 335 patients with nodes are encountered observation should be
sentinel l y m p h n o d e f r e e of m e t a s t a s i s w a s l o w continued. For lesions greater than 4 m m adju-
(5.4 p e r c e n t ) w i t h a v e r y l o w r e g i o n a l n o d a l recur- vant agiment chemotherapy with interferon-a-2B
r e n c e (1.2 per cent). M o r e o v e r , t h e w o r s e n i n g of or other investigational p r o g r a m s should be c o n -
t h e d i s e a s e did not e x c e e d 18.5 per c e n t of c a s e s sidered. A similar approach should be carried out
with m e t a s t a s i s in sentinel lymph node. The for p a t i e n t s w i t h p o s i t i v e l y m p h n o d e s .
authors concluded that sentinel lymph node
b i o p s y is e x t r e m e l y s e l e c t i v e a n d useful to f i n d
early m i c r o m e t a s t a s e s a n d to identify patients
needing regional l y m p h a d e n e c t o m y and adjuvant Current issues and
i m m u n o t h e r a p y . T h e y r e c o m m e n d this in patients future applications
with intermediate thickness of melanoma
(0.76-4.0 m m ) ( L a n d i et al 2000). T h e r e r e m a i n a n u m b e r of q u e s t i o n s . D o e s s e n -
In a s t u d y of patients w i t h m e l a n o m a s of t h e tinel n o d e b i o p s y alter t h e s u r v i v a l r a t e ? Does
h a n d s o r feet t r e a t e d at t h e M a s s a c h u s e t t s G e n e r a l l y m p h n o d e d i s s e c t i o n after a p o s i t i v e sentinel
H o s p i t a l b e t w e e n 1980 a n d 1994, data f r o m 116 n o d e b i o p s y alter s u r v i v a l ? T h i s h a s not y e t b e e n
patients (39 m e n , 77 w o m e n ) w i t h m e l a n o m a s of s h o w n t o b e t h e c a s e . C l e a r l y , t h e rate of u n n e c -
t h e h a n d s ( n = 26) a n d feet ( n = 90) w e r e e v a l u a t e d essary diagnostic lymph node dissections can be
( T s e n g et al 1997). T h e a u t h o r s c o n c l u d e d that considerably reduced as soon as the sentinel
m e l a n o m a s of t h e h a n d s a n d feet < 1.5-mm thick n o d e c o n c e p t is sufficiently v a l i d a t e d f o r g e n e r a l
h a v e a l o w i n c i d e n c e of n o d a l m e t a s t a s e s a n d u s e o u t s i d e c l i n i c a l trials. W h a t r e m a i n s to be
a r e t r e a t e d effectively w i t h w i d e excision of t h e d e t e r m i n e d is w h e t h e r a c a n c e r p a t i e n t w i t h a
 M E L A N O M A S OF THE HAND: SENTINEL NODE BIOPSY 145

positive sentinel node - thus already proven Buttner P, Garbe C, Bertz J et al (1995) Primary cutaneous
l y m p h a t i c m e t a s t a s e s - w o u l d still profit f r o m a melanoma optimized cutoff points of tumor thickness
m o r e o r less e x t e n s i v e l y m p h n o d e d i s s e c t i o n . It and importance of clark's level for prognostic classifica-
tion, Cancer 75:2499-506.
m i g h t b e sufficient t o u s e t h e s t a g i n g i n f o r m a t i o n
o b t a i n e d t h r o u g h t h e s t a t u s of t h e s e n t i n e l n o d e
Clark W J r , From L, Bernardino E et al (1969)The histoge-
alone to decide upon adjuvant therapies. A
nesis and biologic behavior of primary human malignant
further aspect arises from the possibility for
melanomas of the skin, Cancer Res 29:705-26.
investigating this single a n d supposedly most
r e p r e s e n t a t i v e l y m p h n o d e in f a r m o r e d e t a i l t h a n Cochran A, Balda B-R, Starz H et al (2000) The Augsburg
w o u l d b e p o s s i b l e for t h e l a r g e n u m b e r o f n o d e s consensus techniques of lymphatic mapping, sentinel
previously sampled in conventional lymphatic lymphadenectomy, and completion lymphadenectomy in
dissections. This more extensive work-up may cutaneous malignancies, Cancer 89:236-41.
include serial sectioning, immunologic and
molecular techniques to e n h a n c e the sensitivity Cohn-Cedermark G , Rutqvist L E , Andersson R et al
for micrometastases detection. A s Schlag has (2000) Long term results of a randomized study by the
s t a t e d in h i s 1998 c r i t i q u e of t h e t e c h n i q u e ' v e r y S w e d i s h Melanoma Study Group on 2-cm versus
5-cm resection margins for patients with cutaneous
little is k n o w n a b o u t t h e t r u e p r o g n o s t i c signi-
melanoma with a tumor thickness of 0.8-2.0 m m ,
ficance of such conventionally occult micro-
Cancer 89:1495-501.
m e t a s t a s e s , a n d e v e n l e s s e x p e r i e n c e e x i s t s a s to
t h e v a l u e of a d j u v a n t t h e r a p i e s in t h o s e c a s e s .
Elder DE, Murphy G F (1995) M e l a n o c y t i c tumors of the
Thus, while the sentinel node procedure will
skin. In: Rosai J , Sobin L, eds. Atlas ofTumor Pathology.
probably enable a more precise though less Armed Forces Institute of Pathology: Washington DC:
i n v a s i v e l y m p h a t i c staging of m a l i g n a n t d i s e a s e , 1-210.
it r a i s e s a n u m b e r of i m p o r t a n t q u e s t i o n s , a s w e l l .
T h e g e n e r a l p r i n c i p l e s of m u l t i m o d a l treatment Hove L M , Akslen LA (1999) Clinicopathological character-
will h a v e to be redefined w i t h regard to the n e w istics of melanomas of the hand, J Hand Surg 24B:460-4.
diagnostic tool, which will require extensive
prospective a n d randomized testing before a safe J o s e p h E, Brobeil A , Cruse C W et al (1999) Lymphatic
a n d r e l i a b l e a d v a n t a g e for t h e p a t i e n t s m a y b e mapping for melanomas of the upper extremity, J Hand
Surg 24A:675-81.
established.'

Kendall T, Robinson D, Masters F (1969) Primary

malignant tumors of the hand, Plast Reconstr Surg
44:37-40.
References
Landi G , Polverelli M, Moscatelli G et al (2000) Sentinel
Ali-Salaam P, Ariyan S (2000) Lymphatic mapping lymph node biopsy in patients with primary cutaneous
and sentinel lymph node biopsies, Clin Plast Surg melanoma: study of 455 cases, J Eur Acad Dermatol
27:421-9. Venereol 14:35-45.

Balch C M , Soong S J , Murad T M et al (1981) A multi- M c G o v e r n T W , Litaker M S (1992) Clinical predictors of

factorial analysis of melanoma. Ill prognostic factors malignant pigmented lesions, J Dermatol Surg Oncol
in melanoma patients with lymph node metastasis 18:22-6.
(stage II), Ann Surg 193:77-85.
Narayan D, Ariyan S (2000) Surgical management of the
Bongers V, Borel Rinkes I, S i e - G o D et al (1999) primary melanoma, Clin Plast Surg 27:409-19.
Detection of malignant breast tumours in dense
breast tissue: results of 99mTc-tetrofosmin scintimam- National Institute of Health Consensus Conference
mography related to surgery, Eur J Surg Oncol 25: (1992) Diagnosis and treatment of early melanoma,
152-6. J Am Med Assoc 268:1314.

Breslow A (1970) Thickness, cross-sectional areas and Olson J A J r , J a q u e s DP, Coit D G , H w u W - J (2000)
depth of invasion in the prognosis of cutaneous Staging work-up and post-treatment surveillance of
melanoma, Ann Surg 182:572-5. patients with melanoma, Clin Plast Surg 27:377-90.
146 T U M O R S O F T H E HAND

Quirin M J , T h o m p s o n J U , Crotty K et al (1996) Tseng J , Tanabe K, Gadd M et al (1997) Surgical

S u b u n g u a l melanoma of the hand, J Hand Surg management of primary cutaneous melanomas of the
21A:506-11. hands and feet, Ann Surg 225:544-50.

Reintgen D, Cruse C W , Wells K et al (1994) The orderly Wagner J D , Gordon M S , Chuang, T-Y, Coleman III J J
progression of melanoma and nodal metastasis, Ann (2000) Current therapy of cutaneous melanoma, Plast
Surg 220:759-67. Reconstr Surg 105:1774-99.

Reintgen D, S h i v e r s S (1999) Sentinel lymph node W a n g X , Heller R,VanVoorhis N et al (1994) Detection of

micrometastasis from melanoma proven methodology submicroscopic lymph node metastasis with poly-
and evolving significance, Cancer 86:551-2. merase chain reaction in patient's with malignant
melanoma, Ann Surg 220: 768-74.
Schlag P (1998) The 'sentinel node' concept: more
questions raised than answers provided? Oncologist 3: Yu LL, FlotteTJ.Tanabe K et al (1999) Detection of micro-
VI-VII. scopic melanoma metastasis in sentinel lymph nodes,
Cancer 86:617-27.
Temple C, Scilley C, Engel C et al (2000) Sentinel node
biopsy in melanoma using technetium-99m rhenium
colloid: the London experience, Ann Plast Surg 45:
491-9.
 16
Principles of biopsy and surgical
management of soft tissue tumors
Elyazid Mouhsine, Raffaele Garofalo and Pierre F Leyvraz

Introduction or w o u n d n e c r o s i s . T h i s often r e q u i r e s a
c h a n g e in t r e a t m e n t c o u r s e , s u c h a s a m p u t a -
Soft tissue tumors are c o m m o n . Patients usually t i o n or t h e a d d i t i o n of irradiation.
p r e s e n t t o their s u r g e o n w i t h a soft t i s s u e m a s s
of a n extremity. W i t h an annual incidence of In addition, biopsy is a d e m a n d i n g technical
approximately 300 benign soft t i s s u e tumors, p r o c e d u r e , a n d t h e r e a r e m a n y different b i o p s y
compared with only t w o malignant soft t i s s u e pitfalls.
t u m o r s , p e r 100 0 0 0 p e o p l e in a h o s p i t a l popula-
tion, s a r c o m a s are a relatively rare group of
n e o p l a s m s t h a t c o n s t i t u t e a b o u t 0.8-1 p e r c e n t of
all n e w c a n c e r s d i a g n o s e d in t h e U S e a c h y e a r .
Epidemiology
T h e r e is c o n s i d e r a b l e o v e r l a p in t h e clinical
S o f t t i s s u e s a r c o m a s o c c u r at a n y a g e , a l t h o u g h
p r e s e n t a t i o n of b e n i g n a n d m a l i g n a n t soft t i s s u e
t h e m a j o r i t y a r e s e e n in p a t i e n t s o v e r t h e a g e of
t u m o r s . B e c a u s e of t h i s o v e r l a p , c l i n i c i a n s must
40 y e a r s . Half of t h e soft t i s s u e s a r c o m a s o c c u r -
h a v e a s y s t e m a t i c a p p r o a c h t o t h e e v a l u a t i o n of
ring in c h i l d r e n a r e r h a b d o m y o s a r c o m a s . Y o u n g
a n y soft t i s s u e t u m o r in o r d e r t o a v o i d t r e a t m e n t
a d u l t s a r e m o r e likely t o b e d i a g n o s e d w i t h s y n -
e r r o r s ( G l e n c r o s s et al 2003, M u s c u l o et al 2003).
ovial s a r c o m a s and epithelioid s a r c o m a s than
These errors a r e not exceptional and can be
w i t h o t h e r t y p e s of soft t i s s u e s a r c o m a s . T w i c e a s
d i v i d e d into f o u r t y p e s ( F r a s s i c a et al 2000):
m a n y soft t i s s u e s a r c o m a s o c c u r in t h e lower
extremities a s in the upper extremities, with
(1) Delay in diagnosis: e.g. the clinician m a k e s 40 p e r c e n t a n d 20 p e r c e n t of all soft t i s s u e s a r -
the w r o n g m a n a g e m e n t decision while eval- c o m a s o c c u r r i n g in t h e l o w e r a n d u p p e r limbs,
u a t i n g a p a t i e n t w i t h a soft t i s s u e m a s s . T h e r e s p e c t i v e l y . T h i r t y p e r c e n t o f soft t i s s u e s a r c o -
m a s s u s u a l l y g r o w s a n d t h e risk of s y s t e m i c m a s are found in t h e t r u n k a n d p e l v i s , while
metastases increases with time. 10 p e r c e n t o c c u r in t h e h e a d a n d neck region
(2) Incorrect diagnosis: for e x a m p l e , f o l l o w i n g ( D a m r o n et al 2003).
needle or o p e n biopsy.
(3) Incomplete or unplanned excision: e.g. w h e n
the surgeon removes incompletely a malig-
n a n t m a s s , t h e patient t h e n r e q u i r e s a s e c o n d Staging and grading
definitive resection.
(4) Contaminated excision: as seen following S o f t t i s s u e t u m o r s a r e c l a s s i f i e d into 13 different
c o m p l i c a t i o n s s u c h a s infection, hematoma, t y p e s b a s e d o n c e l l u l a r differentiation: f i b r o u s ,
148 T U M O R S O F T H E HAND

fibrohistiocytic, lipomatous, smooth muscle, Table 1 American Joint Committee on Cancer (AJCC) GTNM
skeletal muscle, blood and lymph vessels, classification and stage grouping of soft tissue sarcomas
synovial, mesothelial, neural, paraganglionic, (Flemming et al 1997)
extraskeletal cartilaginous a n d o s s e o u s , pluripo-
G: Histopathologic grade
tential m e s e n c h y m a l , a n d u n c l a s s i f i e d .
G1 well differentiated
Lesions can be broadly defined as benign and G2 moderately differentiated
malignant n e o p l a s m s . T h e f o r m e r a r e unable to G3 poorly differentiated
metastasize a n d a r e non-life threatening. Some G4 undifferentiated
b e n i g n l e s i o n s c a n b e l o c a l l y a g g r e s s i v e or w i t h T: Primary tumor
f r e q u e n t r e c u r r e n c e s s u c h a s g i a n t cell t u m o r s o r T1 < 5 cm greatest diameter
extra-abdominal fibromatosis (desmoid tumors). T2 > 5 cm greatest diameter
In c o n t r a s t , m a l i g n a n t n e o p l a s m s s u c h a s s a r c o - N: Regional lymph nodes
m a s consistently invade normal tissues and have N0 no regional lymph node metastases
N1 verified regional lymph node metastases
t h e p o t e n t i a l to m e t a s t a s i z e ( S i m o n 1998).
M: Distant metastases
S o m e reactive conditions c a n be produced by
MO no distant metastases
a blunt injury to a m u s c l e (e.g. myositis ossifi- M1 known distant metastases
c a n s ) a n d s i m u l a t e a soft t i s s u e s a r c o m a .
S a r c o m a s a r e g r a d e d in a n a t t e m p t to p r e d i c t Stage grouping
their b i o l o g i c a l b e h a v i o r . T h e r e a r e s e v e r a l s y s - Stage IA G1 T1 N0 M0
t e m s that e m p l o y three to four g r a d e s , f r o m 1 to Stage IB G1 T2 NO M0
3/4 w i t h 1 b e i n g t h e l o w e s t g r a d e a n d 3 o r 4 t h e
Stage IIA G2 T1 N0 M0
h i g h e s t . T h e g r a d e is d e t e r m i n e d b y s t u d y i n g t h e
Stage IIB G2 T2 N0 M0
f e a t u r e s of t h e c e l l ' s n u c l e u s a n d t h e m i t o t i c r a t e .
Low-grade l e s i o n s a r e w e l l differentiated with Stage IIIA G3,4 T1 N0 M0
minimal nuclear pleomorphism a n d a t y p i a . In Stage IIIB G3,4 T2 N0 M0
contrast, high-grade lesions have prominent
nuclear p l e o m o r p h i s m a n d nuclear atypia with a Stage IVA Any G AnyT N1 M0
high mitotic rate. A genetic a p p r o a c h will lead to Stage IVB Any G AnyT Any N M1
c o n t i n u e d r e f i n e m e n t s in c l a s s i f i c a t i o n a n d t r e a t -
m e n t of t u m o r s . F r o m a g e n e t i c p e r s p e c t i v e , s a r -
c o m a s c a n b e c l a s s i f i e d in t w o g r o u p s w i t h a
T h e A m e r i c a n J o i n t C o m m i t t e e Task F o r c e o n
different c y t o g e n e t i c l e v e l . O n e g r o u p is i d e n t i -
Bone Tumors adapted and proposed to the
f i e d b y r e l a t i v e l y s i m p l e near-diploid k a r y o t y p e s
International Union Against Cancer (IUCC) the
with f e w c h r o m o s o m e rearrangements.The other
staging system which had been published by
group h a s c o m p l e x karyotypes that a r e character-
E n n e k i n g in 1980 a n d r e v i e w e d a n d p u b l i s h e d in
istic of a s e v e r e d i s t u r b a n c e in g e n o m i c stability
1986 ( E n n e k i n g 1 9 8 6 ) . T h i s s t a g i n g is b a s e d u p o n
( H e l m a n a n d M e l t z e r 2003).
histological g r a d e ( G ) , a n a t o m i c site ( T ) , a n d pres-
T h u s , t h e information elicited f r o m the b i o p s y
e n c e o r a b s e n c e of m e t a s t a s e s ( M ) ( T a b l e 2).
a s w e l l a s t h e e x t e n t of d i s e a s e d e f i n e d b y i m a g -
ing s t u d i e s p e r m i t d e t e r m i n a t i o n of t h e clinical
s t a g e of a soft t i s s u e s a r c o m a . V a r i o u s s t a g i n g
systems have been devised using factors relevant Clinical evaluation
t o t h e p r o g n o s i s of soft t i s s u e s a r c o m a s . S o m e of
the m o r e important factors include tumor grade, Probably the most d a n g e r o u s clinical fallacy
t u m o r size, a n d t h e p r e s e n c e of m e t a s t a s e s . T h e r e g a r d i n g soft t i s s u e m a s s e s is t h a t t h e r e is n o
m o s t c o m m o n l y u s e d s t a g i n g s y s t e m is t h e o n e n e e d f o r c o n c e r n u n l e s s a m a s s is p a i n f u l . O n t h e
defined by the American Joint Committee on c o n t r a r y , m o s t p a t i e n t s w i t h soft t i s s u e s a r c o m a
C a n c e r ( A J C C ) ( A r c a et al 1994).The A J C C s y s t e m present w i t h a painless m a s s , just a s do m a n y
uses tumor grade (G), tumor size ( T ) , a n d t h e w i t h a b e n i g n soft t i s s u e t u m o r . O n l y v e r y o c c a -
p r e s e n c e of n o d a l ( N ) or d i s t a n t m e t a s t a s e s ( M ) s i o n a l l y d o soft t i s s u e s a r c o m a s c a u s e s y m p t o m s
( P i s t e r a n d P o l l o c k 1999) ( T a b l e 1). o t h e r t h a n t h e m e r e p r e s e n c e of a m a s s . W h e n
 BIOPSY AND SURGICAL MANAGEMENT OF S O F T T I S S U E T U M O R S 149

Table 2 Enneking staging adopted by the International Union Against Cancer (IUCC)

Benign tumors Malignant tumors

Stage I Latent Stage I Low grade IA or IB

Stage II Active Stage II High grade IIA or IIB
Stage III Aggressive Stage III I or II with metastases IIIA or IIIB

A<=>Intracompartmental; B <=> Extracompartmental

s y m p t o m s d o o c c u r , t h e y a r e u s u a l l y r e l a t e d to
t h e size of t h e t u m o r , w i t h l a r g e r m a s s e s c a u s i n g
Radiographic evaluation
local discomfort d u e to pressure o n surrounding T h e initial r a d i o g r a p h i c e v a l u a t i o n of a soft t i s s u e
s t r u c t u r e s . O c c a s i o n a l l y , soft t i s s u e m a s s e s m a y mass should be a magnetic resonance imaging
b e a s s o c i a t e d w i t h distally referred neurogenic (MRI). It m u s t be performed every time and
s y m p t o m s of p a i n , p a r e s t h e s i a s , o r f o c a l n e u r o - before any surgery, biopsy, or w i d e biopsy exci-
logic deficits, but t h e s e f i n d i n g s m a y b e c a u s e d s i o n . It is not n e c e s s a r y in t h e p r e s e n c e of b e n i g n
b y b o t h l a r g e soft t i s s u e s a r c o m a s a n d s m a l l e r criteria of t h e m a s s w h i c h w i l l b e o b s e r v e d . It w i l l
benign peripheral nerve-sheath tumors. b e a s s o c i a t e d w i t h plain r a d i o g r a p h s for deep
Another c o m m o n diagnostic fallacy regarding masses and before biopsy. MRI, including
p a t i e n t s w i t h soft t i s s u e s a r c o m a s is t h a t they contrast-enhanced sequences, usually is pre-
appear to be ill, as do patients with non- f e r r e d for e v a l u a t i n g t h e p r i m a r y site in e x t r e m i t y
musculoskeletal disseminated malignant tumors. tumors and lesions of the head and neck.
However, while questions are asked regarding Computed tomography ( C T ) is g e n e r a l l y pre-
f e v e r s , chills, n i g h t s w e a t s , u n i n t e n t i o n a l weight f e r r e d for i d e n t i f y i n g m e t a s t a t i c d i s e a s e ( V a r m a
l o s s , d e c r e a s e in e n e r g y l e v e l a n d a p p e t i t e , l y m - 2000, F e n s t e r m a c h e r 2003).
phadenopathy, cough, hemoptysis, hematuria,
e t c . , p a t i e n t s w i t h a soft t i s s u e s a r c o m a r a r e l y
have a n y of these systemic symptoms. Even
patients w i t h a metastatic lesion rarely h a v e s y s - Biopsy
temic symptoms.
Despite numerous overlapping clinical fea- B i o p s y of a soft t i s s u e m a s s is i n d i c a t e d w h e n
t u r e s b e t w e e n b e n i g n a n d m a l i g n a n t soft t i s s u e clinical and radiographic evaluation does not
m a s s e s , specific physical findings help to deter- y i e l d a c o n c l u s i v e d i a g n o s i s o r w h e n t h e soft tis-
m i n e , to a large extent, the likelihood that a t u m o r sue m a s s must be r e m o v e d . All biopsy principles
is m a l i g n a n t . T u m o r s t h a t a r e e i t h e r > 5 c m or should be c o n s i d e r e d carefully before this crucial
d e e p to the fascia h a v e the highest likelihood of s t e p is u n d e r t a k e n . T w o criteria s h o u l d b e m e t
b e i n g a soft t i s s u e s a r c o m a . C o n v e r s e l y , t h e v a s t b e f o r e p r o c e e d i n g . First, t h e s u r g e o n s h o u l d h a v e
m a j o r i t y of soft t i s s u e m a s s e s t h a t a r e s u p e r f i c i a l e x p e r i e n c e in d e a l i n g w i t h all of t h e possible
and < 5 c m are benign. d i a g n o s e s c o n s i d e r e d in t h e p r e - b i o p s y d i f f e r e n -
W h e n a h i s t o r y is o b t a i n e d f r o m a patient w i t h tial d i a g n o s i s . S e c o n d , t h e pathologist should
soft t i s s u e m a s s , all t h e s a l i e n t f e a t u r e s d e s c r i b e d h a v e e x p e r i e n c e in m u s c u l o s k e l e t a l p a t h o l o g y . If
a b o v e s h o u l d b e n o t e d . In particular, t h e t i m i n g , e i t h e r of t h e s e criteria is not met, the patient
g r o w t h , a n d n a t u r e of d i s c o v e r y of t h e soft t i s s u e s h o u l d b e referred t o a c e n t e r w h e r e p a t i e n t s w i t h
m a s s are important. A n y conditions k n o w n to be musculoskeletal s a r c o m a s are routinely diag-
a s s o c i a t e d w i t h t h e d e v e l o p m e n t of soft t i s s u e nosed a n d treated, early, before inappropriate
sarcomas must be noted such a s , type I neuro- manipulation of the tumor and surrounding
fibromatosis, Gardner s y n d r o m e , multiple lipo- tissues has been performed. W i t h a multidiscipli-
mas, and history of local irradiation, or with nary group (oncologist, radiologist, pathologist,
g e n e t i c a b n o r m a l i t i e s ( H e l m a n a n d M e l t z e r 2003). and surgeon) the patient will have the best
150 T U M O R S OF THE HAND

c h a n c e of r e c e i v i n g l i m b - s p a r i n g s u r g e r y , local t u m o r s v a r i e s f r o m 64 p e r c e n t to 96 p e r c e n t .
c o n t r o l of t h e t u m o r , a n d p o s s i b l y a c u r e . T h i s l o w a c c u r a c y l i m i t s t h e v a l u e of f i n e -
Errors related to the biopsy of soft tissue needle aspiration and very f e w centers use
m a s s e s c o n t i n u e to o c c u r . T h e m o s t common this technique.
e r r o r t a k e s p l a c e w h e n t h e l e s i o n is a s s u m e d t o (2) Core needle biopsy, using a Tru-cut®, trocar,
b e b e n i g n a n d is e i t h e r r e m o v e d i n a p p r o p r i a t e l y or b i o p s y g u n , to obtain five longitudinal
or ignored without histological examination a n d c o r e of tissue s a m p l e s of 3 - 6 m m diameter
without follow-up. Some masses are excised w i t h i n t h e m a s s , is a n e x c e l l e n t t e c h n i q u e ,
without adequate preoperative imaging, because w h i c h is c o s t e f f e c t i v e a n d s a f e . It c a n b e
of t h e e x p e n s e of t h e t e s t s a n d t h e r a t i o n a l e t h a t performed w i t h local or regional a n e s t h e s i a ,
the t u m o r needs to be r e m o v e d a n y w a y , without under CT scan control a n d patients are a m b u -
a n y r e g a r d for t h e s p e c i f i c d i a g n o s i s o r h o w t h a t latory. T h e d i a g n o s t i c a c c u r a c y f o r t h e soft tis-
m i g h t affect t h e u l t i m a t e s u r g i c a l r e s e c t i o n if t h e sue tumors is 78 p e r c e n t . T h e p r e v i o u s l y
l e s i o n is m a l i g n a n t . m e n t i o n e d conditions m u s t be respected for
Transverse incisions are inappropriately used s u c c e s s of t h e t e c h n i q u e .
b e c a u s e of t h e i r often s u p e r i o r c o s m e t i c result. If (3) Incisional biopsy is a t e c h n i c a l l y d e m a n d i n g
a m o r e e x t e n s i v e r e s e c t i o n is r e q u i r e d , a c o m p l e x p r o c e d u r e to r e m o v e part of t h e t u m o r . T h e
excision a n d reconstruction is often necessary smallest incision should b e u s e d that will
b e c a u s e t h e b i o p s y i n c i s i o n a n d all t i s s u e p r e v i - allow adequate tissue to be obtained with the
o u s l y e x p o s e d m u s t b e e x c i s e d at t h e t i m e of t h e least risk of c o m p l i c a t i o n s . C o m p l i c a t i o n s that
definitive surgery. must be avoided include h e m a t o m a s , large
B i o p s y s h o u l d b e d o n e e x p e d i t i o u s l y but not e c c h y m o s e s , infections, d e l a y e d w o u n d heal-
until all i n d i c a t e d i m a g i n g s t u d i e s h a v e b e e n p e r - ing, and w o u n d dehiscence. Even with the
formed. T h e case must be r e v i e w e d with the radi- m o s t c a r e f u l t e c h n i q u e , t h e risk o f c o m p l i c a -
ologist and pathologist; and if necessary, an t i o n is a b o u t 5 p e r c e n t . T h e b i o p s y i n c i s i o n
orthopedic oncologist should be consulted. should be longitudinal a n d short (less than
The m o s t c o m m o n t y p e s of b i o p s i e s utilized 5 c m ) ( F i g . 1). T h e o r i e n t a t i o n should allow
for soft t i s s u e m a s s e s a r e : t h e s u r g e o n to excise t h e entire biopsy tract
at the time of definitive r e s e c t i o n of the
(1) Fine-needle aspiration, p e r f o r m e d in c o n j u n c - tumor. A useful technique is to draw the
t i o n w i t h C T s c a n for e n h a n c e d v i s u a l i z a t i o n , is definitive i n c i s i o n first a n d t h e n place the
typically performed by a n interventional radi- b i o p s y i n c i s i o n in t h e line of t h i s i n c i s i o n o r
ologist a s s i s t e d b y a n o r t h o p e d i c specialist offset b y a s h o r t d i s t a n c e ( 1 - 3 c m ) . T h e s k i n is
identifying the a p p r o a c h for later surgery incised and dissection is carried directly
(Garcia-Solano et al 2000). A 23-gauge d o w n to the tumor without raising flaps.
(0.6 m m ) n e e d l e a t t a c h e d t o a 20-ml d i s p o s - The tumor p s e u d o c a p s u l e is i n c i s e d , a n d
a b l e s y r i n g e u n d e r n e g a t i v e p r e s s u r e is u s e d . s e v e r a l p i e c e s of t h e t u m o r a r e r e m o v e d . T h e
This technique h a s t h e a d v a n t a g e of m i n i m a l p s e u d o c a p s u l e of t h e t u m o r is not e x c i s e d a s
morbidity for the patient, being performed a s it c a n b e c l o s e d t o help reduce bleeding
a n o u t p a t i e n t p r o c e d u r e a n d that it is m o r e and tumor c o n t a m i n a t i o n . T h e a c c u r a c y of
c o s t e f f e c t i v e . Its d i s a d v a n t a g e is t h e r e l a t i v e t h i s t e c h n i q u e is 98 p e r c e n t , b u t w i t h signifi-
paucity of procured tissue, which makes c a n t c o s t a n d h i g h e r c o m p l i c a t i o n s a n d a lot
pathologic interpretation challenging. T h e of c a r e .
pathologist should receive the tissue unfixed, (4) Excisional biopsy entails removal of the
a c c o m p a n i e d with s o m e radiologic illustra- entire m a s s . T h i s m e t h o d should be r e s e r v e d
tions. A negative or non-diagnostic finding f o r m a s s e s of < 3 c m , o r f o r l a r g e r l e s i o n s
o n f i n e - n e e d l e b i o p s y c a n n o t b e r e l i e d o n to (< 5 c m ) t h a t a r e s u p e r f i c i a l to t h e d e e p f a s c i a
e x c l u d e t h e d i a g n o s i s of m a l i g n a n t t u m o r . It and that c a n be r e m o v e d with m i n i m a l dis-
o n l y i m p l i e s t h a t a d d i t i o n a l t i s s u e h a s to b e s e c t i o n . T h e t u m o r is r e m o v e d in w i d e t i s s u e
obtained with another multiple needle aspi- a n d t h e p s e u d o c a p s u l e is not e x p o s e d . T h e
ration or w i t h a n o t h e r t e c h n i q u e . T h e a c c u - specimen should be obtained without crush-
r a c y of f i n e - n e e d l e a s p i r a t i o n for soft t i s s u e ing o r c a u t e r i z a t i o n .
 BIOPSY AND SURGICAL MANAGEMENT OF S O F T T I S S U E T U M O R S 151

Figure 1

Incisional or excisional biopsy.

(a) Inappropriate transverse incision
of hypothenar compartment approach.
(b) Correct orientation of the incision
which is longitudinal and in the
localization of the future surgical
approach.

During incisional or excisional biopsy a tourniquet

is u s e f u l to m i n i m i z e b l e e d i n g d u r i n g s u r g e r y . T h e
l i m b is e x s a n g u i n a t e d b y g r a v i t y a l o n e a n d not
with a n Ace® w r a p or Esmarch® bandage. T h e
t o u r n i q u e t is d e f l a t e d a n d m e t i c u l o u s h e m o s t a s i s
is a c h i e v e d . A d r a i n c a n b e u s e d if n e c e s s a r y . If
u s e d , t h e d r a i n s h o u l d exit t h r o u g h t h e s k i n , in
line w i t h the incision a n d separated from the
end by a m a x i m u m 5 m m . A compression dressing
is a p p l i e d a n d c h a n g e d after 48 h o u r s . T h e s p e c i -
men should be delivered immediately to the
pathologist.

Surgical management
S u r g i c a l t r e a t m e n t of soft t i s s u e t u m o r s is b a s e d
o n their size ( < o r > 5 c m ) , l o c a t i o n ( d e e p t o , o r
i n v o l v i n g t h e f a s c i a , intra- o r e x t r a c o m p a r t m e n -
tal), h i s t o l o g i c a l d i a g n o s i s ( b e n i g n o r m a l i g n a n t ) ,
its s t a g i n g ( w i t h o r w i t h o u t m e t a s t a s e s ) , a n d s u r -
v i v a l p r o g n o s i s ( D a m r o n e t al 2 0 0 3 , M u s c u l o et al Figure 2
2 0 0 3 ) . T i m e of s u r g e r y is d e t e r m i n e d b y a m u l t i -
disciplinary approach. T h e r e are four categories Surgical procedure for a (1) hypothenar compartment
of t u m o r e x c i s i o n ( G e r r a n d et al 2 0 0 1 , L i n e t al tumor: (2) intralesional, (3) marginal, (4) wide, and (5) radical
2 0 0 2 , G e r r a n d et al 2003) ( F i g . 2): excision (excision of the compartment; finger or hand
amputation).

(1) Intralesional excision is d e f i n e d a s t h e p i e c e -

m e a l r e m o v a l of t h e t u m o r . T h i s t e c h n i q u e
can be appropriate for synovial chondro-
matosis, pigmented villonodular synovitis, (3) Wide resection is d e f i n e d a s r e m o v a l of t h e
synovial cysts, and synovitis. tumor with a cuff of n o r m a l t i s s u e in all
(2) Marginal excision is d e f i n e d a s c o m p l e t e planes without exposing the pseudocapsule.
r e m o v a l of t h e t u m o r w i t h t h e p s e u d o c a p s u l e It is t h e o p t i m a l s u r g i c a l p r o c e d u r e for m o s t
left intact. It is a p p r o p r i a t e for n o n - i n f i l t r a t i v e soft t i s s u e s a r c o m a s a n d d e s m o i d t u m o r s .
b e n i g n soft t i s s u e t u m o r s u c h a s l i p o m a s , (4) Radical resection involves removal of
except for d e s m o i d t u m o r s w h i c h a r e aggres- the entire muscle compartment, or com-
sive benign t u m o r s requiring w i d e excision p a r t m e n t s i n v o l v e d b y t h e t u m o r or a m p u t a -
to avoid recurrence. tion. This technique is used for some
152 T U M O R S OF THE HAND

Figure 3

Algorithm for evaluation of a soft tissue tumor. MRI, magnetic resonance imaging.
 BIOPSY AND SURGICAL MANAGEMENT OF S O F T T I S S U E T U M O R S 153

intracompartmental soft tissue sarcomas Flemming ID, Cooper J S , Henson DE et al (1997) Soft
w h e n a w i d e resection w o u l d be associated tissue sarcoma. In: Flemming ID, ed. American Joint
with as much morbidity as a compartmental Committee on Cancer (AJCC) Staging Manual, 5th edn.
resection, if there have been local recur- Lippincott: Philadelphia: 149-56.

rences, or when the sarcoma has been

Frassica F J , McCarty EF, Bluemke DA (2000) Soft-tissue
n e g l e c t e d . T h e a m p u t a t i o n is often required
masses: w h e n and how to biopsy, lnstr Course Lect
w h e n sarcomas are extracompartmental.
49:437-42.

T h e s p e c i m e n , once r e m o v e d , must be submitted Garcia-Solano J , Garcia-Rojo B , Sanchez-Sanchez C

to the pathologist fresh, entire, a n d w i t h marks. et al (2000) On the utility and limitations of fine-needle
T h e rest of t h e p r o c e d u r e is a s d e s c r i b e d b e f o r e . aspiration of palpable lesions located in the hand, Diagn
Cytopathol 23:284-91.

Gerrand C H , W u n d e r J S , Kandel R A et al (2001)

Conclusion Classification of positive margins after resection of soft-
tissue sarcoma of the limb predicts the risk of local
E v a l u a t i o n a n d t r e a t m e n t of a soft tissue tumor recurrence, J Bone Joint Surg Br 83:1149-55.
a b s o l u t e l y n e c e s s i t a t e a multidisciplinary a p p r o a c h
in w h i c h all t h e p a r t n e r s ( s u r g e o n s , radiologists, Gerrand C H , Bell R S , Wunder J S et al (2003) The influ-
o n c o l o g i s t s , pathologists, and radio-oncologists) ence of anatomic location on outcome in patients with
a r e i n v o l v e d not o n l y in t h e definitive treatment soft tissue sarcoma of the extremity, Cancer 97:485-92.
d e c i s i o n s , but a l s o f r o m t h e b e g i n n i n g of t h e e v a l -
uation p r o c e s s . In t h e a b s e n c e of s u c h a n experi- Glencross J , Balasubramanian SP, Bacon J et al (2003)
An audit of the management of soft tissue sarcoma
e n c e d g r o u p , patients must b e referred before
within a health region in the U K , Eur J Surg Oncol
a n y investigation (particularly b i o p s y ) to a center
29:670-5.
a b l e to treat t h e m c o m p l e t e l y . T h i s a p p r o a c h h a s
b e e n s h o w n to i m p r o v e d r a m a t i c a l l y t h e results in
Helman L J , Meitzer P (2003) Mechanisms of sarcoma
diminishing the morbidity and mortality rates development, Nat Rev Cancer 3:685-94.
(Frassica et al 2000, G l e n c r o s s et al 2003, M u s c u l o
et a l 2003). S u c h a s a r c o m a g r o u p h a s existed in Lin PP, Guzel V B , Pisters P W T et al (2002) Surgical man-
o u r institution s i n c e 1997. F i g u r e 3 s u m m a r i z e s t h e agement of soft tissue sarcomas of the hand and foot,
a l g o r i t h m w e u s e to e v a l u a t e a n d treat soft tissue Cancer 95:852-61.
tumors.
Musculo DL, Ayerza M A , Makino A M (2003) Tumors
about the knee misdiagnosed as athletic injuries,
J Bone Joint Surg 85A: 1209-14.
References
Pister PWT, Pollock RE (1999) Staging and prognostic
Arca M J , S o n d a k V K , Chang A E (1994) Diagnostic proce- factors in soft tissue sarcoma, Semin Radiat Oncol
dures and pretreatment evaluation of soft-tissue sarco- 9:307-14.
mas, Semin Surg Oncol 10:323-31.
Simon M A , Springfield D (1998) Surgery for Bone and
Damron TA, Beauchamp CP, Rougraff BT, W a r d W G Soft-tissue Tumors. Lippincott-Raven: Philadelphia,
(2003) Soft-tissue lumps and bumps, J Bone Joint Surg New York: 55-65.
85A:1142-55.
Varma DGK (2000) Imaging of soft-tissue sarcomas,
Enneking W F (1986) A system of staging musculoskele- Curr Oncol Rep 2:487-90.
tal neoplasms, Clin Orthop 204:9-24.

Fenstermacher M J (2003) Imaging evaluation of

patients with soft tissue sarcoma, Surg Oncol Clin North
Am 12:305-32.
17
Malignant soft tissue tumors of
the hand: histological diagnosis
and classification
Louis Guillou

Introduction t h e lesion t o e s t i m a t e its d e g r e e of a g g r e s s i v e n e s s

(potential for local a n d distant r e c u r r e n c e ) , w h i c h
S o f t t i s s u e s a r c o m a s a r e r a r e , a c c o u n t i n g for is a p r e r e q u i s i t e for any valuable therapeutic
a b o u t 1 p e r c e n t of t u m o r s o c c u r r i n g in a d u l t s . s c h e m e . During this diagnostic process, clinical,
M o r e t h a n 7 5 different h i s t o l o g i c a l t y p e s a n d s u b - radiologic, a n d peroperative findings should be
types have been described. These can develop kept in m i n d . For i n s t a n c e , soft t i s s u e l e s i o n s in
a n y w h e r e in t h e b o d y , i n c l u d i n g t h e h a n d s . In this c h i l d r e n a r e different f r o m t h o s e f o u n d in a d u l t s .
l o c a t i o n , s o m e , s u c h a s e p i t h e l i o i d s a r c o m a or Radiograms (X-rays and magnetic resonance
c l e a r cell s a r c o m a , a r e m o r e f r e q u e n t t h a n o t h e r s . i m a g i n g ( M R I ) ) a r e c r u c i a l in differentiating soft
In t h e f o l l o w i n g s e c t i o n s w e w i l l f o c u s p r i m a r i l y t i s s u e f r o m b o n e a n d p e r i o s t e a l l e s i o n s . Size a n d
o n soft t i s s u e m a l i g n a n c i e s t h a t a r e p r e d o m i - d e p t h of t h e l e s i o n a r e a l s o i m p o r t a n t f a c t o r s , a s
n a n t l y o b s e r v e d in t h e h a n d , i n c l u d i n g s o m e a r e a n y specific p e r o p e r a t i v e f i n d i n g s . A n i n f o r m a l
n e w l y d e s c r i b e d entities. d i s c u s s i o n w i t h t h e s u r g e o n often h e l p s t o a s s e s s
t h e local a g g r e s s i v e n e s s of t h e lesion ( a d h e s i o n s ,
infiltrating b o r d e r s , p e r o p e r a t i v e r u p t u r e of t h e
c a p s u l e , etc.) a n d t h e a d e q u a c y of s u r g e r y .
Soft tissue sarcomas:
approach to diagnosis
Histological classification
B o t h t h e h i s t o l o g i c a l d i a g n o s i s a n d e v a l u a t i o n of
t h e p o t e n t i a l b e h a v i o r of soft t i s s u e t u m o r s c a n
of malignant mesenchymal
be problematic for pathologists. According to tumors of the hand
s o m e a u t h o r s ( A r b i s e r et a l 2001), b e t w e e n 10 p e r
c e n t a n d 25 p e r c e n t o f d i a g n o s e s a r e i n a d e q u a t e . Different criteria c a n b e u s e d t o classify soft t i s s u e
In t h e p r e s e n c e of an apparently malignant t u m o r s : clinical d a t a ( e . g . c h i l d h o o d v e r s u s a d u l t
l e s i o n , it s h o u l d first b e c o n f i r m e d t h a t it r e p r e - tumors), morphology (e.g. spindle cell v e r s u s
sents a true s a r c o m a . All sarcomas mimic r o u n d cell v e r s u s e p i t h e l i o i d v e r s u s p l e o m o r p h i c
other t u m o r s including carcinomas (primary or tumors), histological type (synovial sarcoma,
metastatic), m e l a n o m a s , l y m p h o m a s , or neuro- rhabdomyosarcoma, etc.), depth (superficial
b l a s t o m a s in c h i l d r e n , a n d t h e s e s h o u l d b e c a r e - v e r s u s d e e p l e s i o n s ) , a n d d e g r e e of m a l i g n a n c y
f u l l y r u l e d o u t . O n c e t h e d i a g n o s i s of s a r c o m a is (low-grade versus intermediate-grade versus
m a d e , the pathologist should classify a n d grade high-grade t u m o r s ) . Ideally, classifications should
156 T U M O R S OF THE HAND

b e e a s y t o u s e , p r a c t i c a l , a n d m e a n i n g f u l for t h e distal e x t r e m i t i e s in a b o u t o n e - q u a r t e r of c a s e s , is
c l i n i c i a n . I n T a b l e 1 , m a l i g n a n t soft t i s s u e t u m o r s also considered.
of t h e h a n d h a v e b e e n c l a s s i f i e d a c c o r d i n g to
p a t i e n t a g e a n d d e g r e e of a g g r e s s i v e n e s s .

Synovial sarcoma
Epidemiology of soft tissue S y n o v i a l s a r c o m a ( S S ) is t h e m o s t f r e q u e n t soft

sarcomas of the hand t i s s u e s a r c o m a of t h e h a n d , a c c o u n t i n g for 2 0 - 3 0

p e r c e n t of soft t i s s u e s a r c o m a s f o u n d in this
l o c a t i o n (data f r o m t h e F r e n c h S a r c o m a Group
S o f t t i s s u e s a r c o m a s of t h e distal e x t r e m i t i e s a r e
database). It t e n d s t o occur in young adults
r a r e ( L i n et al 2002). In a r e v i e w of 1945 c o n s e c u -
( m e d i a n a g e 28 y e a r s ) , but r a r e e x a m p l e s h a v e
tive soft tissue s a r c o m a s occurring in adult
a l s o b e e n o b s e r v e d in p a t i e n t s > 60 y e a r s ( F i s h e r
p a t i e n t s r e c o r d e d in t h e F r e n c h S a r c o m a G r o u p
1998, W e i s s and Goldblum 2001). A palpable
database from 1980 t o 2000, 36 (32 p e r c e n t )
m a s s , p a i n o r t e n d e r n e s s , p a r e s t h e s i a , a n d limita-
o c c u r r e d in t h e distal u p p e r e x t r e m i t i e s (four in
t i o n of m o v e m e n t a r e t h e m o s t f r e q u e n t p r e s e n t -
wrist, 32 in h a n d s , including fingers) and 76
ing s y m p t o m s . M a l e s a r e a f f e c t e d more often
(68 p e r c e n t ) in t h e distal l o w e r e x t r e m i t i e s (23 in
t h a n f e m a l e s ( m a l e t o f e m a l e ratio of 1.5 : 1 ) . N o
a n k l e , 5 3 in f o o t , i n c l u d i n g toes) (unpublished
etiologic factors h a v e been definitively associated
data f r o m t h e French S a r c o m a Group). W h e n
with S S development, although cases occurring
looking s p e c i f i c a l l y a t t h o s e p a t i e n t s w i t h a t u m o r
after t r a u m a , irradiation ( E g g e r e t al 2002), o r h i p
of t h e distal u p p e r e x t r e m i t y , m e d i a n a g e , m e d i a n
replacement (Weiss and Goldblum 2001) have
tumor size, a n d m a l e to f e m a l e ratio w e r e 32
b e e n r e p o r t e d . T h e d i a g n o s i s of S S m a y b e s u s -
y e a r s ( r a n g e 1 5 - 9 1 y e a r s ) , 3 c m ( r a n g e 1-11 c m ) ,
p e c t e d p r e o p e r a t i v e l y o w i n g t o t h e p r e s e n c e of
a n d 1 : 1.6, r e s p e c t i v e l y . T h e i n c i d e n c e of s p e c i f i c
c a l c i f i c a t i o n s o n X - r a y s . In 15-20 p e r c e n t of c a s e s
h i s t o l o g i c a l t y p e s of t u m o r s w a s a s f o l l o w s : s y n -
t h e r e is i n v o l v e m e n t of t h e u n d e r l y i n g b o n e in t h e
ovial sarcoma ( n = 10), epithelioid sarcoma
f o r m of p e r i o s t e a l r e a c t i o n , s u p e r f i c i a l e r o s i o n , o r
(n = 7 ) , r h a b d o m y o s a r c o m a ( n = 5 , f o u r a l v e o l a r
b o n e i n v a s i o n . G r o s s e x a m i n a t i o n often r e v e a l s
a n d o n e e m b r y o n a l ) , c l e a r cell s a r c o m a s (n = 4 ) ,
a well-delineated but non-encapsulated mass
malignant fibrous histiocytomas (n = 3), unclassi-
a t t a c h e d to s u r r o u n d i n g t e n d o n s h e a t h s o r j o i n t
fied s p i n d l e cell s a r c o m a s (n = 2), K a p o s i ' s s a r c o m a
c a p s u l e . C y s t i c c h a n g e s m a y b e p r e s e n t . S S of
( n = 2), a n d o n e c a s e e a c h of leiomyosarcoma,
h a n d s a n d f i n g e r s a r e u s u a l l y s m a l l , 2.5 c m o n
malignant peripheral nerve sheath tumor, and
a v e r a g e ( r a n g e 1-7 c m ) b a s e d o n t h e t e n c a s e s
extraskeletal c h o n d r o s a r c o m a .
r e c o r d e d in t h e F r e n c h S a r c o m a G r o u p d a t a b a s e .
Several histological v a r i a n t s of S S h a v e been
d e s c r i b e d , i n c l u d i n g b i p h a s i c ( F i g . 1), m o n o p h a -

Description of common and s i c s p i n d l e c e l l ( F i g . 2), p o o r l y

cell, myxoid, calcifying, a n d bone-forming
differentiated/round
forms
less common soft tissue (Fisher 1998, W e i s s and Goldblum 2001). In
malignancies of the hand hands, the monophasic spindle cell f o r m pre-
dominates (80 per cent of cases). Immuno-
In t h i s s e c t i o n , t h e c l i n i c o p a t h o l o g i c f e a t u r e s of h i s t o c h e m i s t r y a n d m o l e c u l a r b i o l o g y a r e of g r e a t
three s a r c o m a s (synovial s a r c o m a , epithelioid h e l p in d i a g n o s i n g S S . Most tumors, at least
s a r c o m a , a n d clear cell s a r c o m a ) a n d t w o focally, display immunoreactivity, for epithelial
u n u s u a l m e s e n c h y m a l t u m o r s of i n t e r m e d i a t e membrane antigen ( E M A ) and cytokeratins
malignancy (inflammatory myxohyaline tumor ( F i s h e r 1998, W e i s s a n d G o l d b l u m 2 0 0 1 , P e l m u s
a n d plexiform fibrohistiocytic tumor) a r e pre- et al 2002); t h e y a r e a l m o s t a l w a y s n e g a t i v e f o r
s e n t e d . A l l of t h e m o c c u r c o m m o n l y in a d u l t s , C D 3 4 a n d d e s m i n ( P e l m u s et al 2002). C D 9 9 ( t h e
except plexiform fibrohistiocytic t u m o r which p r o d u c t o f t h e M I C - 2 g e n e ) is e x p r e s s e d in u p to
u s u a l l y d e v e l o p s in c h i l d r e n o r a d o l e s c e n t s . 7 5 p e r c e n t of c a s e s , e s p e c i a l l y in t h e p o o r l y dif-
M y o e p i t h e l i o m a / m i x e d t u m o r of soft t i s s u e , a f e r e n t i a t e d v a r i a n t ( D e i T o s et al 1995, P e l m u s e t al
r e c e n t l y r e c o g n i z e d e n t i t y w h i c h d e v e l o p s in 2002), a n d S 1 0 0 p r o t e i n in a b o u t 30 p e r c e n t of
 MALIGNANT S O F T T I S S U E T U M O R S O F T H E HAND 157

Table 1 Classification of soft tissue tumors of the hand according to behavior

Benign, locally non-recurring (self-limited)

Fibro-osseous pseudotumor of digits (florid reactive periostitis)
Desmoplastic fibroblastoma (collagenous fibroma)
Schwannoma/neurofibroma
Traumatic neuroma
Neurothekeoma/nerve sheath myxoma
Acral fibromyxoma
Perineurioma and variants (sclerosing perineurioma)
Venous ectasia with thrombosis and/or papillary endothelial hyperplasia
Glomus tumor/glomangioma/glomangiopericytoma
Lipoma and variants (angiolipoma, myxolipoma, etc.)
Superficial hemangioma/lymphangioma*
Leiomyoma/angioleimyoma
Synovial chondromatosis
Gout/pseudogout (calcium pyrophosphate dihydrate deposition)
Lobular capillary hemangioma (pyogenic granuloma)*

Benign, locally recurring

Knuckle pad
Fibroma of tendon sheath (15-25 per cent recurrence rate)
Superficial palmar fibromatosis (Dupuytren's contracture) (10-20 per cent)
Localized giant cell tumor of tendon sheath (nodular tenosynovitis) (10-20 per cent)
Diffuse giant cell tumor of tendon sheath (pigmented villonodular synovitis) (30 per cent)
Ganglion cyst and variants (digital mucous cysts) (10-20 per cent)
Cutaneous fibrous histiocytoma, conventional type (5 per cent)
Cellular/atypical cutaneous fibrous histiocytoma (20-30 per cent)
Deep fibrous histiocytoma (20-30 per cent)
Intramuscular hemangioma (up to 30 per cent)
Angiomatosis (up to 90 per cent)
Infantile digital fibromatosis (inclusion body fibromatosis)* (60 per cent)
Soft tissue chondroma (10-20 per cent)
Bizarre parosteal osteochondromatous proliferation (Nora's lesion) (50 per cent)
Calcifying aponeurotic fibroma* (50 per cent)
Lipomatous hamartoma of nerves*
Spindle cell hemangioma* (60 per cent)
Giant cell fibroblastoma (infantile form of dermatofibrosarcoma protuberans)* (50 per cent)
Lipofibromatosis* (50-70 per cent)

Malignant, do recur but rarely (< 5 per cent) metastasize

Hemangioendotheliomas (retiform, kaposiform, composite, and polymorphous variants)*
Plexiform fibrohistiocytic tumor*
Inflammatory myxohyaline tumor/myxoinflammatory fibroblastic sarcoma of extremities
Kaposi's sarcoma
Congenital (infantile) fibrosarcoma*

Malignant, do recur and/or frequently (> 5 per cent) metastasize

Synovial sarcoma
Epithelioid sarcoma
Clear cell sarcoma
R habdomyosa rcoma *
Unclassified sarcoma/so-called malignant fibrous histiocytoma
Malignant peripheral nerve sheath tumor (MPNST)
Malignant glomus tumor/malignant myopericytoma
Myoepithelioma/mixed tumor of soft tissue
Leiomyosarcoma/liposarcoma/angiosarcoma

•Occurs exclusively or predominantly in infants, children, and adolescents

158 T U M O R S O F T H E HAND

Figure 1 Figure 2
Biphasic synovial sarcoma. Glandular epithelial structures Monophasic synovial sarcoma. Dense proliferation of spin-
are surrounded by malignant spindle cells. Hematoxylin and dle cells showing a fascicular growth pattern. This variant is
eosin x 20. indistinguishable from fibrosarcoma or malignant peripheral
nerve sheath tumor. Hematoxylin and eosin x 10.

tumors, and adnexal tumors, including myo-

s p i n d l e cell S S ( G u i l l o u et al 1996, P e l m u s et al
e p i t h e l i o m a s . T h e s p i n d l e cell m o n o p h a s i c f o r m
2002). O f S S s 9 0 - 9 5 p e r c e n t , r e g a r d l e s s of t h e i r
resembles fibrosarcoma, leiomyosarcoma, mali-
histological appearance, bear the translocation
gnant peripheral nerve sheath tumor, a n d malig-
t ( X ; 1 8 ) (p11;q11) w h i c h is s p e c i f i c f o r t h i s tumor
nant solitary f i b r o u s t u m o r , w h e r e a s t h e poorly
t y p e { v a n d e R i j n e t al 1999, G u i l l o u e t al 2 0 0 1 ,
differentiated/round cell v a r i a n t s h o u l d b e distin-
S a n d b e r g a n d B r i d g e 2002). T h i s translocation
g u i s h e d f r o m s m a l l r o u n d cell t u m o r s , e s p e c i a l l y
generates a transcription-activating fusion pro-
from extraskeletal Ewing's sarcoma/primitive
tein f r o m the products of t h e S S X 1 o r SSX2
neuroectodermal tumor and rhabdomyosarcoma.
genes from chromosome Xp11, and the S Y T gene
S S is a rare but life-threatening t u m o r . H o w e v e r ,
f r o m c h r o m o s o m e 18q11 ( S a n d b e r g a n d B r i d g e
it c a n be c u r e d using a g g r e s s i v e t r e a t m e n t m o d a l i -
2002). Using reverse transcriptase-polymerase
ties. B e c a u s e S S s of t h e h a n d a r e often s m a l l a n d
chain reaction (RT-PCR), fusion g e n e transcripts
detected early, t h e y rarely require amputation,
c a n b e d e t e c t e d in frozen ( v a n d e R i j n et al 1999)
a l t h o u g h their p r o g n o s i s s e e m s to b e no better
a n d in f o r m a l i n - f i x e d , p a r a f f i n - e m b e d d e d m a t e r i a l
than that of similarly sized tumors in other
with an overall sensitivity of 96 p e r c e n t a n d
a n a t o m i c locations (Lin et al 2002). E x a m i n a t i o n of
specificity of 100 p e r c e n t ( G u i l l o u et al 2 0 0 1 ,
t e n localized S S s of t h e h a n d r e t r i e v e d f r o m t h e
S a n d b e r g a n d B r i d g e 2002). F u s i o n p r o t e i n t y p e
French Sarcoma Group database revealed a
( S Y T - S X X 1 v e r s u s S Y T - S S X 2 ) is a l s o t h o u g h t to
median survival of 36 months (range 11-107
b e of p r o g n o s t i c v a l u e ( L a d a n y i et al 2002), but
m o n t h s ) ; t h r e e patients (30 p e r cent) d e v e l o p e d
t h i s h a s not y e t b e e n c o n f i r m e d ( G u i l l o u et al
lung m e t a s t a s e s a n d d i e d of their d i s e a s e .
2003). Of interest, b i p h a s i c S S a r e r a r e l y a s s o c i -
ated with S Y T - S S X 2 fusion transcripts (Ladanyi
et al 2 0 0 2 , G u i l l o u et al 2003).
T h e differential d i a g n o s i s of S S is large. Briefly, Epithelioid sarcoma
p r e d o m i n a n t l y b i p h a s i c S S s h o u l d be differentiated
f r o m metastatic adenocarcinomas, carcinosarco- Epithelioid s a r c o m a ( E S ) w a s recognized a s a dis-
mas, glandular malignant peripheral nerve sheath tinct e n t i t y in 1970 w h e n E n z i n g e r (1970) r e p o r t e d
 MALIGNANT S O F T T I S S U E T U M O R S O F T H E HAND 159

Figure 3

Epithelioid sarcoma. Intimate admixture of epithelioid and

spindle cells, set in a collagenized extracellular matrix.
Hematoxylin and eosin x 20.

62 c a s e s of this unusual neoplasm (Enzinger

1970). In its c l a s s i c a l f o r m , E S o c c u r s in t h e distal
e x t r e m i t i e s ( h a n d , w r i s t , f o r e a r m ) of y o u n g a d u l t s Figure 4
p r e s e n t i n g a s a f i r m , s l o w - g r o w i n g n o d u l e of t h e
s u b c u t i s , t e n d o n s a n d / o r f a s c i a . U l c e r a t i o n of t h e Epithelioid sarcoma. Central necrosis in a dermal nodule
skin m a y o c c u r . A h i s t o r y of t r a u m a is p r e s e n t in resulting in a 'pseudo granuloma annulare' appearance.
u p t o 20 p e r c e n t of c a s e s ( E n z i n g e r 1970). A m o n g Hematoxylin and eosin x 4.
1945 soft tissue s a r c o m a s retrieved from the
F r e n c h S a r c o m a G r o u p d a t a b a s e , s e v e n (0.3 p e r
c e n t ) w e r e E S of t h e h a n d . M e d i a n p a t i e n t a g e
w a s 35 y e a r s , a n d m e d i a n t u m o r size 3.5 c m . Immunohistochemically, E S is characteristi-
On microscopic examination, the conventional c a l l y i m m u n o r e a c t i v e for v i m e n t i n a n d e p i t h e l i a l
' d i s t a l ' f o r m of E S is c h a r a c t e r i z e d b y a mixed m a r k e r s ( c y t o k e r a t i n s a n d / o r E M A ) ( M e i s et al
proliferation of epithelioid and spindle cells 1988, M i e t t i n e n e t a l 1999), a n d a b o u t half of c a s e s
exhibiting slight nuclear atypia, vesicular nuclei, a r e a l s o p o s i t i v e for C D 3 4 a n d s m o o t h muscle
and small nucleoli ( E n z i n g e r 1970, C h a s e and actin ( M i e t t i n e n et al 1999). Ultrastructurally, E S
E n z i n g e r 1985). T r a n s i t i o n b e t w e e n t h e t w o cell s h o w s f e a t u r e s of fibroblastic/myofibroblastic and
t y p e s is g r a d u a l a n d intercellular c o l l a g e n d e p o - epithelial ( d e s m o s o m e - l i k e intercellular junctions,
sition is u s u a l l y m a r k e d . F r e q u e n t l y , t h e tumor microvilli, tonofilaments) differentiation. Many
nodules undergo central necrosis resulting in entities h a v e to b e c o n s i d e r e d in t h e differential
a pseudogranulomatous appearance simulating d i a g n o s i s of E S i n c l u d i n g c a r c i n o m a , m e l a n o m a ,
a benign necrobiotic process such as a rheuma- epithelioid malignant peripheral nerve sheath
t o i d n o d u l e or a g r a n u l o m a annulare (Fig. 4). tumor, smooth a n d striated muscle sarcomas,
P s e u d o a n g i o s a r c o m a t o u s f e a t u r e s d u e to cell d i s - epithelioi d angiosarcoma, and rhabdoid tumor
a g g r e g a t i o n , d y s t r o p h i c c a l c i f i c a t i o n s , b o n e for- ( M i e t t i n e n e t al 1999). B e n i g n c o n d i t i o n s such
mation, and a c c o m p a n y i n g chronic inflammation as rheumatoid nodule and granuloma annulare
a r e p o t e n t i a l a d d i t i o n a l f e a t u r e s ( E n z i n g e r 1970, s h o u l d a l s o b e i n c l u d e d , e s p e c i a l l y if a g r a n u l o -
C h a s e a n d E n z i n g e r 1985). m a t o u s pattern is e v i d e n t . I m m u n o h i s t o c h e m i s t r y
160 T U M O R S OF THE HAND

and/or electron microscopy a r e of paramount

i m p o r t a n c e in t h i s r e g a r d .
Conventional E S is c h a r a c t e r i z e d by a pro-
tracted clinical course (the median overall
survival of our s e v e n c a s e s w a s 73 months).
M e t a s t a s e s , w h i c h d e v e l o p in a b o u t 40 p e r c e n t of
p a t i e n t s , u s u a l l y after r e p e a t e d l o c a l r e c u r r e n c e s ,
p r i m a r i l y i n v o l v e r e g i o n a l l y m p h n o d e s but l u n g s ,
bone, and scalp can also be involved (Chase and
E n z i n g e r 1985, E v a n s a n d B a e r 1993, S p i l l a n e et al
2 0 0 0 , Callister et al 2001). T e n - y e a r o v e r a l l s u r -
v i v a l r a t e s r a n g e b e t w e e n 50 p e r c e n t ( B o s et al
1988, E v a n s a n d B a e r 1993, S p i l l a n e et al 2 0 0 0 ,
Callister et al 2001) a n d 74 p e r c e n t ( H a i l i n g et al
1996). T h e o v e r a l l r e c u r r e n c e rate v a r i e s b e t w e e n
40 p e r c e n t a n d 80 p e r c e n t at 10 y e a r s . T u m o r size
is o n e of t h e m o s t i m p o r t a n t p r o g n o s t i c f a c t o r s ; a
t u m o r > 5 c m is o f t e n a s s o c i a t e d w i t h p o o r p r o g -
Figure 5
n o s i s a n d l o w o v e r a l l s u r v i v a l rate ( E v a n s a n d Clear cell sarcoma. Fibrous septa divide the spindle cell
B a e r 1993, Calliste r et al 2001). proliferation in short fascicles. Hematoxylin and eosin x 20.
R e c e n t l y , a particular t y p e of m a l i g n a n t soft
tissue n e o p l a s m thought to represent a 'proxi-
m a l ' v a r i a n t o f E S h a s b e e n d e s c r i b e d ( G u i l l o u et
al 1997). In t h i s v a r i a n t , t h e t u m o r s d e v e l o p p r e - Clinically, c l e a r cell s a r c o m a is a s l o w - g r o w i n g
dominantly in t h e p e l v i s , p e r i n e u m , a n d g e n i t a l t u m o r ( m e d i a n size 2 - 5 c m ) t h a t m a i n l y affects
tract ( p u b i s , v u l v a , p e n i s ) , r a r e ly in distal e x t r e m - y o u n g a d u l t s ( 2 0 - 4 0 y e a r s ) . T h e distal e x t r e m i t i e s ,
ities. ' P r o x i m a l - t y p e ' E S s e e m s t o b e a s s o c i a t e d particularly the feet, ankles, knees, wrists, and
w i t h a m o r e a g g r e s s i v e clinical c o u r s e a n d e a r l i e r hands are the most common sites of tumor
tumor-related deaths compared with conven- d e v e l o p m e n t ( E n z i n g e r 1965, L u c a s e t a l 1992,
t i o n a l E S ( C h a s e a n d E n z i n g e r 1985, M e i s et al Montgomery et al 1993, Deenik et al 1999,
1988, G u i l l o u et al 1997). M a r q u e s et al 2 0 0 0 , W e i s s a n d G o l d b l u m 2001).
T u m o r s m a y be present for y e a r s before c o m i n g
to clinical attention. Large masses may cause
Clear cell sarcoma pain and tenderness.
Histologically, the t u m o r presents a s b u n d l es
D e s c r i b e d in 1965 b y E n z i n g e r , c l e a r cell s a r c o m a of p a l e s t a i n i n g to e o s i n o p h i l i c s p i n d l e c e l l s s e p -
of t e n d o n s a n d a p o n e u r o s e s is a p e c u l i a r t y p e of arated by dense fibrous tissue septa ( F i g . 5).
s a r c o m a w h i c h s h o w s f e a t u r e s of m e l a n o c y t i c dif- T u m o r cells h a v e prominent vesicular nuclei w i t h
ferentiation (Enzinger 1965). Although some a large central single nucleolus. Scattered multi-
authors occasionally refer t o it as 'malignant nuclear giant cells w i t h peripheral nuclei arranged
m e l a n o m a o f soft parts', it differs f r o m c u t a n e o u s in a w r e a t h - l i k e pattern a r e o c c a s i o n a l l y o b s e r v e d
m e l a n o m a in s e v e r a l r e s p e c t s : it is a l m o s t a l w a y s ( F i g . 6). A p p r o p r i a t e histochemical stains may
deep-seated, associated with tendons, tendon r e v e a l i n t r a c e l l u l ar m e l a n i n a c c u m u l a t i o n in half
s h e a t h s , a n d a p o n e u r o s e s , a n d r a r e ly i n v o l v e s t h e of c a s e s .
e p i d e r m i s . In a d d i t i o n , c l e a r cell s a r c o m a b e a r s Immunohistochemically, clear cell sarcoma
t h e t(12;22) (q13;q12) t r a n s l o c a t i o n w h i c h is s p e - cells e x p r e s s m e l a n o c y t i c m a r k e r s ( S 1 0 0 protein,
cific for t h i s t u m o r t y p e a n d h a s n e v e r b e e n i d e n - HMB45, Melan-A, microphthalmia transcription
tified in c u t a n e o u s melanoma ( S t e n m a n et al factor) a n d a r e n e g a t i v e for E M A , cytokeratins,
1992). T h i s t r a n s l o c a t i o n , p r e s e n t in up to 7 5 per a n d d e s m i n ( L u c a s et al 1992, M o n t g o m e r y et al
c e n t of c a s e s , f u s e s t h e ATF-1 g e n e (a t r a n s c r i p t i o n 1993, D e e n i k et al 1999, M a r q u e s et al 2000).
f a c t o r ) o n c h r o m o s o m e 12 w i t h t h e E W S g e n e o n M e l a n o s o m e s at v a r y i n g s t a g e s of development
c h r o m o s o m e 22 ( Z u c m a n et al 1993). are visible ultrastructurally. C l e a r cell sarcoma
 MALIGNANT S O F T T I S S U E T U M O R S O F T H E HAND 161

Figure 6 Figure 7

Clear cell sarcoma. The spindle to round cell proliferation Inflammatory myxohyaline tumor. Admixture of myxoid and
contains scattered multinucleate giant cells. Wreath-like hyaline areas. Hematoxylin and eosin x 20.
arrangement of nuclei in giant cells. Tumor cells show
prominent central nucleoli. Hematoxylin and eosin x40.

s h o u l d b e differentiated f r o m metastatic s p i n d l e t e r m s : i n f l a m m a t o r y m y x o h y a l i n e t u m o r of distal

cell m e l a n o m a a n d other s p i n d l e cell s a r c o m a s , e x t r e m i t i e s w i t h v i r o c y t e or R e e d - S t e r n b e r g - l i k e
namely fibrosarcoma, synovial sarcoma, malignant c e l l s ( M o n t g o m e r y et al 1998), a n d a c r a l m y x o -
peripheral n e r v e sheath tumor, a n d leiomyosar- i n f l a m m a t o r y fibroblastic s a r c o m a ( M e i s - K i n d b l o m
coma. Both immunohistochemical staining and a n d K i n d b l o m 1998).
d e t e c t i o n of A T F 1 - E W S f u s i o n transcripts b y RT- The inflammatory myxohyaline tumor most
P C R c a n help define the diagnosis. commonly d e v e l o p s in the distal extremities
Clear cell sarcoma has a poor prognosis, ( h a n d , w r i s t , f o o t , a n k l e ) of a d u l t p a t i e n t s . T h e
despite appropriate therapy (Enzinger 1965, upper extremites, especially fingers and hands,
L u c a s et al 1992, M o n t g o m e r y et al 1993, D e e n i k are more frequently affected than the lower
et al 1999, M a r q u e s et al 2000). O f t h e four e x t r e m i t i e s . Clinically, t h e l e s i o n is often m i s t a k e n
p a t i e n t s w i t h a c l e a r cell s a r c o m a of t h e h a n d for a g a n g l i o n c y s t or t e n o s y n o v i t i s . M e a s u r i n g
identified in t h e F r e n c h S a r c o m a G r o u p d a t a b a s e , 3-4 c m on average with a gelatinous, multi-
t w o d i e d of m e t a s t a s e s 34 a n d 39 m o n t h s after n o d u l a r g r o s s a p p e a r a n c e , t h e l e s i o n is f o u n d in
d i a g n o s i s . T h e t u m o r t e n d s to m e t a s t a s i z e to t h e t h e s u b c u t i s o r in t h e d e e p soft t i s s u e s . It i n v o l v e s
l u n g s a n d a l s o to regional lymph nodes and and/or envelops tendon sheaths a n d the s y n o v i u m
b o n e . T h e 5 - y e a r s u r v i v a l rate is a b o u t 5 0 - 6 0 p e r of a d j a c e n t j o i n t s . H i s t o l o g i c a l l y , t h e a l t e r n a t i n g
cent. Long-term follow-up is m a n d a t o r y since p r e s e n c e of cellular, m y x o i d , a n d h y a l i n e z o n e s
m e t a s t a s e s m a y d e v e l o p late in t h e c o u r s e of t h e w i t h m a r k e d c h r o n i c i n f l a m m a t i o n is c h a r a c t e r i s -
d i s e a s e (10 y e a r s or m o r e after d i a g n o s i s ) . tic ( F i g . 7). E x a m i n a t i o n of c e l l u l a r z o n e s r e v e a l s
atypical spindle to pleomorphic cells with
enlarged nuclei a n d prominent central nucleoli
( F i g . 8). T h e h i s t i o c y t o i d o r e p i t h e l i o i d morpho-
Inflammatory myxohyaline
l o g y , v e s i c u l a r n u c l e i , a n d m a c r o n u c l e o l i of s o m e
tumor/myxoinflammatory cells resemble virocytes whereas others may
fibroblastic sarcoma resemble Reed-Sternberg cells.The more myxoid
zones contain uni- or multivacuolated cells with
T h i s l e s i o n w a s r e c o g n i z e d a s a distinct e n t i t y in enlarged hyperchromatic nuclei reminiscent of
1998 b y t w o different t e a m s w h o c o i n e d different lipoblasts. In a d d i t i o n , t h e l e s i o n , characteristically,
162 T U M O R S O F T H E HAND

Plexiform fibrohistiocytic tumor

Plexiform fibrohistiocytic tumor is a r a r e low-
grade sarcoma occurring almost exclusively
in children and young adults, with strong
f e m a l e predilection (Enzinger and Zhang 1988,
H o l l o w o o d e t al 1 9 9 1 , R e m s t e i n et al 1999, W e i s s
a n d G o l d b l u m 2001). M o s t p a t i e n t s c o m p l a i n of
small (1-3 c m ) painless d e r m a l or subcutanous
n o d u l e s , m o s t often l o c a t e d in t h e u p p e r e x t r e m -
ity. W r i s t s , hands, and fingers are affected in
a b o u t o n e - q u a r t e r of c a s e s ( E n z i n g e r a n d Z h a n g
1988, Hollowood et al 1991, Salomao and
Nascimento 1997, R e m s t e i n et al 1999). Histo-
logically, t h e l e s i o n is c o m p o s e d of a v a r i a b l e
m i x t u r e of i r r e g u l a r l y sized h i s t i o c y t i c nodules
Figure 8 containing osteoclast-like multinucleated giant
cells and ramifying fibroblastic fascicles infil-
Inflammatory myxohyaline tumor. Myxoid areas contain trating the dermis and subcutis in a diffuse
vacuolated cells with pleomorphic nuclei resembling m a n n e r , r e s u l t i n g in a p l e x i f o r m a n d infiltrative
lipoblasts. Hematoxylin and eosin x 40. (fibromatosis-like) appearance on low-power
e x a m i n a t i o n ( F i g . 9). N u c l e a r a t y p i a , c e l l u l a r p l e o -
morphism, and mitotic activity are minimal.
contains a p r o m i n e n t l y m p h o c y t i c a n d plasmacytic Osteoclast-like cells a n d m o n o n u c l e a r histiocytic
inflammatory component a n d , less frequently, cells are positive for CD68, w h e r e a s spindle cells
polymorphonuclear neutrophils. Multinucleated within fibroblastic fascicles express smooth
g i a n t c e l l s , h e m o s i d e r i n d e p o s i t s , a n d n e s t s of muscle actin. Desmin and S100 protein are
epithelioid cells m a y be o b s e r v e d . u s u a l l y n e g a t i v e . Differential diagnosis includes
Immunohistochemically, tumor cells express fibromatosis and lipofibromatosis (especially for
vimentin and CD68 (clone K P 1 and P G M 1 ) , and predominantly fibroblastic tumors), giant cell
focally CD34, s m o o t h m u s c l e actin, and/or cyto- tumor, and fibrous histiocytoma.
keratin (Meis-Kindblom and Kindblom 1998, W i d e l o c a l e x c i s i o n w i t h t u m o r - f r e e m a r g i n s is
M o n t g o m e r y et al 1998). t h e t r e a t m e n t of c h o i c e for t h e p l e x i f o r m f i b r o -
Inflammatory myxohyaline tumor recurs in histiocytic tumor. Local recurrence rates range
a b o u t 20 p e r c e n t of c a s e s , a n d m e t a s t a s i z e s rarely f r o m 12 to 40 p e r c e n t , m e t a s t a s e s t o regional
(less t h a n 5 p e r c e n t of c a s e s ) ( M e i s - K i n d b l o m a n d l y m p h n o d e s a n d / o r l u n g s a r e o b s e r v e d in l e s s
K i n d b l o m 1998, M o n t g o m e r y et al 1998). T h e high t h a n 5 p e r c e n t of c a s e s . N o h i s t o l o g i c a l para-
proportion (67 per cent) of recurrent cases meters have been correlated with aggressive
reported b y M e i s - K i n d b l o m a n d K i n d b l o m (1998) is b e h a v i o r ( E n z i n g e r a n d Z h a n g 1988, H o l l o w o o d
probably related to initial i n c o m p l e t e excisions et al 1991, S a l o m a o and Nascimento 1997,
d u e t o e r r o n e o u s d i a g n o s e s of benignity. R e m s t e i n et al 1999).
This tumor should be distinguished from
inflammatory processes such as chronic teno-
synovitis, inflammatory pseudotumor, mucoid
pseudocyst with superimposed inflammatory Myoepithelioma/mixed
c h a n g e s , or g i a n t cell t u m o r of t e n d o n s h e a t h if tumor of soft tissue
multinucleated giant cells are n u m e r o u s . A m o n g
malignant lesions, myxoid malignant fibrous M y o e p i t h e l i o m a s a n d m i x e d t u m o r s b e l o n g to t h e
histiocytoma (myxofibrosarcoma), pleomorphic same morphologic spectrum. Although these
l i p o s a r c o m a ( o w i n g to t h e p r e s e n c e of p s e u d o - t u m o r s a r e m o s t c o m m o n l y o b s e r v e d in t h e skin, a
lipoblastic cells), a n d i n f l a m m a t o ry fibrosarcoma primary localization in soft t i s s u e s h a s recently
a r e s o u r c e s of p o t e n t i a l error. b e e n recognized ( B a n e r j e e et al 1993, Ferreiro a n d
 MALIGNANT S O F T T I S S U E T U M O R S O F T H E HAND 163

Figure 9 Figure 10

Plexiform fibrohistiocytic tumor. The dermis and hypo- Myoepithelioma of soft tissues. Cords and strands of
dermis are occupied by multiple histiocyte-like nodules. epithelioid cells set in an abundant myxo-chondro-hyaline
Hematoxylin and eosin x 10. matrix. Hematoxylin and eosin x 10.

N a s c i m e n t o 1995, Kilpatrick et al 1997, M i c h a l a n d c a s e s r e v e a l e d a 4 2 p e r c e n t r e c u r r e n c e rate a n d

M i e t t i n e n 1999, Hornick a n d Fletcher 2003). In all, a 32 p e r c e n t m e t a s t a t i c rate. C y t o l o g i c a l l y b e n i g n
2 0 - 2 5 per c e n t a r e o b s e r v e d in distal extremities of or low-grade lesions m a y occasionally recur
m i d d l e - a g e d adults (35-45 y e a r s ) , including wrist, (18 per cent of c a s e s ) but, in g e n e r a l , d o not m e t a s -
h a n d , f i n g e r s , a n k l e , feet, a n d t o e s (Hornick a n d tasize. W i d e e x c i s i o n is t h e t r e a t m e n t of c h o i c e .
Fletcher 2003). Men and women are equally R e g a r d i n g t h e differential d i a g n o s i s , m y o e p i t h e -
affected. G r o s s l y , t h e lesion p r e s e n t s a s a w e l l - lial t u m o r s of soft t i s s u e s h o u l d b e distinguished
delineated nodule measuring 2-5 c m on average. f r o m extraskeletal m y x o i d c h o n d r o s a r c o m a s , soft
A b o u t 60 per cent of t u m o r s develop subcuta- tissue chondromas, parachordomas, chondroid
n e o u s l y . Histologically, m o s t t u m o r s a r e l o b u l a t e d , l i p o m a s , a n d o s s i f y i n g f i b r o m y x o i d t u m o r s of
c o m p o s e d of c o r d s or nests of epithelioid t o s p i n - soft tissue. S o m e lesions of t h e distal extremities
dle cells set in a c h o n d r o m y x o i d or c o l l a g e n o u s / reported a s c h o n d r o i d s y r i n g o m a ( B a n e r j e e et al
hyalinized s t r o m a ( F i g . 10). In a d d i t i o n , ductular 1993, Ferreiro a n d N a s c i m e n t o 1995) c o r r e s p o n d , in
differentiation, plasmocytoid cells, cartilage, a n d all likelihood, to m y o e p i t h e l i o m a s of soft t i s s u e s .
bone are variably observed. By immunohisto-
chemistry, t u m o r cells of m y o e p i t h e l i o m a s usually
e x p r e s s epithelial markers (cytokeratins and/or
epithelial m e m b r a n e a n t i g e n ) , S 1 0 0 protein, a n d Conclusion
c a l p o n i n ; half of t h e m e x p r e s s t h e glial fibrillary
acidic protein, and one-third smooth muscle V a r i o u s t y p e s of malignant soft t i s s u e lesions
actin. D e s m i n is positive in a b o u t 15 per cent of m a y b e o b s e r v e d in t h e h a n d , of w h i c h o n l y a
the cases. few have been considered in this review.
Myoepitheliomas and mixed tumors of soft P o t e n t i a l d i a g n o s t i c pitfalls a r e n u m e r o u s . A g o o d
tissue a r e potentially recurring a n d metastasizing u p - t o - d a t e k n o w l e d g e of soft t i s s u e t u m o r p a t h o l -
tumors. According to a recent s e r i e s of 101 o g y c o m b i n e d w i t h a logical d i a g n o s t i c a p p r o a c h ,
m y o e p i t h e l i a l t u m o r s of soft t i s s u e ( H o r n i c k a n d taking into a c c o u n t clinical, radiologic, patho-
F l e t c h e r 2003), 60 p e r c e n t s h o w e d histological logic, a n d m o l e c u l a r d a t a is t h e best w a y t o a v o i d
f e a t u r e s of m a l i g n a n c y ( c e l l u l a r a t y p i a , m i t o s e s , misdiagnoses a n d , thus, w h e r e possible avoid
e t c . ) . A f o l l o w - u p s t u d y of c y t o l o g i c a l l y malignant mutilating surgery.
164 T U M O R S OF THE HAND

Fisher C (1998) Synovial sarcoma, Ann

References 2:401-21.
Diagn Pathol

Arbiser ZK, Folpe A L , Weiss S W (2001) Consultative

(expert) second opinions in soft tissue pathology. Guillou L, Wadden C, Kraus M D , Dei Tos AP, Fletcher
Analysis of problem-prone diagnostic situations, Am J C D M (1996) S-100 protein reactivity in synovial sarcoma:
Clin Pathol 116:473-6. a potentially frequent diagnostic pitfall. Immunohisto-
chemical analysis of 100 cases, Appl Immunohistochem
4:167-75.
Banerjee S S , Harris M, Eyden B P et al (1993) Chondroid
syringoma with hyaline cell change, Histopathology
22:235-45. Guillou L, Wadden C, Coindre J M et al (1997) 'Proximal-
type' epithelioid sarcoma, a distinctive aggressive neo-
plasm showing rhabdoid features. Clinicopathologic,
B o s G D , Pritchard D J , R e i m a n H M et al (1988)
immunohistochemical, and ultrastructural study of a
Epithelioid sarcoma. A n analysis of 51 cases, J Bone
series, Am J Surg Pathol 21:130-46.
Joint Surg 70:862-70.

Guillou L, Coindre J M , Gallagher G,Terrier P, Gebhard S ,

Callister MD, Ballo NIT, Pisters P W et al (2001) Epithelioid
de Saint Aubain Somerhausen N, Michels J J , J u n d t G ,
sarcoma: results of conservative surgery and radio-
Ranchère-Vince D, Collin F, Trassard M , Le Doussal V,
therapy, Int J Radiat Oncol Biol Phys 51:384-91.
Benhattar J (2001) Detection of the synovial sarcoma
translocation t(X;18) (SYT;SSX) in paraffin-embedded tis-
Chase DR, Enzinger F M (1985) Epithelioid sarcoma:
sues using reverse transcriptase-polymerase chain reac-
diagnosis, prognostic indicators and treatment, Am J
tion: a reliable and powerful diagnostic tool for
Surg Pathol 9:241-63.
pathologists. A molecular analysis of 221 mesenchymal
tumors fixed in different fixatives, Hum Pathol 32:105-12.
Deenik W , Mooi W J , Rutgers E J et al (1999) Clear cell
sarcoma (malignant melanoma) of soft parts. A clinico-
Guillou L, Benhattar J , Terrier P et al (2003) S Y T - S S X
pathologic study of 30 cases, Cancer 86:969-75.
fusion type is not a prognostic factor in synovial sar-
coma ( S S ) patients. A multi-institutional study of 182
Dei Tos AP, W a d d e n C, Calonje E et al (1995) Immuno- cases, Mod Pathol 16:13A, abstr 43.
histochemical demonstration of glycoprotein p30/32 mic2

(CD99) in synovial sarcoma: a potential cause of

Hailing A C , W o l l a n P C , Pritchard D J et al (1996)
diagnostic confusion, Appl Immunohistochem 3:168-73.
Epithelioid sarcoma: a clinicopathologic review of 55
cases, Mayo Clin Proc 71:636-42.
Egger J F , Coindre J M , Benhattar J et al (2002) Radiation-
associated synovial sarcoma: clinicopathologic and
Hollowood K, Holley MP, Fletcher C D M (1991) Plexiform
molecular analysis of two cases, Mod Pathol 15:
fibrohistiocytic tumour. Clinicopathological, immuno-
998-1004.
histochemical, and ultrastructural analysis in favour of a
myofibroblastic lesion, Histopathology 19:503-13.
Enzinger F M (1965) Clear cell sarcoma of tendons and
aponeuroses. A n analysis of 21 cases, Cancer 18:
Hornick J L , Fletcher C D M (2003) Myoepithelial tumors
1163-74.
of soft tissue. A clinicopathologic and immunohisto-
chemical study of 101 cases with evaluation of prognos-
Enzinger F M (1970) Epithelioid sarcoma. A sarcoma sim- tic parameters, Am J Surg Pathol 27:1183-96.
ulating a granuloma or a carcinoma, Cancer 26:1029-41.

Kilpatrick S E , Hitchcock M G , Kraus M D et al (1997)

Enzinger F M , Zhang RY (1988) Plexiform fibrohistiocytic Mixed tumors and myoepitheliomas of soft tissue. A
tumor presenting in children and young adults. A n clinicopathologic study of 19 cases with a unifying con-
analysis of 65 cases, Am J Surg Pathol 12:818-26. cept, Am J Surg Pathol 21:13-22.

Evans H, Baer S (1993) Epithelioid sarcoma: a clinico- Ladanyi M , Antonescu CR, Leung DH, Woodruff J M ,
pathologic and prognostic study of 26 cases, Semin Kawai A, Healey J H , Brennan MF, Bridge J A , Neff J R ,
Diagn Pathol 10:286-91. Barr F G , Goldsmith J D , Brooks J S , Goldblum J R , Ali S Z ,
Shipley J , Cooper C S , Fisher C, Skytting B, Larsson O
Ferreiro J A , Nascimento A G (1995) Hyaline-cell rich (2002) Impact of S Y T - S S X fusion type on the clinical
chondroid syringoma. A tumor mimicking malignancy, behavior of synovial sarcoma: a multi-institutional retro-
Am J Surg Pathol 19:912-17. spective study of 243 patients, Cancer Res 62:135-40.
 MALIGNANT S O F T T I S S U E T U M O R S OF THE HAND 165

Lin PP, Guzel V B , Pisters P W et al (2002) Surgical Pelmus M , Guillou L, Hostein I et al (2002) Monophasic
management of soft tissue sarcomas of the hand and fibrous and poorly differentiated synovial sarcoma:
foot, Cancer 95:852-61. immunohistochemical reassessment of 60 t(X;18)(SYT-
SSX)-positive cases, Am J Surg Pathol 26:1434-40.
Lucas DR, Nascimento A G , S i m FH (1992) Clear cell
sarcoma of soft tissues: M a y o Clinic experience with Remstein E D , Arndt C A S , Nascimento A G (1999)
35 cases, Am J Surg Pathol 16:1197-204. Plexiform fibrohistiocytic tumor: clinicopathologic
analysis of 22 cases, Am J Surg Pathol 23:662-70.
Marqués B, Terrier P, Voigt J J et al (2000) Sarcome ã
cellules claires des tissus mous. Etude clinique, histo- Salomao DR, Nascimento A G (1997) Plexiform fibro-
pathologique et évolutive de 36 cas, Ann Pathol 20: histiocytic tumor with systemic metastases. A case
298-303. report, Am J Surg Pathol 21:469-76.

Meis J M , Mackay B, Ordonez N G (1988) Epithelioid Sandberg A A , Bridge J A (2002) Updates on the cyto-
sarcoma: an immunohistochemical and ultrastructural genetics and molecular genetics of bone and soft tissue
study, Surg Pathol 1:13-31. tumors. Synovial sarcoma, Cancer Genet Cytogenet
133:1-23.
Meis-Kindblom J M , Kindblom LG (1998) Acral myxo-
inflammatory fibroblastic sarcoma: a low-grade tumor Spillane A J , Thomas J M , Fisher C (2000) Epithelioid
of the hands and feet, Am J Surg Pathol 22:911-24. sarcoma: the clinicopathological complexities of this
rare soft tissue sarcoma, Ann Surg Oncol 7:218-25.
Michal M, Miettinen M (1999) Myoepitheliomas of the
skin and soft tissues. Report of 12 cases, Virchows Arch Stenman G , Kindblom L G , Angervall L (1992) Reciprocal
434:393-400. translocation t(12;22) (q13;q13) in clear cell sarcoma of
tendons and aponeuroses, Genes Chromosomes
Miettinen M , Fanburg-Smith J C , Virolainen M et al Cancer 4:122-7.
(1999) Epithelioid sarcoma: an immunohistochemical
analysis of 112 classical and variant cases and a discus- van de Rijn M, Barr F G , Collins M H , Xiong Q B , Fisher C
sion on the differential diagnosis, Hum Pathol (1999) Absence of S Y T - S S X fusion products in soft
30:934-42. tissue tumors other than synovial sarcoma, Am J Clin
Pathol 112:43-9.
Montgomery E A , Meis J M , Ramos A G , Martz KL (1993)
Clear cell sarcoma of tendons and aponeuroses. A Weiss S W , Goldblum J R (2001) Enzinger and Weiss's
clinicopathologic study of 58 cases with analysis of SoftTissueTumors, 4th edn. Mosby: S t Louis, M l .
prognostic factors, Int J Surg Pathol 1:89-100.
Zucman J , Delattre O, Desmaze C et al (1993) E W S and
Montgomery E A , Devaney KO, Giordano T J , Weiss S W ATF1 gene fusion induced by t(12;22) translocation in
(1998) Inflammatory myxohyaline tumor of distal malignant melanoma of soft parts, Nat Genet 4:341-5.
extremities with virocyte or Reed-Sternberg-like cells: a
distinctive lesion with features simulating inflammatory
conditions, Hodgkin's disease, and various sarcomas,
Mod Pathol 11:384-91.
18
Soft tissue sarcoma
of the hand and wrist
Massimo Ceruso, Domenico Campanacci,
Patrizio Caldora and Prospero Bigazzi

Introduction Surgical excision and

associated procedures
T h e s o - c a l l e d 'soft t i s s u e s ' I n c l u d e all t h e n o n -
o s s e o u s tissue of the b o d y excluding skin, l y m p h A ' w i d e ' radical r e s e c t i o n is t h e recommended
n o d e s , p e r i o s t e u m , a n d v i s c e r a . B y t h e t e r m soft treatment for non-metastatic S T S . T h e goal of
tissue sarcoma ( S T S ) , w e m e a n a heterogeneous treatment is t h e e r a d i c a t i o n of t h e entire S T S
g r o u p of m a l i g n a n t t u m o r s of m e s e n c h y m a l or i n c l u d i n g not o n l y its g r o s s l y p a l p a b l e e x t e n t but
neuroectodermal o r i g i n . T h e y a c c o u n t for less a l s o all of its proliferating p e r i p h e r a l cellular lines.
t h a n 1 p e r c e n t of all a d u l t m a l i g n a n t tumors, A n u n i n t e r r u p t e d l a y e r of h e a l t h y t i s s u e s u r r o u n d -
e x c l u s i v e of n o n - m e l a n o m a t o u s skin c a n c e r s . T h e ing t h e t u m o r m u s t b e i n c l u d e d in t h e e x c i s i o n t o
e s t i m a t e d i n c i d e n c e of m a l i g n a n t S T S is a b o u t e n c o m p a s s invisible t u m o r e x t e n s i o n s . A c c o r d i n g
20 new cases/million of population per year to t h i s , t h e m i c r o s c o p i c e v a l u a t i o n of t h e m a r g i n s
(Enzinger and Weiss 1995, C a m p a n a c c i 1999). is of t h e f o r e m o s t i m p o r t a n c e to attest to the
T h e y are ubiquitous in l o c a t i o n ; approximately a d e q u a c y of t h e e x c i s i o n in either c o n s e r v a t i v e
50 p e r c e n t of t h e s e t u m o r s o c c u r in t h e l i m b s . or ablative surgery. T h e s e are evaluated accord-
High-degree malignancies are more common ing to t h e M u s c u l o s k e l e t a l T u m o r S o c i e t y criteria
than l o w - d e g r e e m a l i g n a n c i e s , w i t h a ratio of ( E n n e k i n g 1986), w h i c h c l a s s i f y t h e m a r g i n s a s
1.6 : 1. A p p r o x i m a t e l y 50 p e r c e n t of t h e s e l e s i o n s r a d i c a l , w i d e , m a r g i n a l , or i n t r a l e s i o n a l . R a d i c a l
have a diameter larger than 5 c m at the first and w i d e surgical margins are considered a d e -
examination. q u a t e in o r d e r to achieve local c o n t r o l of the
B e n i g n soft t i s s u e l e s i o n s a r e m u c h m o r e f r e - t u m o r , w h i l e a m a r g i n a l o r intralesional e x c i s i o n
q u e n t t h a n s a r c o m a s w i t h a rate of 100 : 1 , m o r e - is c o n s i d e r e d inadequate. A radical margin is
over, the i n c i d e n c e of pseudotumoral benign obtained w h e n the tumor is r e m o v e d together
l e s i o n s of t h e h a n d a n d w r i s t r a n g e s f r o m 50 to w i t h a n entire a n a t o m i c c o m p a r t m e n t (e.g. an
70 p e r c e n t of all n e o p l a s t i c l e s i o n s . entire muscular compartment for a soft tissue
A c c o r d i n g t o t h e literature ( J o h n s o n et al 1985, m a s s l o c a t e d in t h e p r o x i m a l part of a l i m b ) .
B e s s e r et a l 1987, B r i e n e t al 1995, M c P h e e e t al A l e s i o n a r i s i n g in t h e h a n d a n d w r i s t is intra-
1999), 2 - 4 p e r c e n t of m a l i g n a n t b o n e a n d soft tis- c o m p a r t m e n t a l in o n l y t w o c o n d i t i o n s : w h e n it is
sue tumors are located around the hand and s u p e r f i c i a l to t h e d e e p f a s c i a ( b e i n g l o c a t e d in t h e
w r i s t . T h e s e r i e s of S T S t r e a t e d at t h e a u t h o r s ' s u b c u t a n e o u s t i s s u e ) or w h e n it is l i m i t e d to a
institution, w h i c h is a n a t i o n a l referral c e n t e r for s i n g l e digital ray. In a n y o t h e r c o n d i t i o n a soft
t h e s e t u m o r s , a c c o u n t s for 14 p e r c e n t of S T S of t i s s u e t u m o r of t h e h a n d a n d w r i s t is c o n s i d e r e d
the hand a n d wrist. as having an extracompartmental extension.
168 T U M O R S O F T H E HAND

A w i d e m a r g i n is o b t a i n e d w h e n t h e t u m o r is
excised being covered by a cuff of healthy
uninterrupted tissue. W h e n the dissection plane
f o l l o w s t h e p s e u d o c a p s u l e of t h e t u m o r t h e e x c i -
s i o n m a r g i n is c o n s i d e r e d a s m a r g i n a l , w h i l e t h e
v i o l a t i o n of t h e t u m o r p s e u d o c a p s u l e d e t e r m i n e s
an intralesional excision margin. Marginal and
i n t r a l e s i o n a l e x c i s i o n s a r e c o n s i d e r e d to b e i n a d -
e q u a t e for t h e l o c a l c o n t r o l of soft t i s s u e s a r c o m a .
L i m b a m p u t a t i o n , p e r s e , d o e s not g u a r a n t e e
t h a t t h e r e s e c t i o n is r a d i c a l . A n a m p u t a t i o n pro-
cedure, regardless h o w major or extensive, must
b e c l a s s i f i e d a c c o r d i n g t o t h e c h a r a c t e r i s t i c s of its
m a r g i n s ; it c a n b e m a r g i n a l a s w e l l a s i n t r a l e -
sional and can consequently be inadequate.
T h e r e f o r e , s u c h a p r o c e d u r e in itself d o e s not
offer a n y p r o g n o s t i c a d v a n t a g e t o t h e p a t i e n t .
Following S T S excision, adjuvant postopera-
tive radiation therapy h a s b e e n recommended
(Talbert et al 1990, A l e k h e t e y a r e t al 1996, C h e n g
et al 1996, P i s t e r s 1998). R a d i a t i o n t h e r a p y m a y
be delivered by external b e a m irradiation, by
interstitial curietherapy (brachytherapy) and
w i t h a c o m b i n a t i o n of b o t h t e c h n i q u e s . T h e c o m -
b i n e d t r e a t m e n t a l l o w s a n i n c r e a s e of t h e total
d o s e ( 6 5 - 7 5 G y ) w i t h a l o w e r risk of c o m p l i c a t i o n
in r e s p e c t to t h e same dose released with a
s i n g l e t e c h n i q u e , a n d a d e c r e a s e in t h e local
recurrence rate.
C h e m o t h e r a p y h a s been e m p l o y e d to i m p r o v e
s y s t e m i c c o n t r o l of S T S of t h e e x t r e m i t i e s with
b
both preoperative and/or postoperative proto-
c o l s , a l t h o u g h its real e f f i c a c y is still c o n t r o v e r s i a l Figure 1
( T i e r n e y e t al 1995).
T h e a d e q u a t e t r e a t m e n t of S T S of t h e h a n d Malignant fibrous hystiocytoma of the left forearm of a
and w r i s t often i n c l u d e s m a j o r a n a t o m i c s t r u c - 63-year-old man. (a) The excision includes the dorsal skin of
the forearm and the extensor muscles. (b) Reconstruction
tures such as tendons, nerves, vessels, and bone
achieved with a myocutaneous latissimus dorsi flap was
s e g m e n t s in t h e r e s e c t i o n a n d c a n b e a s s o c i a t e d
followed by multiple tendon transfers for wrist and finger
w i t h w i d e skin e x c i s i o n s .
extension.
Limb-sparing procedures, satisfying the
r e q u i r e m e n t s of a c o m p a r t m e n t o r a w i d e - m a r g i n
r e s e c t i o n , offer a n a d e q u a t e o p p o r t u n i t y for local
tumor control and prevention of metastasis. functional reconstructions can consequently be
M i c r o s u r g e r y e n h a n c e s t h e possibilities of f u n c - o b t a i n e d ( F i g . 2). R a d i o t h e r a p y a n d / o r c h e m o -
t i o n a l r e c o n s t r u c t i o n s after a n a d e q u a t e w i d e e x c i - t h e r a p y a r e not c o n t r a i n d i c a t e d , e v e n in t h e i m m e -
s i o n of a S T S . M i c r o s u r g i c a l f l a p s p e r m i t wide diate post-operative phase, b e c a u s e unfavorable
tegumental reconstruction in t h e l i m b s ( F i g . 1). biological conditions induced by adjuvant thera-
Moreover, microvascular flaps and techniques p i e s d o not interfere w i t h b l o o d s u p p l y to t h e s e
p e r m i t , in t h e v a s t m a j o r i t y of c a s e s , a o n e - s t a g e f l a p s w h i c h a r e not d e p e n d e n t o n t h e r e c e i v i n g
r e c o n s t r u c t i o n of t e n d o n s , n e r v e s , v e s s e l s , m o t o r b e d for r e v a s c u l a r i z a t i o n . A s a final c o n s i d e r a t i o n ,
units, or osteoarticular segments following the t h e y c o n c u r in p r e v e n t i n g s e p t i c c o m p l i c a t i o n s at
e x c i s i o n of t h e t u m o r ( C e r u s o et al 1995). C o m p l e x t h e site of r e s e c t i o n .
S O F T T I S S U E SARCOMA O F T H E HAND AND W R I S T 169

Figure 2

Inadequate excision of an epitheloid sarcoma in a 32-year-

old patient. !a) Planning of skin excision includes the scar
of the previous operation. (b) X-ray and (c) computed
tomography (CT) scan show the extent of the mass and
contiguity to the first metacarpal, (d) The tumor is excised
including the first metacarpal bone and the extensor
tendons of the thumb. The skeletal reconstruction is
accomplished with a cortico-cancellous iliac graft. (e) A
dorsalis pedis flap is harvested including an extensor ten-
don and nerve graft. (f) Final result and (g) X-ray.
170 T U M O R S O F T H E HAND

Material and methods nerves

grafts.
were reconstructed with sural nerve

T h e o v e r a l l s e r i e s of S T S of t h e l i m b s t r e a t e d at A soft t i s s u e f l a p c o v e r a g e w a s e m p l o y e d in
t h e a u t h o r s ' institution d u r i n g the past d e c a d e 15 c a s e s (58 p e r c e n t ) : a s a n i s l a n d f l a p in e i g h t
w a s r e v i e w e d . T h e r e w e r e 26 patients t r e a t e d for a cases (six radial forearm flaps, two dorsal
S T S of t h e h a n d or w r i s t w i t h a m i n i m u m f o l l o w - interosseous flaps), a s a free fasciocutaneous flap
u p of 1 y e a r (7.6 p e r c e n t ) . O f t h e s e , 16 w e r e m a l e s in f o u r c a s e s ( t w o r a d i a l f o r e a r m , t w o lateral a r m
a n d t e n f e m a l e s , w i t h a n a v e r a g e a g e of 32 y e a r s flaps), a n d a s a free c o m p o s i t e dorsalis pedis flap
(age r a n g e 8-72). A t histology the lesion w a s a (three cases).
s y n o v i a l s a r c o m a in 11 c a s e s a n d a malignant Complications requiring minor revision
s c h w a n n o m a in t h r e e c a s e s . T h e r e w e r e t w o c a s e s s u r g e r y o c c u r r e d in f o u r c a s e s (15 p e r c e n t ) . T h e
e a c h of clear-cell s a r c o m a , e p i t h e l o i d sarcoma, r e v i s i o n of a f r e e f l a p a n a s t o m o s i s d u e t o v e n o u s
m a l i g n a n t f i b r o u s h i s t i o c y t o m a , a n d extraskeletal t h r o m b o s i s w a s n e c e s s a r y in o n e c a s e .
E w i n g ' s s a r c o m a , a n d o n e c a s e e a c h of rhab- A d j u v a n t radiation t h e r a p y w a s performed in
domyosarcoma, fibrosarcoma, liposarcoma, and 14 c a s e s (54 p e r c e n t ) a n d t e n of t h e m r e c e i v e d a
dermatofibrosarcoma protuberans. All but two combined treatment with brachytherapy and
tumors (liposarcoma and dermatofibrosarcoma e x t e r n a l r a d i a t i o n w i t h a n a v e r a g e total d o s e of
protuberans) were high-grade sarcomas. The 65 G y (47-75 G y ) . Neither w o u n d healing d e l a y s ,
d i a m e t e r of t h e t u m o r w a s less t h a n 5 c m in 85 per nor any other major complication w e r e o b s e r v e d .
c e n t of c a s e s , a s w o u l d b e e x p e c t e d in h a n d a n d T w o patients affected by extraskeletal E w i n g ' s
wrist S T S , a n d the lesion h a d an extra-compart- sarcoma a n d o n e patient affected by rhabdo-
m e n t a l l o c a t i o n in 2 1 c a s e s a n d w a s i n t r a c o m p a r t - myosarcoma underwent chemotherapy following
m e n t a l ( o n e digital r a y ) in f i v e c a s e s . W i t h o n e a standard protocol for these diseases. One
e x c e p t i o n , all p a t i e n t s (96 p e r c e n t ) h a d a l r e a d y patient underwent an adjuvant chemotherapy
b e e n t r e a t e d e l s e w h e r e a n d h a d p r e s e n t e d for a p r o t o c o l for h i g h - g r a d e S T S ( F r u s t a c i e t al 2001)
local r e c u r r e n c e in f o u r c a s e s o r for s u r g i c a l radi- with epirubicin a n d high-dose ifosfamide.
calization after i n a d e q u a t e t r e a t m e n t in 21 c a s e s .
T h e s u r g i c a l t r e a t m e n t r e q u i r e d a m p u t a t i o n in
s e v e n c a s e s (27 p e r c e n t ) . In o n e c a s e it w a s a Results
f o r e a r m a m p u t a t i o n ; in six c a s e s (23 p e r c e n t ) a
single digital ray amputation was performed At an a v e r a g e follow-up of 54 m o n t h s (range
(third r a y in t w o c a s e s ; t h u m b , s e c o n d , f o u r t h , 12-110) 20 patients (77 per cent) w e r e c o n t i n u o u s l y

a n d fifth r a y in o n e c a s e e a c h ) . T h u m b a m p u t a t i o n d i s e a s e f r e e , t h r e e p a t i e n t s w e r e d i s e a s e f r e e after

w a s f o l l o w e d b y a pollicization of t h e i n d e x finger. a n e w t r e a t m e n t for t u m o r r e c u r r e n c e , a n d t h r e e

Limb-sparing procedures were possible in patients h a d d i e d of t h e d i s e a s e . L o c a l r e c u r r e n c e s
19 c a s e s (73 p e r c e n t ) . T h e r e s e c t i o n involved w e r e t r e a t e d w i t h c o n s e r v a t i v e s u r g e r y in one
b o n e s e g m e n t s in f o u r c a s e s : distal radius in c a s e a n d w i t h a n a m p u t a t i o n in t w o c a s e s .
t h r e e c a s e s a n d t h e first m e t a c a r p a l b o n e in o n e Functional results evaluated following the
c a s e . A n osteo-articular m a s s i v e allograft was Musculo-SkeletalTumor S o c i e t y s y s t e m w e r e sat-
u s e d f o r d i s t a l r a d i u s r e c o n s t r u c t i o n in o n e c a s e i s f a c t o r y in 22 p a t i e n t s ( 8 5 p e r c e n t ) , t e n of w h o m
a n d a c o m p o s i t e dorsalis pedis flap including the w e r e e x c e l l e n t (38 p e r c e n t ) , a n d 12 g o o d (46 p e r
extensor tendon and the second metatarsal w a s cent). Four patients w e r e considered unsatisfac-

e m p l o y e d t o r e p l a c e t h e first m e t a c a r p a l b o n e . t o r y (15 p e r c e n t ) , w i t h o n e fair a n d t h r e e p o o r

N o r e c o n s t r u c t i o n w a s r e q u i r e d in t h e r e m a i n i n g results. In six c a s e s t r e a t e d w i t h digital r a y a m p u -

t w o c a s e s , w h e r e o n l y a partial r e s e c t i o n of t h e tation t h e functional result, also considering their
distal r a d i u s w a s p e r f o r m e d . In t h r e e c a s e s t h e emotional acceptance, w a s considered as good.
extensor tendons were reconstructed using
multiple flexor digitorum superficialis ( F D S )
t r a n s f e r s . A r e s e c t i o n of t h e m e d i a n n e r v e w a s Discussion
n e c e s s a r y in t w o c a s e s , t h e u l n a r n e r v e in o n e
c a s e , a n d t h e u l n a r digital n e r v e of t h e t h u m b at T h e m a i n p r o g n o s t i c f a c t o r s of t h e o u t c o m e of
t h e t h e n a r e m i n e n c e in o n e c a s e ; in all c a s e s t h e STS are: the histological g r a d e of malignancy
 S O F T T I S S U E SARCOMA OF THE HAND AND W R I S T 171

Figure 3

Radicalization after inadequate excision of a fibrosarcoma

of the thenar eminence in the left hand of a 37-year-old
patient, (a) Repeat excision includes the scar of the previ-
ous operation and the thenar muscles. (b) Reconstruction
was achieved by means of an abductor digiti minimi muscle
transfer, covered with a radial forearm flap. !c and d)
c d Functional result at 3 years.

( l o w - v e r s u s h i g h - g r a d e ) , t h e l o c a t i o n in r e s p e c t excision or e v e n r e m o v e d piece by piece, leaving

to the superficial fascia (superficial v e r s u s d e e p ) , t h e c o r r e c t d i a g n o s i s of s a r c o m a t o t h e f o l l o w i n g
a n d t h e size of t h e l e s i o n (< 5 c m v e r s u s > 5 c m ) h i s t o l o p a t h o l o g i c e v a l u a t i o n . It s h o u l d b e s t r e s s e d
( G u s t a f s o n 1994). that an excisional biopsy should only be reserved
A s f o r t h e i r a n a t o m i c s i t e , S T S of t h e h a n d a n d for s m a l l l e s i o n s (< 2 c m ) w h i c h a r e e a s i l y r e m o v e d
w r i s t a r e u s u a l l y d e e p l y l o c a t e d , e x c e p t for s o m e w i t h s a f e m a r g i n s a n d w i t h o u t t h e s a c r i f i c e of
r a r e l e s i o n s of t h e skin (dermatofibrosarcoma important anatomic structures (tendons, vessels,
protuberans). Moreover, they are m o r e frequently n e r v e s , or b o n e ) .
in h i g h - g r a d e l e s i o n s ; in o u r s e r i e s t h e l e s i o n w a s As expected, most of the tumors had an
a high-grade tumor in 92 p e r c e n t of c a s e s . In e x t r a c o m p a r t m e n t a l e x t e n s i o n (81 p e r c e n t ) but,
m o r e p r o x i m a l s i t e s , t h e i r size at t h e first o b s e r - nevertheless, conservative treatment w a s pos-
v a t i o n is c o m m o n l y l a r g e r t h a n 5 c m in d i a m e t e r . s i b l e in 72 p e r c e n t of o u r c a s e s , u s i n g different
A t t h e h a n d o r w r i s t t h i s is a rarer c o n d i t i o n ; in reconstructive techniques.
o u r s e r i e s , 15 p e r c e n t of p a t i e n t s h a d a t u m o r at S T S r e s e c t i o n in t h e h a n d a n d w r i s t f r e q u e n t l y
t h e h a n d o r w r i s t l a r g e r t h a n 5 c m in size. i n c l u d e s a w i d e a r e a of skin w h i c h is r e m o v e d in
It is n o t e w o r t h y t h a t a l m o s t all t h e p a t i e n t s of o r d e r t o a c h i e v e a s a f e s u r g i c a l m a r g i n . T h i s is
t h e p r e s e n t s e r i e s h a d a l r e a d y u n d e r g o n e a for- particularly common in secondary procedures
m e r s u r g i c a l t r e a t m e n t . In f a c t , a soft t i s s u e m a s s w h e r e radicalization of a f o r m e r i n a d e q u a t e e x c i -
n o t e d in t h e distal part of a l i m b is often u n d e r - s i o n is p e r f o r m e d i n c l u d i n g t h e existing s c a r or
estimated and inappropriately treated. It is t h e b i o p s y tract ( F i g . 3). In t h e p r e s e n t s e r i e s c u t a -
consequently approached as a simple marginal n e o u s r e c o n s t r u c t i o n s w i t h a n i s l a n d of f r e e soft
172 T U M O R S OF THE HAND

t i s s u e f l a p w e r e r e q u i r e d in 58 p e r c e n t of c a s e s . T h r e e c a s e s h a d a local r e c u r r e n c e : t w o u n d e r w e n t
In s o m e i n s t a n c e s , c o m p o s i t e f r e e f l a p s i n c l u d i n g an amputation a n d o n e had a limb-sparing repeat
s k i n , f a s c i a , t e n d o n s , n e r v e s , v e s s e l s , or bone r e s e c t i o n . T o d a t e , all a r e d i s e a s e f r e e .
m a y b e e m p l o y e d t o r e s t o r e f u n c t i o n ( C e r u s o et al M o r e t h a n half of t h e p a t i e n t s r e c e i v e d a d j u -
1995, C a p a n n a a n d C a m p a n a c c i 2002). In t h r e e v a n t r a d i a t i o n t h e r a p y d e s p i t e t h e difficulties of
c a s e s of o u r s e r i e s a c o m p o s i t e f r e e f l a p a l l o w e d a p p l y i n g b r a c h y t h e r a p y to t h e distal u p p e r l i m b .
t h e s i m u l t a n e o u s r e p l a c e m e n t of s k i n , t e n d o n s , N o complications d u e to radiation therapy h a v e
and bone. Tendons are resected w h e n e v e r they b e e n o b s e r v e d to date.
a r e in strict r e l a t i o n s h i p w i t h t h e t u m o r ; t h e y c a n In c o n c l u s i o n , a w i d e m a r g i n of e x c i s i o n in
be repaired by tendon transfers, free tendon association with adjuvant radiotherapy offers
grafts, o r b y m e a n s of c o m p o s i t e f l a p s i n c l u d i n g a d e q u a t e local c o n t r o l a n d survival rates c o m -
v a s c u l a r i z e d t e n d o n grafts ( e . g . d o r s a l i s p e d i s o r p a r a b l e to t h o s e o b t a i n e d w i t h a r a d i c a l m a r g i n .
radial f o r e a r m flap). M i c r o s u r g e r y i n c r e a s e s t h e p o s s i b i l i t y of a p p l y -
If m a j o r n e r v e s a r e c l o s e b u t n o t infiltrated b y ing non-demolitive limb-sparing procedures to
the tumor, they c a n be spared by dissecting the t h e t r e a t m e n t of S T S of t h e l i m b s . C o n v e r s e l y ,
n e r v e t r u n k f r o m its p e r i n e u r i u m , w h i c h is left t h e s y s t e m i c c o n t r o l of S T S still r e m a i n s a critical
a t t a c h e d to t h e p s e u d o c a p s u l e of t h e t u m o r a n d i s s u e . In o u r i n s t i t u t i o n , a p o s t o p e r a t i v e c h e m o -
e x c i s e d . S h o u l d t h e n e r v e b e w r a p p e d into t h e t h e r a p e u t i c protocol w i t h epirubicin a n d i f o s f a m i d e
tumor mass, it m u s t be resected and can be is r e s e r v e d for h i g h - g r a d e s a r c o m a s , larger t h a n
r e c o n s t r u c t e d w i t h m u l t i p l e n e r v e grafts f r o m t h e 5 c m in size. A p r e o p e r a t i v e c h e m o t h e r a p e u t i c a n d
sural nerve. radiotherapeutic t r e a t m e n t is p e r f o r m e d in locally
If t h e t u m o r is a d j a c e n t to b o n e , a p r e o p e r a t i v e a d v a n c e d t u m o r s w h i c h w o u l d require d e m o l i t i v e
b o n e s c a n is r e c o m m e n d e d . W h e n t h e b o n e s c a n surgery.
is n e g a t i v e a n d t h e m a s s is c l i n i c a l l y a n d intra-
o p e r a t i v e l y m o b i l e in r e s p e c t to t h e b o n e , a s a f e
m a r g i n is u s u a l l y a c h i e v e d r e m o v i n g t h e p e r i o s -
t e u m together with the tumor. S h o u l d the bone References
s c a n b e p o s i t i v e , o r r a d i o g r a p h i c s i g n s of e r o s i o n
or infiltration of the cortex noted, the bone Alekheteyar K M , Leung D M , Brennan MF, Harrison L B
s e g m e n t m u s t be resected. T h e skeletal g a p m a y (1996)The effect of combined external beam radiother-
apy and brachytherapy on local control and wound
t h a n b e r e c o n s t r u c t e d u s i n g e i t h e r a n allograft o r
complications in patients with high grade soft tissue
non-vascularized or vascularized autografts,
sarcoma of the extremities with positive microscopic
depending on the length of reconstruction
margins, Int J Radiat Oncol Biol Phys 36:321.
n e e d e d a n d o n t h e n e c e s s i t y of a n a s s o c i a t e d
articular r e c o n s t r u c t i o n . Besser E, Roessner A , Brug E et al (1987) Bone and soft
Finally, the sacrifice of one of the major tissue tumors of the hand, Arch Orthop Traum Surg
v a s c u l a r b u n d l e s of t h e f o r e a r m is u s u a l l y w e l l 106:241-7.
compensated by the residual vessels through
distal a n a s t o m o t i c p a t h w a y s . T h e s e m u s t in a n y Brien E W , Terek R M , Geer R J et al (1995) Treatment of
c a s e be checked with a preoperative Allen's test soft-tissue sarcomas of the hand, J Bone Joint Surg Am
and a color-Doppler evaluation. 77:564-671.
Although complex reconstructions were
Campanacci M (1999) Bone and Soft Tissue Tumors,
r e q u i r e d in m o s t of t h e c a s e s , t h e o v e r a l l f u n c -
2nd e d n . Springer-Verlag - Piccin Nuova Libraría:
t i o n a l r e s u l t s w e r e s a t i s f a c t o r y in 8 5 p e r c e n t o f
Padova, Italy.
the cases, according to the Musculo-Skeletal
Tumor Society system. Conservative surgery w a s
Capanna R, Campanacci DA (2002) Management and
not p o s s i b l e in six p a t i e n t s a n d a n a m p u t a t i o n surgery of soft tissue tumors. In: Orthopaedics. Mosby:
had to be performed: o n e forearm amputation, St Louis: 1070-7.
e n d i n g in a p o o r f u n c t i o n a l a n d c o s m e t i c result,
a n d f i v e c a s e s of digital r a y a m p u t a t i o n . A t a n Ceruso M , Angeloni R, Innocenti M et al (1995)
a v e r a g e f o l l o w - u p of 5 y e a r s l o c a l c o n t r o l of t h e Reconstruction des pertes de substance des parties
t u m o r w a s a c h i e v e d in 88.5 p e r c e n t of c a s e s . molles du membre supérieur après résection tumorale
 S O F T T I S S U E SARCOMA O F T H E HAND AND W R I S T 173

par lambeaux vascularisés ou en îlot, Ann Chir Main J o h n s o n J , Kilgore E, Newmeyer W (1985) Tumorous
14:21-7. lesions of the hand, J Hand Surg Am 10:284-6.

Cheng EY, Dusembery K E , Winters M R , Thompson RC M c P h e e M , McGrath B E , Zhang P et al (1999) Soft tissue
(1996) Soft tissue sarcoma: preoperative vs postopera- sarcoma of the hand, J Hand Surg (Am) 24:1001-7.
tive radiotherapy, J Surg Oncol 61:90.
Pisters P W (1998) Combined modality treatment of
Enneking W F (1986) A system of staging of muscu- extremity soft tissue sarcomas, Ann Surg Oncol
loskeletal neoplasm, Clin Orthop 204:9-24. 5:464-72.

Enzinger F M , Weiss S W (1995) Soft Tissue Tumors, Talbert M L , Zagars G K , S h e r m a n NE et al (1990)

3rd edn. Mosby: S t Louis. Conservative surgery and radiation therapy for soft
tissue sarcoma of the wrist, hand, ankle and foot,
Frustaci S , Gherlinzoni F, De Paoli A et al (2001 ) Adjuvant Cancer 66:2482-91.
chemotherapy for adult soft tissue sarcomas of the
extremities and girdles: results of the Italian Tierney J F , Mosseri V, Stewart LA et al (1995) Adjuvant
Cooperative Trial, J Clin Oncol 19:1238-47. chemotherapy for soft tissue sarcoma: review and
meta-analysis of the published results of randomised
Gustafson P (1994) Soft tissue sarcoma, epidemiology clinical trials, Br J Cancer 72:469.
and prognosis in 508 patients, Acta Orthop Scand
65(Suppl 259):2-31.
19
Objectives and results of
the Cooperative Osteosarcoma
Study Group
Stefan S Bielack and Silke Flege

Introduction frequent primary bone cancer, with a n approximate

a n n u a l i n c i d e n c e o f 2 - 3 p e r m i l l i o n in t h e g e n e r a l

The successful treatment of malignant bone population. Its i n c i d e n c e p e a k s in a d o l e s c e n c e ,

t u m o r s , of w h i c h o s t e o s a r c o m a is t h e m o s t fre- w h e r e it a c c o u n t s for m o r e t h a n 10 p e r c e n t of all

quent, requires close co-operation within a n expe- solid m a l i g n a n c i e s . A s m a l l e r p e a k in o l d e r patients

r i e n c e d interdisciplinary t e a m . Neither surgeons is d u e t o o s t e o s a r c o m a s arising in abnormal

nor oncologists alone can e v e n h o p e to achieve b o n e s , s u c h a s in P a g e t ' s d i s e a s e after prior r a d i o -

c u r e in a significant n u m b e r of p a t i e n t s . T o g e t h e r , t h e r a p y . T h e p r i m a r y t u m o r is m o s t often l o c a t e d

however, and with the additional expertise of in t h e m e t a p h y s i s of a l o n g e x t r e m i t y b o n e , m o s t

o t h e r specialists s u c h a s radiologists a n d patholo- c o m m o n l y t h e distal f e m u r o r t h e p r o x i m a l tibia.

gists, s u r g e o n s a n d o n c o l o g i s t s c a n h e a l up to t w o - Approximately 15 p e r c e n t of all o s t e o s a r c o m a s

thirds of patients suffering f r o m t h e s e o t h e r w i s e a r i s e in t h e a r m , m a i n l y in t h e p r o x i m a l h u m e r u s ,

rapidly lethal c a n c e r s . A s o s t e o s a r c o m a s a r e r a r e , w h i l e t h e distal h u m e r u s , f o r e a r m , a n d h a n d a r e

progress c a n o n l y be a c h i e v e d by c l o s e co-operation o n l y r a r e l y a f f e c t e d ( F i g . 1). T u m o r s of t h e axial

between many institutions w i t h i n collaborative skeleton or craniofacial bones almost exclusively

g r o u p s . T h e interdisciplinary a n d multi-institutional o c c u r in o l d e r p a t i e n t s .

s u c c e s s s t o r y of o s t e o s a r c o m a t h e r a p y h a s t h u s Patients with osteosarcoma typically present

b e c o m e a m o d e l for m a n y other cancers. This w i t h p a i n of t h e i n v o l v e d r e g i o n , w h i c h is often
chapter will describe the experience of the e r r o n e o u s l y a t t r i b u t e d to t r a u m a . T u m o r - r e l a t e d
Cooperative Osteosarcoma Study Group ( C O S S ) , s w e l l i n g a n d l o s s of f u n c t i o n of a d j a c e n t j o i n t s
an experience gathered in German-speaking u s u a l l y d e v e l o p later. T h e first s i g n of d i s e a s e
countries, spanning more than two a n d a half m a y be a pathologic fracture. S y s t e m i c s y m p -
d e c a d e s a n d including several t h o u s a n d patients. t o m s a r e r a r e in t h e a b s e n c e of v e r y a d v a n c e d
d i s e a s e . T h e differential d i a g n o s i s of o s t e o s a r -
coma must include trauma, osteomyelitis, benign
bone tumors such as exostosis, fibroma, osteoid-

Epidemiology, clinical signs, o s t e o m a , c h o n d r o m a , g i a n t cell t u m o r o f b o n e ,

b o n e cysts, etc., a s w e l l a s other b o n e c a n c e r s
and diagnostic evaluation of such as E w i n g s a r c o m a or l y m p h o m a , and b o n e
osteosarcoma metastases.
Most osteosarcomas are high-grade malignan-
Osteosarcoma affects m a l e s a p p r o x i m a t e l y 1.4 cies arising within b o n e a n d this chapter will
t i m e s m o r e often t h a n f e m a l e s . It is t h e most focus on these 'high-grade central' tumors.
176 T U M O R S OF THE HAND

Clavicle 2 - 0 . 1 %

Scapula 6 - 0.4%

proximal 1 6 9 - 9 . 9 %

Humerus
diaphysis 1 - < 0 . 1 % -
172-10.1%

Ulna 6 - 0.4%

Radius 1 7 - 1 . 0 %

Hand 1 - < 0 . 1 %

Figure 1

Distribution of high-grade osteosarcomas of the hand and arm among 1702 tumors in patients enrolled in the Cooperative
Osteosarcoma Study Group studies (Bielack et al 2002a).

R e a d e r s i n t e r e s t e d in v a r i a n t s s u c h a s l o w - g r a d e isolated tumor foci within the same bone as

central osteosarcoma, parosteal or periosteal t h e p r i m a r y t u m o r . D e a t h f r o m o s t e o s a r c o m a is
o s t e o s a r c o m a , or high-grade surface osteosar- usually due to respiratory failure caused by
coma should refer to the relevant literature extensive pulmonary involvement.
( F l e t c h e r et al 2 0 0 2 , S o b i n a n d W i t t e k i n d 2002). The evaluation of suspected osteosarcoma
High-grade osteosarcomas metastasize fre- m u s t i n c l u d e a c c u r a t e d e s c r i p t i o n of t h e e x t e n t of
q u e n t l y , y e t , at d i a g n o s i s , e v e n t h e m o s t a c c u r a t e the primary tumor as well as adequate assess-
s t a g i n g p r o c e d u r e s w i l l d e t e c t m e t a s t a s e s in n o m e n t of t h e m e t a s t a t i c s t a t u s . P h y s i c a l e x a m i n a -
m o r e t h a n 10-20 p e r c e n t of p a t i e n t s . If n o s y s - t i o n m a y r e v e a l a m a s s at t h e p r i m a r y site. L o s s
t e m i c t r e a t m e n t is g i v e n , h o w e v e r , m o s t p a t i e n t s of m o t i o n of n e i g h b o r i n g j o i n t s , infiltration of t h e
with seemingly localized disease will develop skin, a n d neurologic deficits may be present,
m e t a c h r o n o u s m e t a s t a s e s w i t h i n 1-2 y e a r s (Link depending on the location and extent of the
et al 1986), implying that in most patients tumor. T h e r e are no k n o w n specific laboratory
micrometastatic d i s e a s e is a l r e a d y present at p a r a m e t e r s , but i n c r e a s e d a l k a l i n e p h o s p h a t a s e
diagnosis. Primary as well as metachronous values m a y h a v e negative prognostic significance.
osteosarcoma metastases usually involve the Plain radiographs are used to describe tumor-
l u n g s or, l e s s f r e q u e n t l y , distant b o n e s ( K a g e r et al a s s o c i a t e d c h a n g e s of b o n e . O s t e o s a r c o m a c a n
2003). S o m e p a t i e n t s w i l l h a v e skip m e t a s t a s e s , induce lytic or sclerotic c h a n g e s , or both.
 O B J E C T I V E S AND RESULTS OF THE COSS STUDIES 177

Ossification in t h e soft tissue in a radial or ' s u n b u r s t ' c a n b e n e f i t f r o m m o d e r n , e f f i c a c i o u s interdiscipli-

p a t t e r n is t y p i c a l , but n e i t h e r s e n s i t i v e nor s p e c i - nary therapeutic regimens.
fic. P e r i o s t e a l b o n e f o r m a t i o n w i t h lifting of t h e
c o r t e x m a y l e a d to a ' C o d m a n triangle'. M a g n e t i c
r e s o n a n c e i m a g i n g ( M R I ) is u s e d to d e f i n e t h e
i n t r a m e d u l l a r y t u m o r e x t e n t , soft t i s s u e c o m p o -
The Cooperative Osteosarcoma
n e n t , a n d relation of t h e t u m o r to v e s s e l s a n d Study Group
n e r v e s ( S a n d e r s a n d P a r s o n s 2001). L u n g m e t a s -
tases a r e s e a r c h e d for by plain X - r a y s a n d a c o m - S i n c e t h e f o u n d a t i o n of t h e g r o u p b y Kurt W i n k l e r
puted tomography (CT)-scan of the thorax, et al in 1977, t h e r a p y of o s t e o s a r c o m a patients f r o m
p r e f e r a b l y h i g h r e s o l u t i o n s p i r a l CT, b o n e m e t a s - Germany, Austria, and Switzerland has usually
tases by a 9 9 m T c - M D P b o n e scan. Skip metas- b e e n p e r f o r m e d a c c o r d i n g to protocols d e v e l o p e d
tases m a y also be visualized o n the b o n e s c a n , b y C O S S ( W i n k l e r et al 1984,1988,1990, F u c h s et al
b u t M R I of t h e w h o l e b o n e is p r e f e r a b l e b e c a u s e 1998, Bielack et al 1999,2002a). For m a n y y e a r s , t h e
of its h i g h e r s e n s i t i v i t y . g r o u p ' s studies h a v e b e e n g e n e r o u s l y s u p p o r t e d
W h i l e i m a g i n g w i l l often result in a h i g h i n d e x b y ' D e u t s c h e Krebshilfe'. R e c r u i t m e n t h a s n e v e r
of s u s p i c i o n , t h e d i a g n o s i s of o s t e o s a r c o m a m u s t b e e n limited t o t h e typical y o u n g patients w i t h
always be verified histologically. In o r d e r to localized limb tumors, rather, all patients with
ensure appropriate biopsy techniques and an o s t e o s a r c o m a a r e eligible for registration. It is t h e
a p p r o p r i a t e e v a l u a t i o n of t h e o b t a i n e d m a t e r i a l , it a i m of t h e g r o u p to recruit n a t i o n w i d e a n d h e n c e to
is s t r o n g l y r e c o m m e n d e d t h a t b i o p s i e s s h o u l d b e contribute to i m p r o v e d s t a n d a r d s of c a r e (Bielack et
performed only in specialized centers. Open al 2002b). A c c o r d i n g to G e r m a n Pediatric C a n c e r
biopsy is best suited to obtaining sufficient Registry data, t h e recruitment of pediatric o s t e o s a r -
material for histological evaluation a n d ancillary c o m a patients into C O S S studies h a s b e e n m o r e
studies.The biopsy material should be forwarded or less c o m p l e t e for m a n y y e a r s ( K a a t s c h et al
to the pathologist unfixed. 1999). In addition to t h e pediatric p o p u l a t i o n , m a n y
affected adults have also been registered.
Altogether, t h e g r o u p h a s , s o far, recruited approx-
imately 3000 patients with osteosarcomas and
Rationale for interdisciplinary, related m a l i g n a n c i e s , s u c h a s m a l i g n a n t fibrous
multicenter trials histiocytoma of b o n e . To e n s u r e a u n i f o r m l y high
standard of care despite multiple institutions,
A s mentioned above, only approximately 10 p e r C O S S , a s w e l l a s t h e other pediatric o n c o l o g y trial
c e n t of p a t i e n t s e v e n w i t h s e e m i n g l y localized g r o u p s of t h e S o c i e t y of Pediatric O n c o l o g y a n d
o s t e o s a r c o m a s w i l l s u r v i v e if t r e a t e d b y s u r g e r y H e m a t o l o g y of G e r m a n - s p e a k i n g c o u n t r i e s , per-
a l o n e ( L i n k et al 1986). A d r a m a t i c c h a n g e for t h e f o r m a task that e x t e n d s b e y o n d data collection a n d
better h a s t a k e n p l a c e o n l y w i t h i n t h e p a s t t h r e e h a n d l i n g . Participating institutions c a n rely o n p a n -
decades, w h e n chemotherapy w a s included in els of experts including o n c o l o g i s t s , radiologists,
interdisciplinary therapy. Today, approximately pathologists, o r t h o p e d i c s u r g e o n s , a n d m e m b e r s
60 p e r c e n t of all p a t i e n t s w i t h o s t e o s a r c o m a of other s u b s p e c i a l t i e s to offer c o u n s e l a n d a s s i s -
a n d m o r e t h a n t w o - t h i r d s o f t h o s e w i t h localized t a n c e in all a s p e c t s of local a n d s y s t e m i c therapy.
extremity t u m o r s can achieve long-term survival T h i s structure g u a r a n t e e s that a d v i c e b a s e d o n t h e
w i t h i n t e n s i v e m u l t i m o d a l t r e a t m e n t ( B i e l a c k et al c u m u l a t i v e e x p e r i e n c e a n d expertise of t h e r e s p e c -
2 0 0 2 a ) . H o w e v e r , i n a p p r o p r i a t e u s e of d i a g n o s t i c t i v e g r o u p is a v a i l a b l e for e a c h a n d e v e r y individual
t o o l s a n d s u b o p t i m a l initial t h e r a p y c a n still irre- patient (Bielack et al 2002b).
vocably compromise survival chances. Therefore,
all o s t e o s a r c o m a s s h o u l d b e t r e a t e d in s p e c i a l -
ized c e n t e r s a b l e to p r o v i d e a c c e s s t o t h e full
s p e c t r u m of d i a g n o s t i c s a n d t h e r a p y . It is s t a n - Treatment strategy
d a r d clinical p r a c t i c e in m a n y c o u n t r i e s t o treat in the COSS trials
a f f e c t e d p a t i e n t s w i t h i n p r o s p e c t i v e clinical trials.
T h e s e multicenter trials h a v e b e e n e s s e n t i a l in A u n i f o r m t r e a t m e n t c o n c e p t of p r e o p e r a t i v e a n d
guaranteeing that a s m a n y patients a s possible p o s t o p e r a t i v e c h e m o t h e r a p y in c o m b i n a t i o n w i t h
178 T U M O R S OF THE HAND

t h e m a j o r i t y of c a s e s ( F i g . 3 ) , but a m p u t a t i o n m a y
Osteosarcoma
still b e r e q u i r e d if a p p r o p r i a t e m a r g i n s c a n n o t b e
Therapeutic strategy achieved otherwise.
The surgical margins are classified according
Imaging/biopsy to the Musculoskeletal Tumor Society (MSTS)
classification, w h i c h distinguishes b e t w e e n radi-
cal, w i d e , marginal, a n d intralesional resection
Neoadjuvant chemotherapy m a r g i n s ( E n n e k i n g et al 1980). O s t e o s a r c o m a s
must be r e m o v e d with ' w i d e ' margins a s defined
by the M S T S , m e a n i n g that the c o m p l e t e t u m o r
Definitive surgery i n c l u d i n g b i o p s y s c a r a n d track a r e s u r r o u n d e d b y
a n u n v i o l a t e d cuff of n o r m a l t i s s u e . R a d i c a l m a r -
g i n s , m e a n i n g c o m p l e t e r e m o v a l of all a f f e c t e d
Adjuvant chemotherapy
a n a t o m i c c o m p a r t m e n t s , a r e u s u a l l y not n e c e s -
Figure 2
sary, and marginal or e v e n intralesional margins
General strategy of osteosarcoma therapy in the are i n a d e q u a t e ( P i c c i et al 1994). T h e t y p e of
Cooperative Osteosarcoma Study Group trials. surgery n e e d e d to a c h i e v e w i d e m a r g i n s v a r i e s
w i t h t h e o s t e o s a r c o m a ' s site a n d l o c a l e x t e n t a n d
the regional anatomy. W h i l e 'en bloc' resection
w i t h l i m b s a l v a g e is often f e a s i b l e , a m p u t a t i o n is
a g g r e s s i v e s u r g e r y h a s f o r m e d t h e b a s i s of all still r e q u i r e d in s o m e c a s e s . A r e v i e w of 504
COSS p r o t o c o l s s i n c e 1980 ( W i n k l e r et al 1984, extremity osteosarcomas from the COSS trial
1988, 1990, F u c h s et a l 1998, B i e l a c k et al 1999, ( B i e l a c k et al 1996a) a s w e l l a s s e v e r a l o t h e r p u b -
2002a) ( F i g . 2). P o s t p o n i n g d e f i n i t i v e s u r g e r y until lications report local recurrence rates w h i c h a r e
after a 2 - 3 - m o n t h p e r i o d of p r e o p e r a t i v e ' i n d u c - approximately three times higher than after
tion' chemotherapy allows early treatment of amputations, indicating that the resection mar-
micrometastatic d i s e a s e a n d facilitates the surgi- gins achieved with limb s a l v a g e procedures m a y
cal p r o c e d u r e b e c a u s e of t u m o r s h r i n k a g e a n d s o m e t i m e s not b e a s w i d e a s e x p e c t e d .
decreased vascularity, thereby allowing limb- Surgery with inadequate surgical margins
s a l v a g e in a l a r g e r p r o p o r t i o n of p a t i e n t s t h a n is frequently followed by local recurrences.
w o u l d be the case with immediate surgery. A l s o , P a r t i c u l a r c a r e m u s t b e t a k e n in t u m o r s w i t h p o o r
r e s p o n s e of t h e t u m o r to i n d u c t i o n c h e m o t h e r a p y response to preoperative induction chemo-
can be evaluated by imaging a n d histology, a n d t h e r a p y , a s t h i s is a s s o c i a t e d w i t h a n e x c e s s i v e
g i v e s a n in v i v o a s s e s s m e n t of t h e e f f e c t i v e n e s s l o c a l f a i l u r e rate in c a s e s of i n a d e q u a t e m a r g i n s
of t h e r a p y . ( P i c c i e t al 1994, B i e l a c k e t al 1996a) ( s e e b e l o w ) .
Local r e c u r r e n c e s in turn are almost always
associated with uncontrollable systemic tumor
p r o g r e s s i o n a n d d e a t h ( P i c c i e t a l 1994, B i e l a c k
Osteosarcoma surgery et al 1996a). T h e r e f o r e , c o m p l e t e t u m o r removal
is of utmost importance, w h i l e functional and
As c h e m o t h e r a p y a l o n e c a n n o t reliably control cosmetic aspects can only be secondary goals.
c l i n i c a l l y d e t e c t a b l e o s t e o s a r c o m a , s u r g e r y of t h e If p a t h o l o g i c evaluation of the resected tumor
primary tumor a n d , if p r e s e n t , all m e t a s t a s e s s p e c i m e n r e v e a l s i n a d e q u a t e ( m a r g i n a l or intra-
r e m a i n s a n integral part of s u c c e s s f u l t h e r a p y . l e s i o n a l ) m a r g i n s , t h i s is u s u a l l y a n i n d i c a t i o n f o r
The C O S S group includes a surgical panel which r e v i s i o n s u r g e r y , e v e n if t h i s i m p l i e s s e v e r e m u t i -
h a s f o r m u l a t e d g u i d e l i n e s for s u c h s u r g e r y a n d is lation. O s t e o s a r c o m a patients generally h a v e a
a v a i l a b l e for g u i d a n c e in i n d i v i d u a l cases. The s i n g l e c h a n c e to b e c u r e d . T h e r e is a b s o l u t e l y n o
a i m s of s u r g e r y m u s t b e t o r e m o v e all t u m o r , r o o m for m i s t a k e s b y i n e x p e r i e n c e d s u r g e o n s ( o r
leave the patient w i t h g o o d extremity function, oncologists) and operative procedures should
a n d , if p o s s i b l e , result in a g o o d c o s m e t i c a p p e a r - therefore exclusively be performed by specialized
a n c e . L i m b - s a l v a g e t e c h n i q u e s a r e n o w u s e d for teams.
 O B J E C T I V E S AND RESULTS OF THE C O S S STUDIES 179

o>
3

Figure 3

Distribution of surgical procedures in the Cooperative Osteosarcoma Study Group trials 1980-1997. Note the dramatic
increase of limb-salvage procedures.

Chemotherapy in the COSS trials r e d u c t a s e , w h i c h is r e p o n s i b l e for r e d u c i n g f o l a t e

t o its a c t i v e f o r m . In t h e C O S S s t u d i e s a s w e l l a s
M o s t of t h e C O S S g r o u p ' s p r o t o c o l s h a v e b e e n o t h e r g r o u p s ' p r o t o c o l s , m e t h o t r e x a t e is a d m i n i s -
b a s e d o n t h e f o u r a g e n t s c u r r e n t l y b e l i e v e d to b e t e r e d at s u p r a l e t h a l d o s e s of 12 g / m . 2 Results
the most active against osteosarcoma, namely f r o m e a r l y C O S S trials s u g g e s t t h a t a s e r u m l e v e l
doxorubicin, cisplatin, high-dose methotrexate, of a b o v e 1000 u.mol/1 m a y b e n e e d e d for o p t i m a l
and i f o s f a m i d e . D o x o r u b i c i n w a s first i n t r o d u c e d e f f i c a c y ( G r a f et al 1994). H i g h - d o s e m e t h o t r e x a t e
into o s t e o s a r c o m a t r e a t m e n t in t h e 1970s a n d h a s t h e r a p y requires meticulous attention a n d exten-
r e m a i n e d a n integral part e v e r s i n c e ( S m i t h et al sive supportive m e a s u r e s , including hydration,
1991). G i v e n t h e t h r e a t of l o n g - t e r m a n t h r a c y c l i n e alkalinization o f t h e u r i n e , a n d a n t a g o n i z a t i o n b y
cardiotoxicity, doxorubicin administration to the antidote leucovorin, which must be adminis-
y o u n g p a t i e n t s w i t h a fairly h i g h c u r e rate is t r o u - t e r e d at d o s e s w h i c h a r e a d a p t e d to m e t h o t r e x a t e
b l e s o m e . High anthracycline peak s e r u m levels serum levels. Inadequate supportive care will
s e e m to b e a s s o c i a t e d w i t h i n c r e a s e d c a r d i o t o x i - i n e v i t a b l y result in s e v e r e l y d e l a y e d m e t h o t r e x -
city ( B i e l a c k et al 1989,1996b). C u r r e n t a n d r e c e n t ate c l e a r a n c e a n d l i f e - t h r e a t e n i n g toxicity, i n c l u d -
COSS p r o t o c o l s t h e r e f o r e c a l l for doxorubicin ing m y e l o s u p p r e s s i o n , r e n a l f a i l u r e , a n d s e v e r e
administration by continuous infusions, which gastrointestinal side-effects.
are associated with lower peak drug levels C i s p l a t i n , t h e t h i r d a g e n t i n c o r p o r a t e d in t h e
( B i e l a c k e t al 1989,1996b). S e q u e n t i a l C O S S s t u d - C O S S group's protocols as well as most multi-
ies s u g g e s t t h a t t h e r e is n o m a j o r l o s s of e f f i c a c y agent regimens w o r l d w i d e , requires supportive
(Bielack et al 1999), but no controled studies h y d r a t i o n in t h e f o r m of a f o r c e d d i u r e s i s . O t o -
evaluating w h e t h e r cardiotoxicity c a n be reduced and nephrotoxicity are dose-limiting toxicities.
w i t h o u t influencin g efficacy a g a i n s t o s t e o s a r c o m a Both can be reduced by administering the drug
h a v e been reported. by continuous infusions. C O S S has compared
Methotrexate is a f o l a t e antagonist which s h o r t - t e r m (5-h) i n f u s i o n s w i t h c o n t i n u o u s (72-h)
blocks t h e a c t i o n of t h e e n z y m e dihydrofolate i n f u s i o n s in trial C O S S - 9 1 / - 8 6 C a n d f o u n d s i m i l a r
180 T U M O R S OF THE HAND

anti-osteosarcoma activity ( B i e l a c k et al 1999). important prognostic factor. COSS and other

Intra-arterial c i s p l a t i n a d m i n i s t r a t i o n directly into g r o u p s u s u a l l y arbitrarily d e f i n e a g o o d r e s p o n s e
the artery supplying the t u m o r w a s investigated as less t h a n 10 p e r c e n t v i a b l e t u m o r in the
in t h e 1980s, but w a s later l a r g e l y abandoned r e s e c t e d s p e c i m e n after p r e o p e r a t i v e c h e m o t h e r -
w h e n a c o m p a r a t i v e C O S S trial c o u l d not d e m o n - apy. T h e 10-year s u r v i v a l rate after o s t e o s a r c o m a
strate e n h a n c e d antitumor effects c o m p a r e d to diagnosis w a s found to b e 73.4 per cent for
intravenous administration ( W i n k l e r e t a l 1990, patients w h o s e t u m o r s s h o w e d less t h a n 10 p e r
F u c h s e t al 1998). c e n t v i a b l e c e l l s , c o m p a r e d t o o n l y 47.2 per c e n t for
I f o s f a m i d e h a s b e e n part of C O S S protocols those with more. However, even a very moderate
s i n c e t h e mid-1980s ( W i n k l e r et al 1990, F u c h s et al r e s p o n s e w i t h 50 p e r c e n t o r m o r e v i a b l e t u m o r
1998, B i e l a c k et al 1999). S u p p o r t i v e m e a s u r e s n e c - cells r e m a i n i n g w a s still a s s o c i a t e d w i t h a better
e s s a r y to p r e v e n t h e m o r r h a g i c u r o p a t h y after ifos- p r o g n o s i s t h a n n o histological r e s p o n s e at all or
f a m i d e i n c l u d e h y d r a t i o n a n d t h e a d m i n i s t r a t i o n of n o c h e m o t h e r a p y ( B i e l a c k et al 2002a). In a d d i t i o n
m e s n a ( U r o m i t e x a n ® ) . I f o s f a m i d e m a y a l s o lead to to its p r e d i c t i v e v a l u e for m e t a s t a t i c r e c u r r e n c e ,
chronic renal tubular toxicity, t h e F a n c o n i s y n - p o o r r e s p o n s e to i n d u c t i o n c h e m o t h e r a p y is a l s o
d r o m e , a n d t o sterility. B a s e d o n t h e results of a s s o c i a t e d w i t h a n i n c r e a s e d risk of local recur-
s e q u e n t i a l trials, C O S S h a s a s s u m e d that t h e i n c l u - r e n c e (Picci et al 1994, B i e l a c k et al 1996a), w h i c h is
s i o n of i f o s f a m i d e in m u l t i a g e n t regimens w a s e x c e s s i v e if p o o r r e s p o n s e is a s s o c i a t e d w i t h i n a d -
a s s o c i a t e d w i t h i m p r o v e d e v e n t - f r e e s u r v i v a l rates e q u a t e ( m a r g i n a l or e v e n intralesional) margins
( F u c h s et ai 1998, B i e l a c k et al 1999). A recent r a n - (Picci et al 1994). M e t h o d s u s e d t o predict histolog-
d o m i z e d N o r t h A m e r i c a n trial, h o w e v e r , c o u l d not ical r e s p o n s e p r e o p e r a t i v e l y i n c l u d e serial e v a l u a -
d e m o n s t r a t e that t h e a d d i t i o n of s t a n d a r d d o s e tion by angiography, quantitative bone scans,
ifosfamide to a regimen of high-dose methotrex- d y n a m i c M R I , or positron emission tomography
ate, d o x o r u b i c i n , a n d cisplatin i m p r o v e d o u t c o m e ( P E T ) . If p e r f o r m e d a n d interpreted correctly, all of
( M e y e r s et al 2001). It is p l a n n e d to e v a l u a t e t h e t h e s e carry a rather high predictive v a l u e , but no
e x a c t role of i f o s f a m i d e in future i n t e r g r o u p trials. individual m e t h o d is 100 p e r c e n t a c c u r a t e a n d
t h e r e is h e n c e currently no universally accepted
gold standard.
E a r l y reports s u g g e s t e d t h a t t h e p o o r p r o g n o -
Prognostic factors sis a s s o c i a t e d w i t h p o o r r e s p o n s e t o p r e o p e r a t i v e
induction chemotherapy could be improved by
Due to its l o n g - s t a n d i n g collaborative activities, altering p o s t o p e r a t i v e c h e m o t h e r a p y ( R o s e n et al
C O S S w a s a b l e to contribute t h e largest a n a l y s i s of 1981). Trial C O S S - 8 2 , h o w e v e r , f a i l e d t o d e t e c t a
p r o g n o s t i c f a c t o r s in o s t e o s a r c o m a p u b l i s h e d to benefit of s a l v a g e t r e a t m e n t e v e n in p o o r r e s -
d a t e , a n a n a l y s i s b a s e d o n 1702 patients (Bielack et p o n d e r s w h o had received only v e r y low-intensity
al 2002a). S e v e r a l robust p r o g n o s t i c f a c t o r s w e r e t r e a t m e n t p r e o p e r a t i v e l y a n d t h e n w e n t o n to v e r y
identified in patients w h o w e r e t r e a t e d a c c o r d i n g i n t e n s i v e s a l v a g e t h e r a p y ( W i n k l e r e t al 1988), a n d
to m o d e r n multimodal standards. Primary m e t a s - m a n y o t h e r s u b s e q u e n t a t t e m p t s to i m p r o v e t h e
t a s e s , axial or p r o x i m a l extremity location, a n d p r o g n o s i s of p o o r r e s p o n d e r s b y altering post-
large t u m o r size w e r e f o u n d to b e of i n d e p e n d e n t operative chemotherapy h a v e also failed.
n e g a t i v e p r o g n o s t i c v a l u e (Bielack et al 2002a).
Treatment-associated variables, however, w e r e
f o u n d to b e e v e n m o r e i m p o r t a n t t h a n t h e s e p r e -
senting factors. Incomplete surgery w a s almost Treatment of metastatic
incompatible with long-term survival. O n the other osteosarcoma
h a n d , the C O S S group h a s b e e n able to d e m o n -
strate that e v e n patients w i t h p r i m a r y m e t a s t a s e s All detectable m e t a s t a s e s m u s t be r e m o v e d by
( K a g e r et al 2003) o r axial o s t e o s a r c o m a s (Bielack s u r g e r y if t h e a i m of t h e r a p y is c u r a t i v e . A s m o s t
et al 1995, Ozaki et al 2002, 2003) c a n b e c u r e d if metastases develop in the lung, this usually
t r e a t m e n t i n c l u d e s c o m p l e t e s u r g i c a l r e m o v a l of implies thoracotomy. COSS and most other
all k n o w n t u m o r sites a n d m u l t i d r u g c h e m o t h e r - groups r e c o m m e n d bilateral pulmonary explo-
apy. For resectable osteosarcomas, response ration w i t h m a n u a l p a l p a t i o n of b o t h l u n g s e v e n
to induction chemotherapy becomes the most in c a s e s of s e e m i n g l y u n i l a t e r a l d i s e a s e , a s t h e
 O B J E C T I V E S AND RESULTS OF THE COSS STUDIES 181

experienced thoracic surgeon will often find therapy. Using such therapy, survival rates
additional contralateral metastases. of 60 p e r c e n t a n d a b o v e h a v e b e e n r e a c h e d in
In t h e C O S S trials, a p p r o x i m a t e l y one-quarter of u n s e l e c t e d patients w i t h o s t e o s a r c o m a . In a d d i -
all patients w i t h p r i m a r y m e t a s t a s e s b e c a m e l o n g - tion, C O S S h a s b e e n a b l e to build t h e largest
t e r m s u r v i v o r s ( K a g e r et al 2003), pointing out that osteosarcoma database worldwide, to answer
t h e r a p e u t i c nihilism is c o n t r a i n d i c a t e d in p r i m a r y important treatment-related questions and to
metastatic osteosarcoma. T h e survival rate for m a k e r o b u s t s t a t e m e n t s c o n c e r n i n g t h e relative
patients w h o a c h i e v e a c o m p l e t e surgical r e m i s s i o n i m p o r t a n c e of v a r i o u s clinical p r o g n o s t i c f a c t o r s .
of both t h e p r i m a r y a n d all p r i m a r y m e t a s t a s e s in O v e r t h e past d e c a d e s , t h e t r e a t m e n t regimens
t h e context of a n i n t e n s i v e p o l y c h e m o t h e r a p y regi- h a v e b e e n refined in o r d e r to r e d u c e toxicity a n d
men r o s e to a p p r o x i m a t e l y 40 per c e n t a n d t h e yet m a i n t a i n or e v e n e n h a n c e a n t i - t u m o r activity.
p r o g n o s i s of patients w i t h solitary p r i m a r y m e t a s - D e s p i t e all t h e a d v a n c e s in o s t e o s a r c o m a ther-
t a s e s w a s indistinguishable f r o m that of patients apy, s e v e r a l p r o b l e m s r e m a i n , including t h e d i s -
w i t h localized d i s e a s e ( K a g e r et al 2003). m a l results a c h i e v e d in i n o p e r a b l e t u m o r s a n d t h e
Osteosarcoma recurrences also usually involve p o o r o u t c o m e after r e l a p s e . A l s o , o s t e o s a r c o m a is
t h e l u n g . U n f o r t u n a t e l y , t h e s u r v i v a l rates after infrequent, s o that e v e n t h e largest c o - o p e r a t i v e
o s t e o s a r c o m a r e l a p s e a r e poor, w e l l b e l o w 20 per g r o u p s n e e d long p e r i o d s to c o m p l e t e their p r o s -
c e n t a t 10 y e a r s . In t h e C O S S e x p e r i e n c e , w h i c h p e c t i v e trials. C O S S has therefore joined forces
recently allowed an a n a l y s i s of 576 affected w i t h other r e n o w n e d E u r o p e a n a n d A m e r i c a n c o -
patients, a short latency period and multiple o p e r a t i v e g r o u p s , w h o w i l l t o g e t h e r run intergroup
metastases are associated with an especially poor o s t e o s a r c o m a trials f r o m 2004. It is e x p e c t e d that
outcome ( B i e l a c k et al 2003). A g a i n , c o m p l e t e this c o - o p e r a t i o n w i l l a l s o lead to co-ordinated
s u r g e r y of all s i t e s of r e c u r r e n c e is a s m u c h a efforts in b a s i c r e s e a r c h a n d w i l l t h e r e f o r e help
p r e r e q u i s i t e for c u r e . It m a y b e p r u d e n t to irradi- to u n c o v e r s o m e of t h e biological s e c r e t s still
ate s u i t a b l e i n o p e r a b l e l e s i o n s in o r d e r to s l o w t h e s u r r o u n d i n g this intriguing m a l i g n a n c y .
progression of disease, but it is unlikely that
t h i s w i l l l e a d to p e r m a n e n t c u r e . P a t i e n t s w i t h
inoperable osteosarcoma relapse w h o received
chemotherapy survived longer than those who Acknowledgment
d i d not in a r e c e n t Italian s e r i e s (Ferrari et al 2003),
The C O S S studies are generously supported by
a s w e l l a s in t h e C O S S trials ( B i e l a c k et al 2003).
Deutsche Krebshilfe.
The e x a c t role of a d j u v a n t c h e m o t h e r a p y in t h e
t r e a t m e n t of o p e r a b l e r e l a p s e d o s t e o s a r c o m a is
still b e i n g d e b a t e d , but t h e C O S S results s u g g e s t
that s e c o n d r e l a p s e s m a y at least b e p o s t p o n e d References
( B i e l a c k et al 2003). To d a t e , h o w e v e r , t h e s u c c e s s
of c h e m o t h e r a p y for r e l a p s e d o s t e o s a r c o m a h a s Bielack S , Erttmann R, Winkler K, Landbeck G (1989)
b e e n limited at b e s t , a n d t h e r e is n o a c c e p t e d Doxorubicin: effect of different schedules on toxicity
and anti-tumor efficacy, Eur J Cancer Clin Oncol
s t a n d a r d r e g i m e n o u t s i d e of clinical trials.
25:873-82.

Bielack S , Wulff B, Delling G et al (1995) Osteosarcoma

of the trunk treated by multimodal therapy: experience
Conclusions and perspectives of the cooperative osteosarcoma study group C O S S ,
Med Pediatr Oncol 24:6-12.
Treatment according to protocols of the
Cooperative Osteosarcoma Study Group ( C O S S ) Bielack S , Kempf-Bielack B, Winkler K (1996a)
h a s b e c o m e t h e s t a n d a r d of c a r e for o s t e o s a r c o m a Osteosarcoma: relationship of response to preoperative
chemotherapy and type of surgery to local recurrence,
patients in G e r m a n y , Austria, a n d S w i t z e r l a n d . T h e
J Clin Oncol 15:683-4 (letter).
g r o u p h a s b e e n a b l e to f o r m a n d m a i n t a i n a c l o s e l y
knit n e t w o r k of specialists f r o m all a r e a s of sar-
Bielack S , Erttmann R, Kempf-Bielack B, Winkler K
c o m a t h e r a p y w h o s e collaboration g u a r a n t e e s that (1996b) Influence of scheduling on toxicity and clinical
all affected patients f r o m t h e s e c o u n t r i e s c a n h a v e efficacy of doxorubicin: what do w e know in the mid-
access to m o d e r n , efficacious interdisciplinary nineties? Eur J Cancer 32:1652-60.
182 T U M O R S OF THE HAND

Bielack S , Kempf-Bielack B, Schwenzer D et al (1999) patients with osteosarcoma of the extremities, N Engl J
Neoadjuvante Therapie des lokalisierten Osteosarkoms Med 314:1600-6.
der Extremitäten. Erfahrungen der Cooperativen Osteo-
sarkomstudiengruppe C O S S an 925 Patienten, Klin Meyers PA, Schwartz CL, Bernstein M et al (2001) Addition
Padiatr 211:260-70. of ifosfamide and muramyl tripeptide to cisplatin, doxo-
rubicin and high-dose methotrexate improves event-free
Bielack S , Kempf-Bielack B , Delling G et al (2002a) survival ( E F S ) in localized osteosarcoma ( O S ) . Pro-
Prognostic factors in high-grade osteosarcoma of the ceedings of the American Society of Clinical Oncology
extremities or trunk. A n analysis of 1702 patients 20:1463 (abstract).
treated on neoadjuvant Cooperative Osteosarcoma
Study Group protocols, J Clin Oncol 20:776-90. OzakiT, Flege S , Liljenqvist U et al (2002) Osteosarcoma
of the spine: Experience of the Cooperative Osteo-
Bielack S , Jürgens H (2002b) In reply to 'Comment on sarcoma Study Group ( C O S S ) , Cancer 94:1069-77.
Prognostic factors in high-grade osteosarcoma of the
extremities or trunk: an analysis of 1,702 patients treated Ozaki T, Flege S , Kevric M et al (2003) Osteosarcoma of
on neoadjuvant cooperative Osteosarcoma Study Group the pelvis: experience of the Cooperative Osteosarcoma
Protocols' by Ferrari et al.' J Clin Oncol 20:2910. Study Group ( C O S S ) , J Clin Oncol 21:334-341.

Bielack S , Kempf-Bielack B, Branscheid B et al (2003) Picci P, Sangiorgi L, Rougraff BT et al (1994)The relation-

Relapsed osteosarcoma: an analysis of 576 Cooperative ship of chemotherapy-induced necrosis and surgical
Osteosarcoma Study Group ( C O S S ) patients. Presented margins to local recurrence in osteosarcoma, J Clin
at 39th Annual Meeting of the American Society of Oncol 12:2699-705.
Clinical Oncology, M a y 31 - J u n e 3, Chicago: abstr.
Rosen G , Caparros B , H u v o s A G et al (1981)
Enneking W E , Spanier S S , Goodmann M A (1980) A sys- Preoperative chemotherapy for osteogenic sarcoma:
tem for the surgical staging of musculo-skeletal tumors, selection of postoperative adjuvant chemotherapy
Clin Orthop 153:106-20. based on the response of the primary tumor to pre-
operative chemotherapy, Cancer 49:1221-39.
Ferrari S , Briccoli A , Mercuri M et al (2003) Postrelapse
survival in osteosarcoma of the extremities: prognostic Sanders T G , ParsonsTW III (2001) Radiographic imaging
factors for long-term survival, J Clin Oncol 21:710-15. of musculoskeletal neoplasia, Cancer Control 8:221-31.

Fletcher C D M , Unni K, Mertens K eds. (2002) WHO Smith M A , Ungerleider R S , Horowitz M E et al (1991)
Classification of Tumours. Pathology and Genetics of Influence of doxorubicin dose intensity on response and
Tumours of SoftTissue and Bone. IARC Press: Lyon. outcome for patients with osteogenic sarcoma and
Ewing's sarcoma, J Natl Cancer Inst 83:1460-70.
Fuchs N, Bielack S , Epler D et al (1998) Long-term results
of the co-operative G e r m a n - A u s t r i a n - S w i s s osteo- Sobin LH, Wittekind C (2002) UICCTNM Classification of
sarcoma study group's protocol C O S S - 8 6 of intensive MalignantTumors, 6th edn. Wiley: New York.
multidrug chemotherapy and surgery for osteosarcoma
of the limbs, Ann Oncol 9:893-9. Winkler K, Beron G , Kotz R et al (1984) Neoadjuvant
chemotherapy for osteogenic sarcoma: results of a
Graf N, Winkler K, Betlemovic M et al (1994) cooperative German/Austrian study, J Clin Oncol
Methotrexate pharmacokinetics and prognosis in 2:617-23.
osteosarcoma, J Clin Oncol 12:1443-51.
Winkler K, Beron G , Delling G et al (1988) Neoadjuvant
Kaatsch p, Kaletsch U, Spix C et al (1999) Jahresbericht chemotherapy of osteosarcoma: results of a random-
1998: Deutsches Kinderkrebsregister. Mainz, Germany, ized cooperative trial (COSS-82) with salvage chemo-
Johannes-Gutenberg-Universität, Institut für Medizinische therapy based on histological tumor response, J Clin
Statistik und Information. Oncol 6:329-37.

Kager L, Zoubek A, Pötschger U et al (2003) Primary Winkler K, Bielack S , Delling G et al (1990) Effect of
metastatic osteosarcoma: presentation and outcome of intraarterial versus intravenous cisplatin in addition to
202 patients treated on neoadjuvant Cooperative systemic doxorubicin, high dose methotrexate, and
Osteosarcoma Study Group ( C O S S ) protocols, J Clin ifosfamide on histologic tumor response in osteo-
Oncol 21:2011-18. sarcoma (Study COSS-86), Cancer 66:1703-10.

Link MP, Goorin A M , Miser A W et al (1986)The effect of

adjuvant chemotherapy on relapse-free survival in
20
Chondrosarcoma
Caroline Leclercq

C h o n d r o s a r c o m a a c c o u n t s f o r 30 p e r c e n t of all
p r i m a r y m a l i g n a n t t u m o r s of b o n e . It is e x t r e m e l y
r a r e in t h e h a n d , h o w e v e r , a m o u n t i n g to only
1 p e r c e n t of all c h o n d r o s a r c o m a a c c o r d i n g t o
D a h l i n (1973). It w a s first r e p o r t e d in t h e h a n d b y
L i c h t e n s t e i n a n d J a f f e (1943), w h o d e s c r i b e d a
l e s i o n of t h e p r o x i m a l p h a l a n x of t h e i n d e x finger.
T h e m a i n a n d m o s t c h a r a c t e r i s t i c f e a t u r e is a
s l o w l y , b u t s t e a d i l y , i n c r e a s i n g s w e l l i n g ( F i g . 1),
usually painless, developing in a n a d u l t over
50 y e a r s o l d , a n d w h i c h c a n r e a c h a c o n s i d e r a b l e
size o v e r s e v e r a l y e a r s ( F i g . 2 ) . In t h e h a n d , it is
often l o c a t e d o n e i t h e r s i d e o f t h e m e t a c a r p o p h a -
l a n g e a l j o i n t (distal m e t a c a r p a l , o r p r o x i m a l part
of t h e p r o x i m a l p h a l a n x ) , a n d p r e f e r a b l y o n t h e Figure 1

fifth ray. A f e w c a s e s of chondrosarcomas have

Chondrosarcoma of the first metacarpal. (Coll Pr Genin)
b e e n d e s c r i b e d in t h e c a r p u s ( D a h l i n a n d S a l v a d o r
1974, G r a n b e r r y a n d B r y a n 1978). O c c u r r e n c e in
t h e m i d d l e p h a l a n x is r a r e , a n d e x t r e m e l y r a r e in
t h e distal p h a l a n x .
R a d i o g r a p h i c f e a t u r e s i n c l u d e a l a r g e lytic a r e a
in t h e b o n e , irregular, w i t h d i s s e m i n a t e d calcifica-
t i o n s . B o n e c o n t o u r s a r e irregular, w i t h a p o s s i b l e
c o r t e x r u p t u r e a n d s p r e a d i n g o f t h e l e s i o n into
t h e soft t i s s u e s ( F i g . 3 ) .
A f e w c a s e s of juxtacortical c h o n d r o s a r c o m a s
have been reported, either periosteal with a
faintly radiopaque m a s s creating a saucer-shaped
cortical e r o s i o n ( J o k l 1971, W u a n d K e l l y 1977), o r
extraperiosteal without a n y modification of t h e
b o n e c o n t o u r ( V i c h a r d et a l 1980).
Macroscopically, chondrosarcoma presents a s
a bluish-white lobulated cartilaginous substance,
w i t h softer g e l a t i n o u s a r e a s . T h e t y p i c a l h i s t o l o g i -
c a l a s p e c t of c h o n d r o s a r c o m a is t h a t of h y p e r -
Figure 2
cellularity with binucleated cells, a n d swollen
n u c l e i c o n t a i n i n g v i s i b l e m i t o s i s . In m o s t c a s e s Voluminous chondrosarcoma of the fifth metacarpal, of
t h e h i s t o l o g i c a l g r a d i n g of c h o n d r o s a r c o m a in t h e 7-years' duration. (Courtesy Pr Le Viet)
184 T U M O R S OF THE HAND

h a n d is a g r a d e 1. B u t i d e n t i c al a s p e c t s m a y b e m e t a s t a s i s is v e r y rare ( J u s t i s a n d Dart 1983), a n d

encountered in chondromas, especially hyper- the rate of recurrence after curettage is low
cellularity a n d b i n u c l e a t e d c e l l s . In s o m e c a s e s ( 9 - 3 3 per c e n t ) . T h i s led M a n k i n (1999) t o v i e w t h e m
w i t h clinical and radiographic features resem- a s a s e p a r a t e entity w h i c h h e t e r m e d 'primitive
bling chondrosarcomas, but with histological n o n - m e t a s t a s i n g c h o n d r o s a r c o m a s of t h e fingers'.
f e a t u r e s t y p i c a l of c h o n d r o m a s , t h e t e r m ' a g g r e s - It is not clear from the literature whether
sive chondroma' has been coined. chondrosarcoma m a y be derived f r o m solitary
S p o n t a n e o u s e v o l u t i o n of t h e s e l e s i o n s in t h e e n c h o n d r o m a o r not. T h r e e a r t i c l e s in t h e litera-
h a n d is u n u s u a l c o m p a r e d w i t h o t h e r chondro- t u r e h a v e c o m p a r e d f e a t u r e s of b o t h t u m o r s on
sarcomas, a s they grow very slowly over the years, a l a r g e s c a l e ( B a u e r et al 1995, 40 c h o n d r o m a s
versus 40 chondrosarcomas; Geirnaerdt et al
1997, 3 5 c h o n d r o m a s v e r s u s 4 3 chondrosarco-
m a s ; C a w t e et al 1998, 12 c h o n d r o m a s v e r s u s
12 c h o n d r o s a r c o m a s ) . E a c h of t h e s e g r o u p s of
w o r k e r s c a m e to t h e c o n c l u s i o n that it w a s i m p o s -
s i b l e t o differentiate c h o n d r o m a s a n d c h o n d r o s a r -
c o m a s b o t h clinically a n d r a d i o g r a p h i c a l l y , at least
d u r i n g t h e e a r l y s t a g e of c h o n d r o s a r c o m a . T h e
s w e l l i n g is i d e n t i c a l , a n d t h e r a d i o g r a p h i c a s p e c t
is v e r y similar b e f o r e cortex rupture o c c u r s ( F i g . 4).
T h e y also c o n c l u d e d that histological differentia-
t i o n w a s not reliable in a n u m b e r of c a s e s .
M o r e o v e r , a r e v i e w of four large series of
c h o n d r o s a r c o m a of t h e h a n d s ( a n d f e e t ) r e v e a l e d
t h a t m a l i g n a n t d e g e n e r a t i o n of a b e n i g n l e s i o n ,
w h i c h o c c u r r e d in 0 - 2 2 p e r c e n t of c a s e s , w a s
Figure 3
never s e c o n d a r y to a solitary chondroma, but
Radiographic appearance of a chondrosarcoma of the prox- r a t h e r to a c h o n d r o m a t o s i s , a n o s t e o c h o n d r o m a ,
imal phalanx of the thumb, with a typical polylobulated lytic or a n osteochondromatosis (Dahlin a n d S a l v a d o r
image, and obvious cortex rupture on both sides. 1974, P a l m i e r i 1984, O g o s e et al 1997, C a w t e et al

Figure 4

Radiographic appearance of the

case shown in Fig. 2: (a) initial
X-rays which misled to a reassur-
ing diagnosis of chondroma, and
(b) X-rays 7 years later. (Courtesy
a b Pr Le Viet)
 CHONDROSARCOMA 185

Figure 5

First ray amputation for a chondrosarcoma of the proximal

phalanx of the thumb. The patient, aged 72, refused any
type of reconstruction.

1998). T h e o n l y series w h e r e chondrosarcoma

w a s said to h a v e d e g e n e r a t e d f r o m c h o n d r o m a s
(in 4/35 c a s e s ) w a s not a p e r s o n a l s e r i e s , but a
r e v i e w of t h e c a s e s r e g i s t e r e d at a n a t i o n a l c o m -
m i t t e e of t u m o r s ( B o v e e et al 1999).
A f e w r e p o r t s s e e m t o b r i n g a r g u m e n t s for s a r -
c o m a t o u s transformation of a c h o n d r o m a (Culver b
et al 1975, R o b e r t s a n d P r i c e 1977), but s e v e r a l
Figure 6
o t h e r s t e n d t o d e m o n s t r a t e t h e o p p o s i t e ( W u et al
1983, B o n n e v i a l l e et al 1988, G a u l k e a n d P r e i s s e r Same case as Fig. 3. (a) Both initial biopsy and curettage led
1997). T h e t w o cases reported by G a u l k e and to a histological diagnosis of enchondroma, and the lesion
P r e i s s e r a r e r a t h e r c h a r a c t e r i s t i c , a s in b o t h c a s e s was packed with artificial bone substitute. (b) Recurrence at
a histological diagnosis of c h o n d r o m a h a d b e e n 6 years, with rupture of the volar cortex and dissemination
m a d e , a n d w h e n t h e s l i d e s w e r e c h e c k e d after a into the soft tissues. The thumb underwent amputation,
and the diagnosis was corrected to chondrosarcoma.
r e c u r r e n c e , t h e initial d i a g n o s i s w a s c o r r e c t e d to
that of a primary chondrosarcoma. Reviewing
t h e p r e v i o u s literature on 18 c h o n d r o s a r c o m a s t h e p r e o p e r a t i v e d i a g n o s i s is u n c e r t a i n , it is prefer-
a r i s i n g f r o m a pre-existing e n c h o n d r o m a , N e l s o n a b l e to p e r f o r m a n e x c i s i o n - b i o p s y rather t h a n a
et al (1990) f o u n d o n l y o n e w e l l - d o c u m e n t e d c a s e , s i n g l e biopsy, a s c h o n d r o s a r c o m a m a y contain
and added one personally documented case. juxtapositions of c h o n d r o m a t o u s a n d c h o n d r o s a r -
Recent genetic investigations (Tallini et al c o m a t o u s a r e a s . In s u c h a c a s e , t h e skin incision
2002) h a v e s h o w n c h r o m o s o m a l a b n o r m a l i t i e s in m u s t b e p l a n n e d b e a r i n g in m i n d a p o s s i b l e s e c o n -
4 1 o u t of 92 i n v e s t i g a t e d c a r t i l a g i n o u s t u m o r s . A dary amputation through the s a m e approach.
different a b n o r m a l pattern for e a c h t y p e of t u m o r , If a m p u t a t i o n is d e c i d e d u p o n , insofar a s o n e is
a n d s p e c i f i c t y p e s of a b n o r m a l p a t t e r n s for e a c h often d e a l i n g w i t h elderly patients, e l a b o r a t e r e c o n -
g r a d e in m a l i g n a n t t u m o r s w e r e f o u n d . T h i s t y p e structive s u r g e r y is not a l w a y s indicated. In fact,
of i n v e s t i g a t i o n should b e v e r y helpful in the s u c h s u r g e r y m a y e v e n b e refused by t h e patient
f u t u r e for e a r l y d i a g n o s i s of c h o n d r o s a r c o m a . themself ( F i g . 5), but it is theoretically justified,
M o s t a u t h o r s a g r e e that a m p u t a t i o n is t h e treat- g i v e n t h e overall f a v o r a b l e p r o g n o s i s of chon-
m e n t of c h o i c e for c h o n d r o s a r c o m a of t h e t u b u l a r d r o s a r c o m a in t h e h a n d .
b o n e s o f t h e h a n d . H o w e v e r , in v i e w o f t h e l o w Recurrence after surgical treatment of an
a g g r e s s i v e c o u r s e of this l e s i o n , b o n e excision enchondroma should always arouse the suspi-
m a y be justified w h e n t h e r e is n o cortical rupture cion of a n initial f a i l u r e to recognize a chon-
a n d i n v a s i o n of t h e a d j a c e n t soft t i s s u e s . W h e n e v e r d r o s a r c o m a ( F i g . 6). R e o p e r a t i o n is r e q u i r e d in
186 T U M O R S O F T H E HAND

these cases, with careful histological examination Jokl P, Albright J A , Goodman A H (1971) Juxtacortical
of t h e w h o l e s p e c i m e n , a s w e l l a s a r e v i e w of t h e chondrosarcoma of the hand, J Bone Joint Surg 53A:
slides from the primary curettage. 1370-6.

Justis E J , Dart RC (1983) Chondrosarcoma of the hand

with metastasis: a review of the literature and case

References report, J Hand Surg 8:320-4.

Bauer H, Brosjo 0, Kreicbergs A, Lindholm J (1995) Lichtenstein L, Jaffe HL (1943) Chondrosarcoma of

Low risk of recurrence of enchondroma and low-grade bone, Am J Pathol 19:553-90.
chondrosarcoma in extremities. 80 patients followed for
2-25 years, Acta Orthop Scand 66:755-60. Mankin H J (1999) Chondrosarcomas of digits: are they
really malignant? Cancer 85:1635-7.
Bonnevialle P, Mansat M , Durroux R et al (1988) Les
chondromes de la main: etude d'une série de trente cinq Nelson DL, Abdul-Karim F W , Carter J R , Makley J T (1990)
cas, Ann Chir Main 7:32-44 (article in French and in Chondrosarcoma of small bones of the hand arising
English). from enchondroma, J Hand Surg Am 15:655-9.

B o v e e J V , v a n der Heul RO, Taminiau A H , Hogendoorn Ogose A , Krishnan Unni K, S w e e R G et al (1997)

PC (1999) Chondrosarcoma of the phalanx: a locally Chondrosarcoma of small bones of the hands and feet,
aggressive lesion with minimal metastatic potential: a Cancer 80:50-9.
report of 35 cases and a review of the literature,
Cancer 86:1724-32. PalmieriTJ (1984) Chondrosarcoma of the hand, J Hand
Surg Am 9:332-8.
Cawte T, Steiner G C , Beltran J , Dorfman HD (1998)
Chondrosarcoma of the short tubular bones of the Roberts P H , Price CHG (1977) Chondrosarcoma of the
hands and feet, Skeletal Radiol 27:625-32. bones of the hand, J Bone Joint Surg 59B:213-21.

Culver J E , S w e e t DE, McCue FC (1975) Chondrosarcoma Tallini G , Dorfman H, Brys P et al (2002) Correlation
of the hand arising from a pre-existent benign solitary between clinicopathological features and karyotype
enchondroma, Clin Orthop 113:128-31. in 100 cartilaginous and chordoid tumours. A report
from the Chromosomes and Morphology ( C H A M P )
Dahlin DC (1973) Bone Tumors, 2nd edn. Charles C. Collaborative Study Group, J Pathol 196:194-203.
Thomas: Springfield.
Vichard P, Pinon P, Carbillet J P et al (1980) Le chon-
Dahlin DC, Salvador A H (1974) Chondrosarcoma of the drosarcoma juxta-osseux, Rev Chir Orthop 66:111-14.
bones of the hand and feet, Cancer 34:755-60.
W u KK, Kelly A P (1977) Periosteal (juxtacortical) chondro-
Gaulke R, Preisser P (1997) 'Secondary' chondrosar- sarcoma: report of a case occurring in the hand,
coma of the hand. Case report and review of the litera- J Hand Surg 2:314-15.
ture, Handchir Mikrochir Plast Chir 29:251-5.
W u K, Frost H, Guise E (1983) A chondrosarcoma of the
Geirnaerdt M , Hermans J , B l o e m J L et al (1997) hand arising from an asymptomatic benign solitary
Usefulness of radiography in differentiating enchon- enchondroma of 40 y e a r s ' duration, J Hand Surg
droma from central grade 1 chondrosarcoma, Am J 8A:317-19.
Roentgenol 169:1097-104.

Granberry W , Bryan W (1978) Chondrosarcoma of the

trapezium; a case report, J Hand Surg 3:277-9.
21
Metastatic tumors to the
hand and wrist
Vincent R Hentz

Introduction malignancy
particularly
elsewhere
true of
in
subungual
the body.
metastases.
This is
In

P r i m a r y t u m o r s of a l m o s t all t y p e s a n d l o c a t i o n s u p to 40 p e r c e n t of c a s e s of s u b u n g u a l m e t a s -

h a v e b e e n reported t o m e t a s t a s i z e t o t h e h a n d , tases, this has been the initial evidence of

w i t h b o n y m e t a s t a s e s e x c e e d i n g soft t i s s u e m e t a s - malignancy. Occasionally, an acral presentation

t a s e s b y a factor of six t o o n e . Interestingly, in t h e is t h e only determinable evidence of malig-

c u m u l a t i v e total of c a s e s , m e t a s t a s e s to t h e h a n d nancy, with no primary tumor to be found

outnumber t h o s e to t h e f o o t 2 : 1 . T h e highest ( A b r a h a m s 1995).

i n c i d e n c e o c c u r s in m e n (66 per c e n t ) 4 0 - 6 0 y e a r s Acral metastases frequently mimic inflamma-

of a g e ( a v e r a g e a g e 55 y e a r s ) ( G l i c e n s t e i n et al tory lesions such a s nail-bed infections or more
1988). W h i l e o s s e o u s m e t a s t a s e s , in g e n e r a l , a r e commonly, pulp-space infections (felon). Many
c o m m o n , r e p r e s e n t i n g a p p r o x i m a t e l y 30 per c e n t entities are treated a s s u c h , again delaying proper
of metastases, tumors that metastasize to the diagnosis and treatment.
carpus or hand are uncommon. The incidence, A s e l s e w h e r e in t h e b o d y , t u m o r s t h a t m e t a s -
although probably under-reported, is s a i d to be tasize to the hand favor marrow-containing
a b o u t 0.1 per c e n t of all m e t a s t a s e s ( K e r i n 1983), in b o n e s . T h e r e f o r e , m e t a s t a s e s t o t h e l o n g b o n e s of
k e e p i n g w i t h t h e d i c t u m t h a t m e t a s t a s e s a r e rare t h e h a n d f a r e x c e e d m e t a s t a s e s t o t h e b o n e s of
b e l o w t h e k n e e a n d distal to t h e e l b o w . T h i s belief t h e c a r p u s . T h e m o s t c o m m o n site of m e t a s t a s e s
t r a n s l a t e s into f e w patients w i t h a d i a g n o s i s of a is t h e t e r m i n a l p h a l a n x (52 per c e n t ) , t h e n the
m a l i g n a n c y h a v i n g r a d i o g r a p h s of h a n d s a n d feet p r o x i m a l p h a l a n x (30 per c e n t ) , f o l l o w e d by the
as part of the a s s e s s m e n t of their neoplasm. middle phalanx (18 per c e n t ) , a n d , finally, t h e
B e c a u s e of this relative rarity, t h e d i a g n o s i s of m e t a c a r p a l (17 per c e n t ) . C a r p a l b o n e m e t a s t a s e s
m a l i g n a n t s p r e a d to t h e h a n d s is s c a r c e l y c o n s i d - a c c o u n t for about 17 per c e n t of c a s e s (Kerin
e r e d , e v e n in patients w i t h k n o w n malignancies 1983, G l i c e n s t e i n et al 1988). E v e r y c a r p a l b o n e
p r e s e n t i n g w i t h s o m e t y p e of h a n d c o m p l a i n t , a n d h a s b e e n r e p o r t e d a s a site of m e t a s t a s e s ; w i t h
m a n a g e m e n t is c o n s e q u e n t l y d e l a y e d . H e a l e y et al capitate, scaphoid, and lunate predominating.
(1986) s t u d i e d the r e c o r d s of 29 patients with H a n d d o m i n a n c e p l a y s n o r o l e in t h e l o c a t i o n o r
4 1 m e t a s t a t i c l e s i o n s of t h e h a n d o r foot. In f i v e i n c i d e n c e of t h e s e m e t a s t a s i z i n g tumors.
patients t h e l e s i o n s m i m i c k e d a b e n i g n condition,
and inappropriate treatment w a s given. In 11
patients t h e a c r o m e t a s t a s e s w e r e t h e first indica-
t i o n of m a l i g n a n t d i s e a s e , a n d f o u r o t h e r patients Findings
had no k n o w n primary malignant lesion.
In a p p r o x i m a t e l y 16-20 per c e n t of c a s e s , a n The most common symptom of an osseous
a c r a l m e t a s t a s i s is t h e first e v i d e n c e of a p r i m a r y m e t a s t a s i s is p a i n . T h e pain is s a i d to b e of v a r y i n g
188 T U M O R S OF THE HAND

intensity, and may b e of spontaneous onset

o r b e a s s o c i a t e d w i t h t e n d e r n e s s of t h e a f f e c t e d
Occurrence
area. Usually the pain is a s s o c i a t e d w i t h an
M o s t s t u d i e s h a v e b e e n c o n s i s t e n t in t e r m s of
e n l a r g i n g m a s s ; r a r e l y a r e t h e s e silently e n l a r g -
w h i c h p r i m a r y t u m o r s a r e m o s t likely t o m e t a s t a -
ing masses. The second common finding is size t o t h e h a n d . In 1983, K e r i n r e v i e w e d t h e liter-
evidence of an inflammatory reaction with ature a n d m a p p e d the location a n d type, w h e r e
redness and warmth. Since the inflammatory
k n o w n , of 156 c a s e s of t u m o r s t h a t h a d m e t a s t a -
components m a y be the dominant symptoms,
sized to t h e h a n d . T h e r e w e r e 77 i n s t a n c e s of
these lesions suggest alternative diagnoses such
p h a l a n g e a l i n v o l v e m e n t in e a c h h a n d . E i g h t y - f o u r
a s r h e u m a t i c o r i n f e c t i v e l e s i o n s . Interestingly, a s
p e r c e n t w e r e o s s e o u s a n d 16 p e r c e n t w e r e of
o p p o s e d to such primary bone tumors of the
soft t i s s u e i n v o l v e m e n t . M o s t of t h e t u m o r s w e r e
d i g i t s s u c h a s e n c h o n d r o m a s , t h e literature i n d i -
c a r c i n o m a s , o n l y six s a r c o m a s m e t a s t a t i c t o t h e
c a t e s t h a t a p a t h o l o g i c f r a c t u r e d u e to a b o n e
h a n d w e r e r e p o r t e d . In K e r i n ' s s t u d y , t h e l u n g w a s
m e t a s t a s i s of t h e h a n d a s t h e initial i n d i c a t i o n of
t h e m a j o r p r i m a r y site (52 p a t i e n t s ) . T h e b r e a s t
p a t h o l o g y is r a r e ( G i r a u d e t al 1984). a n d k i d n e y w e r e next in f r e q u e n c y (15 b r e a s t a n d
Soft tissue metastases may more often be 14 r e n a l t u m o r s ) .
a s y m p t o m a t i c and h a v e a demonstrated predilec- F r a s s i c a e t al 1989 r e p o r t e d o n t h e M a y o C l i n i c
t i o n for t h e p a l m a n d nail b e d . T h e y m a y p r e s e n t e x p e r i e n c e w i t h 22 l e s i o n s in 18 p a t i e n t s seen
a s a n initial s o l i t a r y m a s s f o l l o w e d b y t h e a p p e a r - o v e r a 43-year period. T h e r e w a s a n e q u a l inci-
a n c e of additional masses. These are typically d e n c e of lung a n d renal t u m o r s a n d 12 o f 22
very firm and non-tender and are subcutaneous l e s i o n s w e r e l o c a t e d in t h e d i s t a l p h a l a n x .
in l o c a t i o n . U l c e r a t i o n of t h e o v e r l y i n g skin is a
T h e p r e d o m i n a n c e of p r i m a r y l u n g t u m o r s a s
late m a n i f e s t a t i o n .
t h e s o u r c e of a c r a l m e t a s t a s e s is felt t o b e a c o n -
R a d i o g r a p h i c f i n d i n g s in b o n e m e t a s t a s e s a r e s e q u e n c e of direct arterial i n v a s i o n b y t h e t u m o r .
almost a l w a y s characterized by bone destruction. In t h i s c a s e , t h e r e is n o filtration b y t h e l i v e r a s is
T h e osteolytic l e s i o n is t y p i c a l l y irregular with the case with most bowel primaries. Because
r e p l a c e m e n t of t h e e n t i r e b o n e , but w i t h p r e s e r - t h e f i n g e r s a r e rich in b l o o d s u p p l y a n d b e c a u s e
v a t i o n of t h e j o i n t s p a c e . S o f t t i s s u e m e t a s t a s e s , of distal c i r c u l a t o r y s l o w i n g , t h e s e t u m o r s l o c a l -
as they enlarge, m a y cause scalloping of the ize to t h e f i n g e r s . Lung tumors metastatic to
underlying bone. the digits are m o r e often a d e n o c a r c i n o m a s a s
Subungual metastases are more frequently o p p o s e d to anaplastic or e p i d e r m o i d c a r c i n o m a s .
c h a r a c t e r i z e d b y a n e n l a r g e d digit, in a s s o c i a t i o n Subdiaphragmatic neoplasms such as gastro-
w i t h d i s t o r t i o n of t h e n a i l . A r e d d i s h - p u r p l e d i s - intestinal, vesical, renal, a n d uterine malignan-
c o l o r a t i o n is f r e q u e n t l y s e e n . cies, metastasize m o r e frequently to the foot. A
p o s s i b l e e x p l a n a t i o n f o r t h i s latter f i n d i n g is t h e
retrograde s p r e a d of tumor emboli from the
vertebral v e n o u s plexus d o w n incompetent leg
Differential diagnosis v e i n s ( G l i c e n s t e i n 1988).
B r e a s t c a n c e r s s p r e a d t o t h e h a n d s v e r y late in
From the clinical picture, acral metastases, t h e d i s e a s e . T h e p r o p o s e d m e c h a n i s m is v i a t h e
particularly subungual metastases may mimic internal mammary trunk a n d t h e n c e to intra-
m a n y different inflammatory conditions includ- pulmonary and alveolar vessels.
ing gout, herpetic w h i t l o w , rheumatoid arthritis, Renal tumors have a predilection for bone
a n d osteomyelitis.The radiographic findings m a y m e t a s t a s e s w i t h u p t o 30 p e r c e n t o c c u r r i n g in
s u g g e s t t h a t t h e l e s i o n is a n osteochondroma l o n g b o n e s . In t w o - t h i r d s o f p a t i e n t s t h e r e w i l l b e
or giant cell t u m o r of b o n e . T h e literature is a solitary b o n e m e t a s t a s i s (Fusetti et al 2003).
r e p l e t e w i t h c a s e r e p o r t s of t u m o r s of t h e d i s t a l All reports of hand metastases published
phalanx presenting as a felon w i t h d e l a y s in since the study reported b y K e r i n (1983) w e r e
appropriate treatment as a consequence of r e v i e w e d to determine if t h e r e has been any
misdiagnosis (Moss 1984, Henderson 1987, change in t h e primary s o u r c e of t u m o r s that
F a r o u k et al 1990). metastasize to the h a n d . Fifty-seven such articles
 METASTATIC T U M O R S TO THE HAND AND W R I S T 189

Figure 1 Figure 2

A metastatic lung tumor presenting as an inflamed, swollen Adenocarcinoma of the colon metastatic to the index finger
distal phalanx. Acral metastases frequently masquerade as metacarpal.
either an inflammatory condition such as gout, or an infec-
tious process, such as a felon.

h a v e b e e n p u b l i s h e d s i n c e 1983. M o s t a r e s i n g l e 2 0 0 1 , S i l f e n et al 2001) a n d s e v e n o r o p h a r y n g e a l
c a s e r e p o r t s . S o m e articles d o not m e n t i o n t h e s o u r c e s (10 p e r c e n t ) ( V a n e l et al 1990, C a s t e l l o
p r i m a r y t u m o r s o u r c e a n d in o t h e r s , n o primary et al 1996, W r i g h t a n d G u d e l i s 2002). O n l y f i v e
h a d b e e n d e t e r m i n e d , at least b y t h e article's w e r e b r e a s t p r i m a r i e s ( B l o o m et al 1992, A s t h a n a
p u b l i c a t i o n d a t e . In 70 c a s e s f r o m t h i s g r o u p of et a l 2 0 0 1 , V a d i v e l u a n d D r e w 2002). O n e o r t w o
57 a r t i c l e s , t h e r e w e r e 22 l u n g p r i m a r i e s (31 per cases of metastatic melanoma (Tochigi et al
c e n t ) (Celik et a l 1998, G a l m a r i n i et al 1998, 2000), t h y m u s ( S h a n n o n et al 2 0 0 0 ) , prostate
V a n h o o t e g h e m et a l 1999, C a r v a l h o et al 2 0 0 2 , (Massraf and W a n d 1998), o v a r i a n ( T u r a n et al
M a t s u n o et a l 2002), a n d 20 p r i m a r y r e n a l t u m o r s 1990), t h y r o i d ( G i r a u d et al 1984), chondrosar-
(29 per c e n t ) ( V i n e a n d C o h e n 1996, B a u e r et al coma (Lambert et al 1992), and metastatic
1997, A d e g b o y e g a et al 1999, G h e r t et al 2 0 0 1 , a c r o s p i r o m a ( G o r t l e r et al 2001) w e r e i n c l u d e d in
T o l o et al 2002). T h e r e w e r e 11 b o w e l t u m o r s t h e s e 57 r e p o r t s . T h i s w o u l d suggest a falling
(16 p e r c e n t ) ( M e n d e z L o p e z et a l 1997, incidence of carcinoma of the breast as the
U m e b a y a s h i 1998, O k a d a et a l 1999, C h a n g et al s o u r c e of h a n d m e t a s t a s e s .
190 T U M O R S O F T H E HAND

Management of tumors
metastatic to the hand
Bone metastases
The management of osseous metastases
d e p e n d s upon several key circumstances includ-
i n g t h e o v e r a l l h e a l t h of t h e p a t i e n t a n d e s t i m a t e d
s u r v i v a l t i m e of t h e s p e c i f i c t u m o r . F o r e x a m p l e ,
t h e p r o g n o s i s for a m e t a s t a t i c l u n g t u m o r is m u c h
m o r e dismal than for a renal tumor.
T h e g o a l of m a n a g e m e n t is palliation in m o s t
c a s e s . In m o s t c i r c u m s t a n c e s , t h e p r i m a r y s y m p -
t o m is p a i n . T h e r e f o r e , if t h e t u m o r is in a distal
l o c a t i o n , for e x a m p l e t h e p h a l a n x , t h e n a m p u t a -
t i o n t h r o u g h t h e next most proximal joint will
p r o v i d e g o o d p a i n relief a n d t h i s is t h e r e c o m -
mendation of m o s t . T h e m a n a g e m e n t of more
proximal lesion, for e x a m p l e , in t h e c a r p u s is
m o r e controversial a n d individualized. M o s t sur-
g e o n s r e c o m m e n d excision of the t u m o r f o l l o w e d
b y radiation t h e r a p y . L o c a l r a d i a t i o n t h e r a p y to
solitary i n t r a o s s e o u s l e s i o n s h a s a l s o b e e n effec-
t i v e palliation. A m p u t a t i o n of a p h a l a n x , digit, o r
r a y is r e c o m m e n d e d for m o s t s o l i t a r y p h a l a n g e a l ,
metacarpal, or metatarsal lesions when the
e x p e c t e d p e r i o d of s u r v i v a l of t h e p a t i e n t e x c e e d s
a f e w m o n t h s ( H e a l e y et al 1986).

Soft tissue metastases

F o r t u m o r s m e t a s t a t i c to t h e soft t i s s u e s of t h e
h a n d , t h e g o a l is c o n t r o l of l o c a l d i s e a s e . M o s t
s u r g e o n s r e c o m m e n d ' e n b l o c ' r e s e c t i o n of t h e
t u m o r either w i t h or w i t h o u t post-resection radia-
tion therapy. W h e t h e r or not reconstruction is
r e q u i r e d is d e t e r m i n e d b y t h e s i g n i f i c a n c e of t h e
functional loss following resection a n d the prog-
nosis for s u r v i v a l .

b
Outcomes
Figure 3
A s mentioned above, o u t c o m es depend primarily
Renal cell tumor metastatic to the triquetrum. The plain
o n t h e n a t u r e of t h e p r i m a r y t u m o r . S u r v i v a l h a s
radiograph (a) shows osteolysis of the triquetrum although
this is masked by superimposition of the pisiform. The mag- v a r i e d g r e a t l y ( A m a d i o et al 1987). F o r e x a m p l e ,
netic resonance image (b) further delineates the lesion. 18 p a t i e n t s w h o d e v e l o p e d a c r a l m e t a s t a s e s to
(Courtesy of William Cooney.) (Reproduced from Tolo et al h a n d s a n d f e e t , w e r e t r e a t e d b y local e x c i s i o n o r
2002 with permission.) amputation plus or m i n u s radiation. Of the entire
 METASTATIC T U M O R S TO THE HAND AND W R I S T 191

g r o u p , t h e m e a n s u r v i v a l w a s 14 m o n t h s with Celik G , Saryal S , Enacar N (1998) Mixed squamous and

a m e d i a n s u r v i v a l t i m e of 5 m o n t h s . Of t h o s e small cell lung carcinoma with separate histologic sub-
d y i n g of d i s e a s e , t h e i r m e a n s u r v i v a l t i m e w a s types in e y e and phalanx metastases, J Exp Clin Cancer
Res 17:129-30.
8 months, median 5 months.
In t h e M a y o s e r i e s p u b l i s h e d in 1987 ( A m a d i o
Chang HC, Lew K H , L o w CO (2001) Metastasis of an
et al 1987), t r e a t m e n t c o n s i s t e d of l o c a l e x c i s i o n
adenocarcinoma of the stomach to the 4th metacarpal
o r r a d i a t i o n for p r o x i m a l l e s i o n s a n d a m p u t a t i o n
bone, Hand Surg 6:239-42.
f o r distal l e s i o n s . A l t h o u g h t h e m e d i a n s u r v i v a l
w a s only 5 m o n t h s , five patients lived longer than Farouk A R , McBride D J , Bingham J (1990) A case of
2 y e a r s ; i n c l u d i n g t w o w h o h a d n o further evi- tumour simulating pulp space infection, Br J Clin Pract
d e n c e of m a l i g n a n t d i s e a s e . 44:737-8.
Because occasional patients with tumors
m e t a s t a t i c to t h e h a n d s u r v i v e for l o n g p e r i o d s if Frassica F J , Amadio P C , W o l d LE et al (1989) Primary
p r o p e r l y d i a g n o s e d a n d t r e a t e d , it is i m p o r t a n t t o malignant bone tumors of the hand, J Hand Surg Am
consider this diagnosis when faced with any 14:1022-8.
a g g r e s s i v e lytic o r blastic l e s i o n of t h e h a n d a n d
w r i s t in a patient in t h e fifth d e c a d e of life o r older. Fusetti C, Kurzen P, Bonaccio M et al (2003) Hand
metastasis in renal cell carcinoma, Urology 62:141.

Galmarini C M , Kertesz A , Oliva R et al (1998) Metastasis

References of bronchogenic carcinoma to the thumb, Med Oncol
15:282-5.
Abrahams T G (1995) Occult malignancy presenting as
metastatic disease to the hand and wrist, Skeletal Ghert M A , Harrelson J M , Scully S P (2001) Solitary renal
Radiol 24:135-7. cell carcinoma metastasis to the hand: the need for
w i d e excision or amputation, J Hand Surg Am
Adegboyega PA, Adesokan A , Viegas S F (1999) 26:156-60.
Acrometastasis in renal cell carcinoma, South Med J
92:1009-12. Giraud C, Hoff J , Manelfe A et al (1984) Pathological
fracture of the thumb disclosing thyroid cancer, Presse
Amadio P C , Lombardi R M (1987) Metastatic tumors of Med 13:100.
the hand, Hand Surg Am 12:311-16.
Glicenstein J , Ohara J , Leclercq C (1988) Tumors of the
Asthana S , Deo S V , Shukla N K , Raina V (2001) Hand. Springer-Verlag: Berlin: 207-9.
Carcinoma breast metastatic to the hand and the foot,
Australas Radiol 45:380-2. Gortier I, Koppl H, Stark G B , Horch RE (2001) Metastatic
malignant acrospiroma of the hand, Eur J Surg Oncol
Bauer P, Saalfeld U, Schmidt G , Partecke BD (1997) A 27:431-5.
rare case of metastasis of urinary bladder carcinoma in
the pisiform bone. A case report, Handchir Mikrochir Healey J H , Turnbull A D , Miedema B, Lane J M (1986)
PlastChir 29:154-7. Acrometastases. A study of twenty-nine patients with
osseous involvement of the hands and feet, J Bone
Bloom RA, Sulkes A , Freilick G , Libson E (1992) Breast Joint Surg Am 68:743-6.
metastases to bones of the extremities: simultaneous
involvement of all four limbs, Clin Oncol (R Coll Radiol) Henderson J J (1987) Metastatic carcinoma in the hand
4:58-9. presenting as an acute paronychia, Br J Clin Pract
41:805-6.
Carvalho Hde A , Tsai P W , Takagaki T Y (2002) Thumb
metastasis from small cell lung cancer treated with radi- Kerin R (1983) Metastatic tumors of the hand. A review
ation, Rev Hosp Clin Fac Med Sao Paulo 57:283-6. of the literature, J Bone Joint Surg Am 65:1331-5.

Castello J R , Garro L, Romero F et al (1996) Metastatic Lambert D, Escallier F, Collet E et al (1992) Distal
tumours of the hand. Report of six additional cases, J phalangeal metastasis of a chondrosarcoma presenting
Hand Surg Br 21:547-50. initially as bilateral onycholysis, Clin Exp Dermatol
17:463-5.
192 T U M O R S OF THE HAND

Massraf A B , W a n d J S (1998) Haemorrhagic secondary Turan I, Sjoden G O , Kalen A (1990) Ovarian carcinoma
prostatic metastasis of the terminal phalanx of the metastasis to the little finger. A case report, Acta Orthop
thumb, Injury 29:243-5. Scand 61:185-6.

Matsuno Y, Watanabe K, Satoh H et al (2002) Thenar Umebayashi Y(1998) Metastasis of esophageal carci-
metastasis from lung cancer, Eur J Cancer Care (Engl) noma manifesting as whitlow-like lesions, J Dermatol
11:61-2. 25:256-9.

Mendez Lopez J M , Garcia M a s R, Salva Coll G (1997) Vadivelu R, Drew S J (2002) Phalangeal metastasis
Metastasis of an adenocarcinoma of the colon to the 1st secondary to an occult breast carcinoma, Arch Orthop
metacarpal bone, Ann Chir Main Memb Super 16:134-7. Trauma Surg 122:530-1.

Moss A L (1984) Secondary carcinoma simulating a pulp Vanel D, Luboinski B , Micheau C et al (1990) Phalangeal
infection, Injury 16:196-7. metastases of cancer of the mouth. A n uncommon
mode of propagation, J Radiol 71:237-44.
Okada H, Qing J , Ohnishi T, W a t a n a b e S (1999)
Metastasis of gastric carcinoma to a finger, Br J Vanhooteghem O, Dumont M , Andre J et al (1999)
Dermatol 140:776-7. Bilateral subungual metastasis from squamous cell
carcinoma of the lung: a diagnostic trap! R e v Med Liege
S h a n n o n F J , Antonescu CR, Athanasian E A (2000) 54:653-4.
Metastatic thymic carcinoma in a digit: a case report, J
Hand Surg Am 25:1169-72. Vine J E , Cohen PR (1996) Renal cell carcinoma meta
static to the thumb: a case report and review of sub
Silfen R, Amir A,Tobar A , Hauben D J (2001) The digital ungual metastases from all primary sites, Clin Exp
pulp as a presenting site of metastatic esophageal Dermatol 21:377-80.
carcinoma, Ann Plast Surg 46:183-4.
Wright J R , Gudelis S (2002) Digital necrosis associated
Tochigi H, Nakao Y, Horiuchi Y, Toyama Y (2000) with squamous cell carcinoma of the tonsil, Head Neck
Metastatic malignant melanoma in the hand muscle - a 24:1019-21.
case report, Hand Surg 5:69-72.

Tolo ET, C o o n e y W P , Wenger DE (2002) Renal cell carci-

noma with metastases to the triquetrum: case report,
J Hand Surg Am 27:876-81.
INDEX

acrolentiginous melanoma 124 blue rubber bleb nevus syndrome 106

adenocarcinoma, metastatic 189 bone tumors,
A I D S 125 benign 7
A J C C classification of soft tissue biopsy 4
sarcomas 148 chondromas of hand 39-45
amelanotic nail unit melanomas 124 imaging of 15
aneurysmal bone cysts, imaging importance 1
of 16 incidence 1,12
aneurysms, incidence 11, 12 malignant 7
angiodysplasias, incidence 11 osteoid osteoma 55-71
angiography, 3 osteosarcoma, Cooperative Osteosarcoma
MR-enhanced 23 Study Group 175-81
angioleiomyoma 110 principles 1
radiologic therapy 118 surgical management 6
angiolymphangioma 108 Bourneville's tuberous sclerosis 82
angiomas, incidence 11, 12 Bowen's disease 12
angiosarcoma 109
radiologic therapy 118 café-au-lait spots 83
argon laser in glomus tumors 51 capillary malformations 105
arteriography, carpals, osteoid osteoma in 56
of arteriovenous malformations 108 cavernous hemangioma 105
of osteoid osteoma 65 chemotherapy,
arteriovenous malformations 107 for osteosarcoma 179
arthrography 3 use in soft tissue sarcoma 168
aspiration 2 childhood melanosis 125
atypical melanocytic hyperplasia 124 chondromas 39-45
clinical presentation 39
benign tumors, diagnosis 42
imaging of 19 etiological aspects 43
in bone 7 histology 43
in soft tissue 7 incidence 12, 39
biopsy 2 locations 39
excisional 127 multiple 41
for bone tumors 4 pathological fracture of 40
limited surgical 127 periosteal 42
of soft tissue tumors 3, 149 radiologic features 40
core needle biopsy 150 recurrence 45
excisional 150 spontaneous course 43
fine-needle aspiration 150 surgery for 44
incisional 150 treatment 44
open vs closed 4 X-rays 40, 41
principles of 147-53 chondrosarcoma 183-6
punch biopsy 126 cf chondroma 44
technique 4 cf enchondroma 185
techniques for melanonychia 126 characteristics 183
bleeding of hemangiomas 104 development from other tumors 184
194 INDEX

histology of 183 Enneking staging for soft tissue tumors 149

incidence 183 epidermal cysts 12
macroscopic appearance 183 epithelioid sarcoma 158, 159
X-rays 18,184 surgery 169
classifications,
A J C C classification of soft tissue sarcomas 148 fibrolipoma, M R I scan 21
Enneking staging 149 fibromatosis 12
histological of mesenchymal tumors of hand 155 fibrosarcoma, radicalization 171
of giant cell tumors of the tendon sheath 28 fibrous histiocytoma, excision and
of hemangiomas 100, 102 reconstruction for 168
of soft tissue tumors 147 fibrous residuum 104
by behavior 157 fibrous xanthoma, see giant cell tumors
of surgical margins for osteosarcoma 178 of the tendon sheath
of tumors 5 fine-needle aspiration 150
of vascular tumors 99-110 fingers,
pT classification of melanoma 135 chondroma of hand 44
clear cell sarcoma 160 giant cell tumors of the tendon
computed tomography (CT) scans 3 sheath 28, 29
CT-assisted percutaneous surgery 62 macrodactyly in neurofibroma 85
for osteoid osteoma 60 osteoid osteoma of 58
of schwannomas 76 subungual tumor, imaging of 23
congenital hemangiomas 101 vascular malformations, imaging of 22
congenital lymphedema 87 foreign bodies cf longitudinal
congenital nevus 125 melanonychia 122
Cooperative Osteosarcoma Study Group 175-81
core needle biopsy 150 ganglia, intraosseous 12
cutaneous neurofibroma 83 ganglia, incidence 11
ganglion cysts,
desmoid tumor, M R I scans 24 imaging of 19
differential diagnosis, MRI scan 19
chondroma of hand 42 gender and incidence of disease and 11, 12
chondrosarcoma 184, 185 genetics,
dangers of misdiagnosis 77, 78 molecular genetics of malignant peripheral
diagnostic algorithm for osteoid osteoma 71 nerve sheath tumors 94
giant cell tumors of the tendon sheath 29 of chondrosarcoma 185
glomus tumors 49, 50 of neurofibromatosis 82, 89
longitudinal melanonychia 122 giant cell tumors,
macrodactyly 86 imaging of 16
metastases to hand and wrist 188 incidence 11
nail matrix nevi 125 X-ray, 18
neurofibromas 81 giant cell tumors of the tendon sheath 27-36
synovial sarcoma 158 causes 27
schwannomas 75 cf giant cell tumor of the bone 35
classification 28
Ehler-Danlos syndrome 83 clinical features 28
electromyography, clinical status 29
for malignant peripheral nerve sheath tumors 95 complications 33
for schwannomas 77 after surgery 33
embolotherapy, differential diagnosis of 29
in arteriovenous malformations 116 extensions of 29
in venous malformation 115 histology of 31
products used in 117 histopathology of 29
enchondromas, imaging 19
cf chondrosarcoma 185 incidence 27
imaging of, 16 location 28, 29
MRI scans 17 MRI scans 20, 31
X-ray 17 peroperative view 3 1 , 32, 33, 34
see also chondromas recurrence rates 35
 INDEX 195

treatment 30 growth 101

typical sizes 30 imaging of 20
X-ray 20 incidence 20
gliomas in neurofibromatosis 84 involution 101, 102
glomagiomatosis 51 skeletal distortion by 104
glomus body, subcutaneous 103
description of normal 47 surgical management 104
glomus tumors, tuberous 103
anatomic site 48 ulceration of 103, 104
classification of malignancy 52 hemangiopericytoma 110
clinical features 48 radiologic therapy 118
clinical findings 48 Hippel-Lindau angiomatosis 82
clinical tests for 49 Hippocrates 99
diagnosis 48 histiocytofibromas, incidence 12
distribution by age and sex 48 histology,
etiology of 47 of chondroma of hand 43
imaging of 21 of malignant melanoma 134
MRI scans 50 histopathology,
scintigraphy 50 of giant cell tumors of the tendon
ultrasound 49 sheath 29
X-rays 49 of malignant peripheral nerve sheath
incidence 12, 21 tumors 93
malignant transformation of 51 of osteoid osteoma 61
of hand 47-52 Hutchinson's sign 123
pathology of, possible causes 125
macroscopic 47
microscopic 47 imaging,
peroperative views 51 angiography 3
radiologic therapy 118 arthrography 3
recurrence of 51 bone tumors 15-18
size and numbers of lesions 48 choice of 2
subungual, CT scans 3
angiography 23 MRI scans 3
MRI scans 23 relative signal intensity of tissues 15
X-ray 23 on hands and fingers 15-26
symplastic 51 scintigraphy 3
treatment, surgery 50 soft tissue tumors 18-25
growth factors, role in hemangioma ultrasonography 3
development 101 X-rays 3
immunohistochemistry
hamartoma, fibrolipomatous in macrodactyly 87 of clear cell sarcoma 160
hand tumors, of epithelioid sarcoma 159
at I'lnstitut de la Main 11-13 of malignant peripheral nerve sheath tumors 93
bone tumors 15 of schwannoma 75
imaging of 15-26 indomethacin in glomus tumors 51
hemangioendothelioma 110 inflammatory myxohaline tumor 161, 162
hemangioma, intraosseous ganglion cysts, imaging of 16
radiologic therapy 118 involution 101, 102
sclerosing, see giant cell tumors of the
tendon sheath Klipper-Trénaunay-Weber syndrome 86
hemangiomas,
bleeding of 104 Laugier-Hunziker syndrome 125
classification of 102 lentigo,
clinical examination 102 cf subungual melanoma 124
complications 103 localization and excision 127
congenital 101 of nail unit 125
description 100 lentigo maligna 134
evolution 101 Leri's melorheostosis 85
196 INDEX

lipomas, melanoma,
imaging of 19 acral lentiginous 134
incidence 12, 19 acrofentiginous 124
M R I scans 21 amelanotic nail unit 124
Lish nodules 83 atypical melanocytic hyperplasia and 1
longitudinal melanonychia, s e e classification 134
melanonychia clinical diagnosis 133
lung cancer, metastatic 189 clinical experience with 143
lymphangioma 108 definitive growth under nail 140
in babies 109 diagnostic procedures 135
incidence 11, 12 early-evolving 125
radiologic therapy 114 follow-up schedule 136
lymphangiosarcoma 110 fungating cutaneous 140
radiologic therapy 118 future applications for 144
lymphatic malformations 108 histological diagnosis 134
lymphoscintigraphy 142 histological type 135
incidence 133
macrodactyly, lentigo maligna 134, 134
differential diagnosis 86 location 133
in neurofibroma 85 malignant 133-7
in neurofibromatosis 86 management 144
of phalanx 88 margins for excision 136
typical findings 87 metastatic, systemic treatment of 137
Mafucci's syndrome 86 micrometastasis 142
magnetic resonance imaging nail unit 123
(MRI) scans 3 nodular 134
for osteoid osteoma 61 of hand 139-45
of schwannomas 76 excision margins 140
relative signal intensity of tissues 15 management 139
malignant tumors, vascular tumors 109 metastatic 141
malignant melanoma 133-7 tumor thickness 140
malignant peripheral nerve sheath tumors, pT classification of 135
cf schwannomas 75 sentinel node biopsy 139-45, 141
malignant peripheral nerve sheath subungual 140
tumors ( M P N S T ) 93-7 cf lentigo 124
clinical findings 94 prognosis 124
histology of 93 superficial spreading 134
immunohistochemistry of 93 treatment 136
in neurofibromatosis 95 melanomas 12
incidence 94 melanonychia,
investigations of 95 biopsy techniques for 126
macroscopic appearance 93 causes of 123
metastasis 96 description 121
microscopic appearance 94 epidemiological studies 121
molecular genetics of 94 excisional biopsy 127
recurrence 96 central melanonychia 128, 129
after amputation 96 large bands 130
treatment 95 lateral melanonychia 127, 128
malignant tumors, lentigo cf subungual melanoma 124
imaging of 17 limited surgical biopsy 127
in bone 7 management 121-130
incidence 7,13 monodactylous 123
radiologic therapy 118 polydactylous 123
transformation of glomus tumors 51 pseudo- 123
malnutrition 125 punch biopsy 126
median nerve, fibrolipoma 21 simulators of,
melanocytic nevus 125 foreign body under nail plate 122
 INDEX

mycotic infection 122 schematic representation 80

onychopapilloma 122 terminology 79
meningiomas, in neurofibromatosis 84 treatment 89
mesenchymal tumors of hand, histological neurofibromatosis
classification 155 cafe-au-lait spots in 83
metacarpals, clinical symptoms 82
chondrosarcoma of 18, 183 cutaneous neurofibroma in 83
enchondroma of 17 genetics of 82
giant cell tumor 18 macrodactyly in 86
osteoid osteoma of 60, 67 malignant peripheral nerve sheath tumors in 95
metastases, nerve tumors seen in 84
bone 190 peroperative views 84
identification of 142 skeletal anomalies 85
management of 190 type 1 82
melanoma 137 type 2 82
of hand, 141 neurofibrosarcoma 82
of epithelioid sarcoma 160 nodular tenosynovitis, see giant cell tumors
osteosarcoma 180 of the tendon sheath
soft tissue 190 Nonne-Milroy disease 87
to hand and wrist 187-91 non-steroidal anti-inflammatory drugs (NSAIDs),
differential diagnosis of 188 use in osteoid osteoma 61
incidence 187
management, 190 Ollier's disease 86
occurrence 188 onychopapilloma 122
outcomes 190 osteochondromas, incidence 12
typical findings 187 osteoid osteoma 55-71
typical sites of 187 age distribution 64
metastasis, of malignant peripheral nerve appearance of 58
sheath tumors 96 arteriography 6 1 , 65
mixed tumor of soft tissue 162 CT scan 57, 60
mixoid fibromas 12 diagnosis 68, 69
molluscum fibrosum, see cutaneous neurofibroma diagnostic algorithm 69, 71
mucous cysts, incidence 11 histopathology of 61
multiple endochondromatosis of Ollier 4 1 , 42 incidence 55
myoepithelioma 162,163 MRI scans for 61
myxoinflammatory fibroblastic sarcoma 161 of metacarpophalangeal joint 60
of phalanx 58, 59
nail matrix nevi 125 of ulnar 57
nail tumors, incidence 12 pain as principal symptom 63
nail unit melanoma 123 peroperative views 59, 65, 67, 68, 69, 70
nerve tumors, personal series of results 63
imaging of 24 recurrence of 63
incidence 12 symptoms and signs 55
neurilemomas, see schwannomas three-phase bone scan for 60
neurinomas, see schwannomas treatment,
neurocutaneous syndrome 82 choice of 62
neurofibromas 79-90 classical surgery 62
diagnosis 81 medical 61
embryology 79 minimally invasive surgery 62
imaging of 24 X-rays 56, 57, 59
in neurofibromatosis 84 osteosarcoma,
incidence 12, 79 chemotherapy 179
macroscopic appearance 80 clinical signs 175
microscopic appearance 81 Cooperative Osteosarcoma Study Group 175-81
pathology 80 diagnosis 175
peroperative views 85, 87, 88 epidemiology 175
prognosis 81 incidence 176
198 INDEX

interdisciplinary multicenter trial 177 diagnosis 155

location of 176 epidemiology of 156
metastatic 180 epithelioid 158
prognostic factors 180 excision of recurrent 169
surgery for 178 immunohistochemistry of 159
treatment strategy 177 metastatic 160
grading system of 148
PET scans for malignant peripheral nerve histology 155
sheath tumors 95 soft tissue imaging of 24, 25
Peutz-Jeghers syndrome 125 surgical excision of 167
phakomatosis 82 synovial 156
phalanx, biphasic 158
osteoid osteoma on 68, 69, 70 differential diagnosis of 158
phlebectasia 106 monophasic 158
phleboliths 106 schwannomas 75-8
physical examination 2 clinical findings 76
pigmented villonodular synovitis, see giant cell common fibular nerve 77
tumors of the tendon sheath differential diagnosis 75
plexiform fibrohistiocytic tumor 161,162,163 histology 75
politeal nerve, malignant peripheral nerve imaging of 76
sheath tumors on 94 immunohistochemistry 75
port-wine stains 105 in neurofibromatosis 83, 84
primitive neuroectodermal tumor, radiologic incidence 12
MRI scan 24
therapy 118
locations 76
prostaglandin inhibition therapy, in glomus
tumors 51 median nerve in upper arm 75
Proteus syndrome 86 misdiagnosis 78
pseudo-melanonychia 123 pathology 80
pseudoarthrosis 85 radial nerve at elbow 76
treatment of 89 recurrence of 78
pyogenic granulomas 12 schematic representation 80
treatments 77
radiologic therapy, scintigraphy 3
of glomus tumors 51 sclerosing agents 115
of lymphatic malformations 114 sclerosing hemangioma, see giant cell tumors
of vascular malformations 113-18 of the tendon sheath
of venous malformations 113 scoliosis 85
use in soft tissue sarcoma 168 sebaceous cysts 12
reconstruction in soft tissue sarcoma 168 sentinel node biopsy of melanoma 139-45
recurrence, clinical experience with 143
of chondroma of hand 45 future applications 144
of chondrosarcoma 185 management 144
of giant cell tumors of the tendon sheath 35 technique 142
of glomus tumors 51 skeleton,
of lentigo maligna 134 abnormalities in neurofibromatosis 85
of malignant peripheral nerve sheath tumors 96 distortion of by hemangiomas 104
of myxoinflammatory fibroblastic sarcoma 162 skin tumors,
of osteoid osteoma 63 incidence 12
of schwannomas 78 types 13
renal cell tumor, metastatic 190 split fat sign 24
spongy venous malformations 106
sarcoma, soft tissue tumors, 3
myxoinflammatory fibroblastic 161 soft tissue tumors,
of hand and wrist 167-72 benign lesions of upper extremity 7
sarcomas, biopsy of 149
clear cell 160 core needle biopsy 150
immunohistochemistry of 160 excisional 150
 INDEX 199

fine-needle aspiration 150 for giant cell tumors of the tendon sheath 30
incisional 150 for glomus tumors 50
classification of by behavior 157 for hemangiomas 104
clear cell sarcoma 160 for osteoid osteoma
clinical evaluation 148 classical 62
distribution of types 11 minimally invasive 62
Enneking staging for 149 percutaneous CT-assisted technique 62
epidemiology 147 for osteosarcoma 178
epithelioid sarcoma 158 for schwannomas 77
errors in treatment 147 for soft tissue sarcomas 167
evaluation algorithm 152 for soft tissue tumors 6
fibrous histiocytoma 168 incisions 151
giant cell tumor of the tendons 27-35 intralesional excision 151
glomus tumors 47-52 marginal excision 151
histological classification of 155 radical resection 151
imaging of 18-25 wide resection 151
incidence 11,12 for subungual melanoma 126
inflammatory myxohaline tumor 161 for venous malformations 106,107
malignant 24, 155-63 of vascular tumors 99-110
malignant peripheral nerve sheath peroperative view, osteoid osteoma 59
tumors ( M P N S T ) , 93-7 peroperative views,
management 1 epithelioid sarcoma 169
most common types 18 fibrosarcoma 171
M R I scans of 149 for glomus tumors 51
myoepithelioma 162 for venous malformations 107
myxoinflammatory fibroblastic sarcoma 161 giant cell tumors of the tendon sheath
neurofibromas 79-90 31,32, 33,34
plexiform fibrohistiocytic tumor 162 neurofibromatosis 84
principles 1 osteoid osteoma 65, 67, 68, 69, 70
radiographic evaluation 149 schwannomas 75, 76, 77
sarcomas 155, 167-172 synovial sarcoma 156
diagnosis 155 biphasic 158
epidemiology 156 differential diagnosis of 158
radical surgery for 171 monophasic 158
results of surgery 170 synovialosarcoma, radiologic therapy 118
surgical excision 167 synoviasarcoma, MRI scan 24
schwannomas 75-8 synovioma, benign, see giant cell tumors of the
staging and grading of 147 tendon sheath
surgical management 6
intralesional excision 151 telangiectasic stains 105
marginal excision 151 three-phase bone scan 60
radical resection 151 treatment, of giant cell tumors of the tendon sheath 30
wide resection 151 tuberous hemangiomas 103
synovial sarcoma 156
Sturge-Weber syndrome 85 ulceration 103
Sturge-Weber's angiomatosis 82 ulna, osteoid osteoma on 57, 65, 66
subcutaneous hemangiomas 103 ulnar nerve, schwannoma 24
subungual melanoma, surgical treatment 126 ultrasonography 3
surgery, peroperative views, in neurofibromas classification of vascular anomalies by 100,101
85, 87, 88 color Doppler 22
surgery 5
amputation cf limb-sparing procedures 168 vascular anomalies,
for arteriovenous malformations 108 classification 100
for bone tumors 6 ultrasonography of 100, 101
for chondroma of hand 44 vascular malformations,
for chondrosarcoma 185 arteriovenous malformations,
for fibrous histiocytoma 168 arteriography 108
200 INDEX

description 107 vascular tumors 99-110

Doppler results 116 classification 99-110
embolotherapy 116 history of 99
explorations 107 imaging of 20
giant 108 incidence 11
opacification methods 116 malignant 109-110
radiologic therapy 115 morphologic appearance 113
surgical management 108 radiologic therapy 113-118,118
capillary malformations 105 surgery 99-110
color Doppler view 22 terminology used in 99
imaging of 20, 22 types of 12
lymphatic malformations 108 see also vascular malformations
diagnosis 109 venous malformations 105
management 109 von Recklinghausen's disease 12, 81
radiologic therapy 114 neurofibromas in 24
sclerosing agents used in 114,115 symptoms 82
M R I scans 22 see neurofibromatosis
radiologic therapy 113-18
see also vascular tumors wrist, synoviasarcoma of 24
spongy 106
venous malformations 105 X-rays 3
diagnosis 106 chest X-ray in melanoma 140
embolotherapy 115 of schwannomas 76
histology 106 xanthoma, fibroid, see giant cell tumors of
M R I scan, 107 the tendon sheath
peroperative view 107
radiologic therapy 113
treatment 106