Chemotherapy for extraocular retinoblastoma.

Because few patients with extraocular retinoblastoma are seen in the United States and Europe, it has been difficult to establish the optimal role of chemotherapy in such cases. We examined the efficacy of two chemotherapy combinations (cisplatin/etoposide and cyclophosphamide/doxorubicin) in patients with extraretinal retinoblastoma, administered prior to radiotherapy in four patients with recurrent or advanced primary disease. Responses were evaluable in three patients, each of whom was treated by both chemotherapy regimens and radiotherapy. Multimodality therapy promises to be of benefit to patients with extraocular retinoblastoma, and future studies will explore alternative chemotherapy combinations for this purpose.