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Retinoblastoma Treatment (PDQ®): Treatment - Health Professional Information [NCI] - Treatment Option Overview for Retinoblastoma


The issue of balancing long-term tumor control and the consequences of chemotherapy is unresolved. Most patients who receive chemotherapy are exposed to etoposide, which has been associated with secondary leukemia in patients without predisposition to cancer, but at modest rates when compared with the risks associated with EBRT in heritable retinoblastoma. An initial report conducted by informal survey methods described 15 patients who developed acute myeloid leukemia after chemotherapy. Half of the patients also received radiation therapy.[46] This finding has not been substantiated by formal studies. A more recent report of 245 consecutive patients treated with vincristine, carboplatin, and etoposide found a single patient with subsequent acute promyelocytic leukemia. This patient had also undergone EBRT.[47] Additionally, the Surveillance, Epidemiology, and End Result Registry (SEER) calculated standardized incidence rates for secondary hematopoietic malignancies in 34,867 survivors of childhood cancer. The observed-to-expected ratio of secondary acute myeloid leukemia in patients treated for retinoblastoma was zero.[48]

The standard treatment options for intraocular, extraocular, and recurrent retinoblastoma are described in Table 8.

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