Response of Orbital and Central Nervous System Metastases of Retinoblastoma Following Treatment with Cyclophosphamide/Doxorubicin

A 3.5-year-old boy with orbital and central nervous system extension of unilateral retinoblastoma received chemotherapy consisting of intravenous cyclophosphamide and doxorubicin and intrathecal methotrexate. Complete shrinkage of orbital tumor, phthisis bulbi,'and disappearance of intracranial metastases occurred following chemotherapy. Response of the intra-cranial tumors reflected the combined effects of cyclophosphamide and doxorubicin; the contribution of each agent could not be assessed. Cerebrospinal fluid tumor cells persisted prior to delivery of craniospinal irradiation, and were detected again 6 weeks after completion of irradiation.