EXTRAOCULAR RETINOBLASTOMA: SUSTAINED REMISSION IN 10 OF 12 CHILDREN TREATED WITH IRRADIATION AND COMBINATION CHEMOTHERAPY

Abstract

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

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