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

Adjuvant Treatment in Extensive Unilateral Retinoblastoma Primary Enucleated

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

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:

1. Written informed consent - a signed informed consent and/or assent (as age appropriate) will be obtained according to institutional guidelines;

2. Male or female ≥2 months and <10 years of age at the time of signing the informed consent form;

3. Diagnosis of non familial extensive unilateral retinoblastoma treated by primary enucleation

4. 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).

5. Patients affiliated to a Social Security Regimen or beneficiary of the same

6. No chemotherapy or radiotherapy prior to administration of the first dose of study treatment for retinoblastoma or other tumor types

7. 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