Ocular Conservative Treatment for Retinoblastoma : Efficacy of the New Management Strategies and Visual Outcome

Inclusion Criteria:

Overall study inclusion criteria:

1. Newly diagnosed retinoblastoma (RB).

2. Retinoblastoma with at least one eye eligible for conservative management.

3. Patients likely to be compliant with the study requirements and visits, includinglate follow-up.

4. Patients not previously treated with chemotherapy or radiotherapy for this or anyother cancer.

5. Patients with no contraindication to the proposed treatments.

6. Informed consent signed by parents or legal representative.

7. French Social Security System coverage.

Study 1 inclusion criteria:

8.1. Children aged from 6 months to 6 years. 9. Retinoblastoma in at least one eye, deemed manageable with IAC in one side and without IV chemotherapy:

1. Unilateral Retinoblastoma classified as group B, C (if vitreous seeds < 3 mm), D orE with no invasion of the anterior segment, and without massive tumor of more than 2/3 of the eye, eligible for conservative management, or

2. Bilateral Retinoblastoma but with very unbalanced lesions between the two eyes: oneeye group D or E without invasion of the anterior segment or massive tumor of morethan 2/3 of the eye, eligible for IAC, and the other eye eligible for localtreatment only (without IAC).

Study 2 inclusion criteria:

8.2. Retinoblastoma eligible for conservative management although not manageable with IAC:

1. Unilateral Retinoblastoma in children below 6 months of age, classified as Group A,B, C or D, with or without vitreous seeding, compatible with conservativemanagement, or

2. Bilateral Retinoblastoma classified as group A, B, C D, or E without invasion of theanterior segment, and/or massive tumors of more than 2/3 of the eye and eligible forconservative management.

Exclusion Criteria:

Overall study non-inclusion criteria:

1. RB not eligible for conservative management :

2. Extra-ocular extension of the disease, or

3. Group E eyes with invasion of the anterior segment, and/or massive tumors ofmore than 2/3 of the eye.

4. Patient older than 6 years of age.

5. Patients with another associated disease contra indicating systemic chemotherapy.

6. Previously treated retinoblastoma by chemotherapy.

7. Patients already treated for another malignant disease.

8. Patient with any psychological, familial, sociological or geographical conditionpotentially hampering compliance with the study protocol and follow-up schedule.

9. Patients whose parents have not accepted the treatment regimen after explanation ofit.

10. Contraindication to study drug mentioned in SmPC (Summary of ProductsCharacteristics) of the study drugs.

11. Inclusion in another experimental anti-cancer drug therapy. Study 1 non-inclusion criteria:

12. Any contraindication or concomitant disease that would preclude the Study 1treatment procedure and could delay treatment.

These patients should be eligible for Study 2.