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

Prior treatment

• 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

Other illness

• Uncontrolled medical conditions, psychological, familial, sociological, or geographical conditions that do not permit compliance with the protocol

Other

• Bilateral and/or familial or trilateral retinoblastoma.
• Unilateral retinoblastoma with possibility of conservative treatment:
• Metastatic extension at diagnosis
• One inclusion criteria non observed

• 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.