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THINK HADROM: discovering Hadrontherapy within Multidisciplinarity

Focus on Paediatric Radiation Oncology

Retinoblastoma

Alessia Pica
Proton Therapy Center
Paul Scherrer Institut PSI
Villigen (CH)

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Introduction
• Most common malignant ocular tumor in childhood
• Diagnosed before 3 years of age
• Bilateral retinoblastoma: 20%-30%, younger age (14-16 months), family history of the disease
• Developped from immature retinal cells, high mitotic and apoptotic rate, necrosis and calcification

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Introduction

n Time of diagnosis: average age of 18 months


n 9 months for bilateral disease
n 24 months for unilateral disease Courtesy of Dr Uffer

n late diagnosis or late referral ð increased risk of advanced disease, less chance of cure
(developing countries)
n positive family history ð reduced time to diagnosis
n In Switzerland:
n easy access to medical care
n since 1960, most Rbl patients treated in Lausanne (at the national reference center
specialized in the treatment of ocular tumors in children and adults)

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Genetics: « Two hit » model heritable form

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Genetics nonheritable form

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Clinical Presentation

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Retinoblastoma overall survival @ 5 years

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Retinoblastoma: conservative treatment without irradiation

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Radiation therapy in retinoblastoma: the past

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Radiation therapy in retinoblastoma: the past

Int J Rad Oncol Biol Phys, Oct 2010

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
`What`s Up in the new Era?

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Clinical Case 1: 45 Gy RBE

• 5ys old boy


• Bilateral retinoblastoma group D
• Enucleation right eye: the tumor is located in the posterior chamber as well as the anterior chamber. The tumor
consists of atypical cells with large hyperchromatic nuclei and narrow cytoplasm. About 40% of the tumor is
necrotic. The tumor invades the CB, iris, choroid and sclera. Extensive invasion of the optic nerve (N3) with positive
resection margin.

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina
Clinical case 2: 36 Gy RBE
• 3 ys old boy
• Retinoblastoma of the left eye group D
• Status post multiple treatments for retinoblastoma with
currently focus of poorly differentiated central active
retinoblastoma, without anaplasia, without optic nerve
invasion
• Central temporal focus of massive choroidal invasion 4.5
mm in maximum diameter in continuity with the central area
of incomplete mixed regression
• Infiltration of a paracentral temporal scleral canal with
atypical cells at the level of the emergence of scleral
vessels on the episclera (safety margin with orbital margin:
129 μm) opposite the area of massive choroidal invasion
• Infiltration of another scleral canal without externalization
opposite the massive choroidal invasion

Alessia Pica - Think Hadrom: discovering Hadrontherapy within Multidisciplinarity. Focus on Paediatric Radiation Oncology - © Accademia Nazionale di Medicina

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