Overview
Postoperative Treatment of Unilateral Retinoblastoma After Primary Enucleation according to histopathological risk factors of the International Retinoblastoma Staging Working Group.
Description
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 .
Eligibility
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