Adjuvant Treatment in Extensive Unilateral Retinoblastoma Primary Enucleated (RB SFCE 2009)

Post operative chemotherapy +/- radiotherapy according to histopathological risk factors of the International Retinoblastoma Staging Working Group.

• Low risk group :

• No optic nerve involvement.
• Intra and prelaminar involvement
• No choroidal involvement.
• Minimal superficial choroidal involvement .
• Intermediate risk group, 2 sub groups :

• Sub group 1 :

• Retrolaminar involvement without Invasion of surgical margin associated or not to massive choroidal involvement
• Anterior segment involvement.
• Intrascleral involvement.
• Sub Group 2 :

• Isolated massive choroidal involvement.
• High risk group :

• Invasion of the surgical margin of the optic nerve
• and/or microscopic extrascleral involvement
• Optic nerve meningeal sheat involvement .

Inclusion criteria

• Written informed consent - a signed informed consent and/or assent (as age appropriate) will be obtained according to institutional guidelines;
• Male or female ≥2 months and <10 years of age at the time of signing the informed consent form;
• Diagnosis of non familial extensive unilateral retinoblastoma treated by primary enucleation
• In case of post operative chemotherapy, patients must have adequate organ function:

• Adequate hematopoietic function Neutrophils>1.0x109/l, Platelets >100 x 109/l.
• Adequate hepatic function: grade II NCI CTC
• Adequate renal function: serum creatinemia <1.5 x ULN for age with normal creatinine clearance estimated by SCHWARTZ formula
• Audiometry < Grade II de Brock.
• Echocardiography normal in case of high dose cyclophosphamide chemotherapy (3 g/m²).
• Patients affiliated to a Social Security Regimen or beneficiary of the same
• No chemotherapy or radiotherapy prior to administration of the first dose of study treatment for retinoblastoma or other tumor types
• Without medical cons-indication to study drugs.

Exclusion criteria

• Bilateral and/or familial or trilateral retinoblastoma.
• Unilateral retinoblastoma with indication of primary chemotherapy before enucleation:

• One or several surgical risk factors
• Buphthalmia Exophthalmia.
• Peri ocular inflammatory signs.
• Extraocular extension :
• Radiological retrolaminar extension (more than 3 mm behind the lamina cribrosa) and or meningeal sheat optic nerve extension.
• Extrascleral extension
• Lymp nodes extension
• Unilateral retinoblastoma with possibility of conservative treatment:
• Metastatic extension at diagnosis
• One inclusion criteria non observed
• Uncontrolled medical conditions, psychological, familial, sociological, or geographical conditions that do not permit compliance with the protocol