Chemotherapy With or Without Radiation Therapy or Observation in Treating Young Patients With Advanced Retinoblastoma Who Have Undergone Surgery to Remove the Eye

Retinoblastoma

intraocular retinoblastoma, extraocular retinoblastoma

RATIONALE: Drugs used in chemotherapy, such as carboplatin, vincristine, etoposide, and cytarabine, work in different ways to stop the growth of tumor cells, either by killing the cells or by stopping them from dividing. Radiation therapy uses high-energy x-rays to kill tumor cells. Giving chemotherapy with or without radiation therapy after surgery may kill any tumor cells that remain after surgery. Sometimes, after surgery, the tumor does not need more treatment until it progresses. In this case, observation may be sufficient. PURPOSE: This clinical trial is studying how well chemotherapy with or without radiation therapy or observation works in treating young patients with advanced retinoblastoma who have undergone surgery to remove the eye.

OBJECTIVES: * Compare outcome data in children with advanced unilateral retinoblastoma with historical controls in order to determine whether post-enucleation chemotherapy with or without radiotherapy improves outcome. * Determine the disease-free and overall survival of children with unilateral retinoblastoma with no adverse histological features who undergo observation after enucleation. * Determine the toxicity of these regimens in these patients. OUTLINE: This is a nonrandomized study. Patients are assigned to 1 of 3 treatment groups according to histological features. * Group 1 (no adverse histological features): Patients are observed and monitored for the development of orbital recurrence and metastatic disease. * Group 2a (deep choroidal invasion and/or retrolaminar invasion of the optic nerve and disease in the anterior chamber): Patients receive carboplatin IV over 1 hour, vincristine IV, and etoposide IV over 4 hours on day 1, and, if necessary, intrathecal cytarabine on day 2. Treatment repeats every 21 days for 4 courses. * Group 2b (invasion of the cut end of the optic nerve): Patients receive carboplatin IV over 1 hour, vincristine IV, and etoposide IV over 4 hours on day 1, and, if necessary, intrathecal cytarabine on day 2. Treatment repeats every 21 days for 6 courses. Patients also undergo orbital radiotherapy 5 days a week for 4 weeks. After completion of study treatment, patients are followed periodically for up to 10 years. PROJECTED ACCRUAL: Not specified

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United Kingdom