TUMORS OF TH EAR

AND UPPER
RESPIRATORY TRACTS

SAJITH SUBRAMANIAN
GRP 64
BASIC ANATOMY
OF EAR
• The ear is made up of three parts: the
outer, middle, and inner ear. All three parts
of the ear are important for detecting
sound by working together to move sound
from the outer part through the middle and
into the inner part of the ear. Ears also help
to maintain balance.
• The outer ear consists of skin and cartilage,
called the auricle, or pinna, and the ear
canal. The ear drum, or tympanic
membrane, is a thin membrane that
separates the outer ear from the middle
ear. The middle ear is an air-filled chamber
containing three small bones called ossicles.
UPPER
RESPIRATORY
SYSTEM
• The major passages and
structures of the upper
respiratory tract include
the nose or nostrils, nasal
cavity, mouth, throat
(pharynx), and voice box
(larynx). The respiratory
system is lined with a
mucous membrane that
secretes mucus. The mucus
traps smaller particles like
pollen or smoke.
EAR TUMORS
• An ear tumor is a mass or lump of abnormal cells that forms in the ear.
Most ear tumors are benign or not cancerous. But some ear tumors are
malignant (cancerous).
• Ear tumors can form in any part of the ear, including the inner ear,
middle ear and outer ear. They may affect your hearing.

• Both cysts and tumors can cause a bump or lump in the ear.
• Cysts are small sacs that often contain fluid. They may also have solid
material. Most cysts are not cancerous.
• Tumors are solid masses of tissue that may or may not be cancerous.
• What are the types of benign (noncancerous)
ear tumors?
• Noncancerous ear tumors can block your ear canal, leading to earwax buildup. Types of benign
ear tumors include:
• Acoustic neuromas (also called vestibular schwannomas) form on the vestibular nerve. This
nerve in your inner ear connects to your brain.
• Adenomas are rare noncancerous tumors that develop in the middle ear.
• Cholesteatomas are sacs of fluid, air or skin cells that form behind the eardrum in the middle
ear. They can lead to hearing loss if not treated.
• Glomus tympanicum paraganglioma affects the tympanic nerve. This nerve in the middle ear
connects to the eardrum.
• Keloids are a type of fibrous scar tissue. They can form after an ear piercing or trauma to the
outer ear.
• Osteomas and exostoses form on bones in the external ear canal (benign bone tumor).
• Sebaceous cysts contain skin cells and oil. They can develop in the ear canal, behind the ear or
on the earlobe. They are also called epidermal inclusion cysts.
What are the types of malignant (cancerous)
ear tumors?
• Cancer can form inside or on the outside of your ear. Ear cancer is rare.
• Most cancer that affects the ear is skin cancer. Skin cancer may first appear on the outer
ear. Skin cancers that affect the ear include:
• Basal cell carcinoma.
• Melanoma.
• Squamous cell carcinoma.
• Cancers that directly affect the middle or inner ear are even more uncommon. They
include:
• Ceruminous adenoma forms in the cells that make earwax. This cancer doesn’t spread,
but it can destroy parts of the ear canal.
• Rhabdomyosarcoma is a rare childhood cancer that affects muscle tissue. It may
develop in the head or neck, including the middle ear.
• What causes an ear tumor?
• Tumors occur when your body makes new cells faster than usual. Sometimes, old,
damaged cells don’t die off the way they should. Clumps of old and new cells group
together, forming a tumor.
• Cancerous tumors occur when the cells grow uncontrollably. Untreated, these
malignant cells may spread to other locations in your body (metastatic cancer).
• What are the risk factors for ear tumors?
• People of all ages, including children, can get ear tumors. Factors that increase the
chances of developing an ear tumor include:
• Chronic ear infections.
• Ear piercings.
• Inherited conditions, such as neurofibromatosis (NFS).
• Prior radiation exposure.
• Repeated exposure to cold water, such as from scuba diving (surfer’s ear).
• Smoking, including exposure to secondhand smoke.
 What are the symptoms
of an ear tumor?
• Symptoms of an ear tumor vary depending on the
tumor type and the part of the ear it affects. You may
be able to feel a bump on the outer part of the ear.
• Signs of an ear tumor include:
• Dizziness or balance problems.
• Ear bleeding or discharge.
• Ear pain.
• Headaches.
• Hearing loss.
• Nonhealing wound or sore.
• Skin discoloration, new moles or changes to a mole.
• Swollen lymph nodes.
• Tinnitus (ringing in the ear).
• Weak facial muscles.
Neuroendocrine adenoma middle ear

• Neuroendocrine adenoma middle ear (NAME) is a tumor which

arises from a specific anatomic site: middle ear.NAME is a benign
glandular neoplasm of middle ear showing histologic and
immunohistochemical neuroendocrine and mucin-secreting
differentiation (biphasic or dual differentiation).
• Neuroendocrine adenoma of the middle ear has gone by several
different names, including middle ear adenoma, carcinoid
tumor,amphicrine adenoma, adenocarcinoid, and adenomatoid
tumor of middle ear. The various names have created some confusion
about this uncommon middle ear tumor. Regardless of the name
applied, the middle ear anatomic site must be known or confirmed.
• Signs and symptoms
• This uncommon tumor accounts for less than 2% of all ear tumors. While patients present
with symptoms related to the middle ear cavity location of the tumor, the tumor may
expand into the adjacent structures (external auditory canal, mastoid bone, and eustachian
tube). Patients come to clinical attention with unilateral (one sided) hearing loss, usually
associated with decreased auditory acuity, and particularly conductive hearing loss if the
ossicular bone chain (middle ear bones) is involved. Tinnitus (ringing), otitis media, pressure
or occasionally ear discharge are seen. At the time of otoscopic exam, the tympanic
membrane is usually intact, with a fluid level or mass noted behind the ear drum. Even
though this is a "neuroendocrine" type tumor, there is almost never evidence of
neuroendocrine function clinically or by laboratory examination.
• Imaging findings
• In general, an axial and coronal bone computed tomography study without contrast will
yield the most information for this tumor. The tumor appears as a soft tissue mass usually
within a well-aerated mastoid bone. The features of chronic otitis media are not usually
seen. Bone invasion and destruction are usually not seen in this tumor which expands
within the mesotympanum (middle ear cavity). Encasement of the ossicles is usually
present. There may be an irregular margin at the periphery, especially if the tumor has been
present for a long time, with associated bone remodeling.
A hematoxylin and eosin stained intermediate power showing an
infiltrating tumor with glandular and neuroendocrine features in a
neuroendocrine adenoma of the middle ear.
 PATHOLOGICAL FINDINGS
• At the time of surgery, the tumor tends to peel away from the adjacent bones, although not the ossicles. It is usually
fragmented, soft, rubbery and white to gray-tan. Due to the anatomic confines of the region, tumors are usually <1 cm. The
tumors arise below the surface, are unencapsulated, and have an infiltrative pattern of growth, composed of many different
patterns (glandular, trabecular, cords, festoons, single cells). The tumor shows duct-like structures with inner luminal, flattened
cells and outer, basal, cuboidal cells. The cells may have an eccentrically placed nucleus. The nuclear chromatin distribution is
"salt-and-pepper", giving a delicate, fine appearance. Nucleoli are small with inconspicuous mitoses. There may be secretions in
the gland tumor. It is possible to see a concurrent cholesteatoma.
• Histochemistry
• It is possible to see intracytoplasmic as well as luminal mucinous material highlighted by a Periodic acid-Schiff (PAS) or Alcian
blue stain.
• Immunohistochemistry
• The tumor cells differential stain with markers for epithelial and neuroendocrine immunohistochemistry markers.
• Both cell types positive: pancytokeratin, CK7, CAM5.2
• Inner luminal cells positive: CK7
• Outer basal cells positive: chromogranin, synaptophysin, CD56, and human pancreatic polypeptide (HPP).
• Electron microscopy
• Scanning electron microscopy shows two distinct cell types:
• Type A: Apical dark cells with elongated microvilli and secretory mucus granules;
• Type B: Basal cells with cytoplasmic, solid, dense-core neurosecretory granules.
• In a few areas, transitional forms with features of both cell types may be present
• Diagnosis
• From a pathology perspective, several tumors need to be considered in the
differential diagnosis, including paraganglioma, ceruminous adenoma, metastatic
adenocarcinoma, and meningioma.
• Management
• The tumor must be removed with as complete a surgical excision as possible. In
nearly all cases, the ossicular chain must be included if recurrences are to be avoided.
Due to the anatomic site of involvement, facial nerve paralysis and/or paresthesias
may be seen or develop; this is probably due to mass effect rather than nerve
invasion. In a few cases, reconstructive surgery may be required. Since this is a benign
tumor, no radiation is required. Patients experience an excellent long term outcome,
although recurrences can be seen (up to 15%),especially if the ossicular chain is not
removed. Although controversial,metastases are not seen in this tumor. There are
reports of disease in the neck lymph nodes, but these patients have also had other
diseases or multiple surgeries, such that it may represent iatrogenic disease.
• Epidemiology
• Most individuals come to clinical attention during the 5th decade, although the age
range is broad (20 to 80 years). There is an equal gender distribution
Ceruminous adenocarcinoma

• Ceruminous adenocarcinoma is a malignant neoplasm derived from ceruminous glands of

the external auditory canal. This tumor is rare, with several names used in the
past. Synonyms have included cylindroma, ceruminoma, ceruminous adenocarcinoma, not
otherwise specified (NOS), ceruminous adenoid cystic carcinoma (ACC), and ceruminous
mucoepidermoid carcinoma
• Classification
• This tumor only affects the outer 1/3 to 1/2 of the external auditory canal as a primary site.
If this area is not involved, the diagnosis should be questioned. The most common tumor
type is ceruminous adenoid cystic carcinoma and ceruminous adenocarcinoma, NOS.
• Signs and symptoms
• Pain is the most common symptom, followed by either sensorineural or conductive hearing
loss, tinnitus or drainage (discharge). A mass lesion may be present, but it is often slow
growing.
• Diagnosis
• ImaginG
• Imaging studies are used to define the extent of the tumor and to exclude direct extension from the parotid gland or nasopharynx.
[
The imaging findings are usually non-specific, and cannot give a specific diagnosis.
• Pathology
• Tumors are polypoid, identified most often in the posterior canal. It is not uncommon to have ulceration of the surface squamous
epithelium. Most tumors are about 1.5 cm in greatest dimension, a limitation of the anatomic site rather than of the tumor type
itself.[ The tumors are separated into three main histologic or microscopic types:
• Ceruminous adenocarcinoma, NOS
• Ceruminous adenoid cystic carcinoma
• Ceruminous mucoepidermoid carcinoma
• All of the tumors are infiltrative into the soft tissue, benign ceruminous glands, and/or bone. The tumor may expand into the
overlying squamous surface epithelium, but it usually does not arise from the surface epithelium. The tumors are cellular, arranged
in solid, cystic, cribriform, glandular, and single cell patterns. It is uncommon to see tumor necrosis, but when it is present, it is
diagnostic of cancer. The same is true of perineural invasion. Nuclear pleomorphism is usually easily to identify, with the nuclei
containing prominent nucleoli. There are usually increased mitotic figures, including atypical forms. There are usually areas of
stromal fibrosis. Ceroid (cerumen or ear wax) is not seen in malignancies, although it is seen in benign tumors. The specific features
of each tumor type can help with the separation into adenoid cystic carcinoma or mucoepidermoid types.
• Immunohistochemistry
• Immunohistochemistry will help to show the biphasic appearance of the tumor, highlighting the basal or the luminal cells:
• Luminal cells: positive with CK7 and CD117
• Basal cells: positive with p63, S100 protein and CK5/6
• Differential diagnoses
• It is important to exclude a tumor which is directly extending into the ear canal from the parotid salivary gland, especially when
dealing with an adenoid cystic or mucoepidermoid carcinoma. This can be eliminated by clinical or imaging studies. Otherwise, the
histologic differential diagnosis includes a ceruminous adenoma (a benign ceruminous gland tumor) or a neuroendocrine adenoma
of the middle ear (middle ear adenoma).
A high power photomicrograph of a
ceruminous type adenoid cystic carcinoma.
• Management
• Wide, radical, complete surgical excision is the treatment of choice, with free surgical
margins to achieve the best outcome and lowest chance of recurrence. Radiation is
only used for palliation. In general, there is a good prognosis, although approximately
50% of patients die from disease within 3–10 years of presentation.
• Epidemiology
• This is a very rare neoplasm accounting for approximately 0.0003% of all tumors and
about 2.5% of all external ear neoplasms. Ceruminous adenocarcinomas (CA) is a rare
type of tumor and has a very small amount of literature on it. The ages to develop
ceruminous adenocarcinomas are between 21 and 92 and being most present around
48. There is no statistical difference between being male and female and developing
the tumor and there is no racial inclination. The typical treatment of CA is through
surgery and radiation. The type of cut is important with the recurrence rate. Long-term
outcomes are better when having the wide cut near the tumor. If the tumor reoccurs
there is an 83% chance of mortality and if it does not reoccur there is a 9% chance of
mortality. Older people have a lower survival rate than younger ones. The local
reoccurrence rate is 49% and the distant metastasis rate is 13%. With local
reoccurrence being at 49%, follow-up is very important
vestibular schwannoma
• A vestibular schwannoma (VS), also called acoustic neuroma, is a benign tumor that develops
on the vestibulocochlear nerve that passes from the inner ear to the brain. The tumor
originates when Schwann cells that form the insulating myelin sheath on the nerve
malfunction. Normally, Schwann cells function beneficially to protect the nerves which
transmit balance and sound information to the brain. However, sometimes a mutation in
the tumor suppressor gene, NF2, located on chromosome 22, results in abnormal production
of the cell protein named Merlin, and Schwann cells multiply to form a tumor. The tumor
originates mostly on the vestibular division of the nerve rather than the cochlear division, but
hearing as well as balance will be affected as the tumor enlarges.
• The great majority of these VSs (95%) are unilateral, in one ear only. They are called "sporadic"
(i.e., by-chance, non-hereditary). Although non-cancerous, they can do harm or even become
life-threatening if they grow to press on other cranial nerves and vital structures such as
the brainstem. Variations in the mutation determine the nature of the tumor's development.
The only environmental exposure that has been definitely associated with the growth of a VS is
therapeutic radiation exposure to the head
Symptoms of Sporadic VS

• Sporadic VSs originate within the confining bony walls of the small (ca. 2 cm long) internal auditory canal. The most
common early symptoms of these intracanalicular (IAC) VSs are gradual hearing loss and a feeling of fullness in the
affected ear, some imbalance or dizziness, and tinnitus (ringing or other noise in the ear). Gradual single-sided hearing
loss in the high frequencies is the first most obvious symptom for the great majority of patients. Headache as a
presenting symptom of VS specifically is rare; facial symptoms (facial numbness, weakness) usually occur only as the
tumor grows out of the canal and/or after therapeutic treatment. Delayed diagnosis and misdiagnosis are not unusual.
Initial hearing loss is usually subtle and may be attributed mistakenly to aging, earwax buildup, or perhaps exposure to
some loud environmental noise. A sudden hearing loss, which is uncommon, might be misdiagnosed as Ménière's
disease, an abnormality of the inner ear that also has tinnitus as a symptom. The brain's vestibular system usually
compensates for early balance problems.
• There have been cases of tumors that were actually asymptomatic until very large and at a critical stage. Tumor growth
rates are highly variable: some small VSs (perhaps 50%) do not grow at all; some few grow for a time and then shrink;
some appear dormant but suddenly grow rapidly. In general, although studies differ, VSs that grow are slow-growing at
an average rate of 1.2 to 1.9 mm per year. IAC tumors that grow beyond 1.5 cm in diameter expand into the relatively
empty space of the cerebellopontine angle, taking on the characteristic 'ice-cream-cone' appearance seen on MRIs. As
'space-occupying-lesions,' the tumors can reach 3 to 4 cm or more in size and infringe on the facial nerve (facial
expression) and trigeminal nerve (facial sensation). Advanced hearing loss and spells of true vertigo may occur. Very large
tumors are life-threatening when they press on the cerebellum or cause brainstem compression. Late symptoms of very
large VS include headache, nausea, vomiting, sleepiness, mental confusion and eventually coma.
DIAGNOSIS

• Preliminary diagnostic procedures include ear examination, hearing and vestibular testing. The auditory
brainstem response test (ABR) is a cost-effective test to see if a VS has perhaps compromised the cochlear
nerve.
• Computed tomography (CT scan) of the head will detect moderate to large sized VS but can miss small
sized VS. VS appears as isodense to surrounding brain parenchyma on CT. VS does not have calcifications
in it. Large VS will expand the size of internal acoustic meatus (IAC), thus associated with poor hearing
function because the nerves within the IAC are compressed, particularly the cochlear nerve. However,
facial nerve is less commonly affected. The main advantage of CT scan is to assess the extent of bony
involvement by VS. VS enhances when iodinated contrast is given. Contrasted CT scan of temporal
bone can done if the subject is unsuitable to undergo MRI scan.
• MRI scan is the imaging of choice because it can more accurately differentiate the mass from other
tumours such as meningioma, facial nerve schwannoma, epidermoid cyst, arachnoid cyst, aneurysm, and
brain metastasis. MRI scan also helps in surgical planning and follow-up of the tumour after surgery. [VC is
usually isointense on T1 weighted images, hyperintense on T2 weighted images, and enhances after
given gadolinium contrast.
MANAGEMENT
• Microsurgery for Sporadic VS
• The three main surgical approaches to the tumor were the translabyrinthine (incision behind the ear to
reach the bony labyrinth), the retrosigmoid (incision behind the ear to reach cerebellopontine angle) and
the middle cranial fossa (incision in front of the ear to access the IAC from above). Tumor size was a major
factor in determining approach selection.
• Radiosurgery and radiotherapy
• Radiosurgery is the delivery to the VS of a concentrated high radiation dose in a one-day session, whereas
radiotherapy involves multiple treatment sessions where the total radiation dose is spread out in
fractions over a few days or 3–4 weeks. The main objective in either case is 'tumor control' by damaging
tumor cell DNA and stopping blood vessel proliferation (angiogenesis) needed for tumor growth.
• Medical and gene therapies
• An anti-VEGF drug named bevacizumab (Avastin) was developed and showed promise in stopping this
vascular proliferation. Unfortunately, when tested for NF2 tumors, the therapy required prolonged
treatment resulting in hypertension and impaired wound healing. Clinical trials are in progress for other
drugs such as everolimus, lapatinib and mifepristone. Common aspirin has been studied as a low-risk
therapeutic option.
TUMORS OF UPPER RESPIRATORY
TRACTS
• The most common malignant tracheal tumors include adenoid cystic
carcinoma, squamous cell carcinoma, carcinoid, and mucoepidermoid
carcinomas.
• The most common benign airway tumor is a squamous papilloma
(most often related to human papillomavirus), although pleomorphic
adenomas and granular cell and benign cartilaginous tumors also
occur.
SIGNS AND SYMPTOMS
• Patients often present with
• Dyspnea
• Cough
• Wheezing
• Hemoptysis
• Stridor
• Hemoptysis, while uncommon, more often occurs with a squamous cell
carcinoma and can potentially lead to earlier diagnosis, whereas wheezing or
stridor occurs more often with the adenoid cystic variant. Dysphagia and
hoarseness can also be present initially and usually indicate advanced disease.
• Diagnosis of Tumors
• Bronchoscopic biopsy
• Symptoms of airway narrowing (eg, stridor, dyspnea, wheezing) can herald life-threatening airway
obstruction. An airway tumor should be considered a possible cause if such symptoms are unexplained,
are of gradual onset, are associated with other symptoms of airway tumors (eg, unexplained
hemoptysis), and respond poorly to standard treatments (eg, if aggressive asthma treatments do not
relieve wheezing, or antibiotics for apparent pneumonia do not improve symptoms and x-ray findings).
• If an airway tumor is suspected, patients require immediate evaluation with bronchoscopy.
Bronchoscopy can both treat airway obstruction and allow specimens to be obtained for diagnosis. If
cancer is found, more extensive testing for staging is done.
• Prognosis for Tumors
• Prognosis depends on the histology.
• Squamous cell carcinomas tend to metastasize to regional lymph nodes and directly invade mediastinal
structures, leading to high local and regional recurrence rates. Even with definitive surgical resection,
the 5-year survival is only 20 to 40%.
• Adenoid cystic carcinomas are typically indolent but tend to metastasize to the lungs and to spread
perineurally, leading to high recurrence rates after resection. However, these patients have a higher 5-
year survival of 60 to 75% because of the slow tumor growth rate.
TREATMENT
• Surgery
• Sometimes radiation therapy
• Obstruction reduction techniques
• Primary airway tumors should be treated definitively with surgical resection if possible. Tracheal,
laryngotracheal, or carinal resections are the most common procedures. Up to 50% of the length
of the trachea can be safely resected with primary reanastomosis. If a lung or thyroid cancer
invades the airway, surgery is sometimes still feasible if assessment indicates sufficient tissue is
available for airway reconstruction. Adjuvant radiation therapy is recommended if adequate
surgical margins cannot be obtained.
• Most primary airway tumors are not resectable because of metastasis, locally advanced stage, or
patient comorbidities. In cases of endoluminal tumors, therapeutic bronchoscopy can
mechanically core-out the tumor. Other techniques to eliminate obstruction include laser
vaporization, photodynamic therapy, cryotherapy, and endobronchial brachytherapy. Tumors
that compress the trachea are treated with airway stenting, radiation therapy, or both.
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