Of 170 children with retinoblastoma screened at our institution for metastatic disease since the fall of 1979, 20 (12%) had extraocular tumor. Twelve of these children, including 4 with bone marrow disease, were started on a modification of the former CCSG 962 protocol for extraocular retinoblastoma. This protocol utilized two years of systemic cyclophosphamide, adriamycin, and vincristine; intrathecal methotrexate, cytosine arabinoside, and hydrocortisone succinate; and cranial irradiation. Our modifications include: 1) omission (two cases) of cranial irradiation when bone marrow, but not cranial or cerebrospinal fluid (CSF), disease is present; 2) substitution (one case) of external beam irradiation to the calvarium when involvement of the calvarium, but not the cranium or CSF, is present; 3) scaling doses of intrathecal chemotherapy (all cases) to CSF volume; and 4) giving 2 mg/kg doses of adriamycin over 5 days instead of one. One child died of tumor progression, while another died of adriamycin-related cardiomyopathy. However, the remaining ten children are in bone marrow and CSF remission with a mean time from diagnosis of metastatic disease of 42 months (range 5 to 80 months). In contrast, all eight of the non-study children, treated palliatively or elsewhere, have expired.
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Grabowski, E., Ellsworth, R., McCormick, B. et al. EXTRAOCULAR RETINOBLASTOMA: SUSTAINED REMISSION IN 10 OF 12 CHILDREN TREATED WITH IRRADIATION AND COMBINATION CHEMOTHERAPY. Pediatr Res 21, 299 (1987). https://doi.org/10.1203/00006450-198704010-00792