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

• Outcome data compared to historical controls [ Designated as safety issue: No ]
  
• Disease-free and overall survival in children undergoing observation after enucleation [ Designated as safety issue: No ]
  
• Toxicity [ Designated as safety issue: Yes ]
  

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