TREATMENT OF EXTRAOCULAR RETINOBLASTOMA IN BRAZIL

Between January 1986 and December 1990, 120 patients with retinoblasto ma were admitted at A. C. Camargo Hospital. Among these, 32 (26.6%) we re extraocular, (24 unilateral and 8 bilateral). The hereditary type w as present in 9 patients and sporadic in 23. Fifteen patients had opti cal nerve involvement, 5 scleral vessel and 9 orbital. The cerebrospin al fluid was positive in 1, and bone marrow was positive in 2 patients . The mean time before referral was 6 months. Chemotherapy was adminis tered to all cases after enucleation or eyelid biopsy. This comprised cisplatin, etoposide, cyclophosphamide, vincristine and adryamicin, an d intrathecal methotrexate. External irradiation (45 Gy) was initiated after surgery in patients with optical nerve involvement and in patie nts with measurable disease after 3 cycles of chemotherapy. Twenty-six to 32 were evaluated for response. The exclusions criteria were patie nts that refused treatment, or patients who received one cycle of chem otherapy and were lost to follow-up, making the evaluation for respons e impossible to be assess. Among them, 8 are free of disease and off t reatment, with median follow-up of 14.6 months; 1 is alive with locali zed disease, and 3 are lost to follow-up. Fourteen patients died of di sease; the median time of relapse was 6 months (range 1 to 13 months). We conclude that whereas the current approach yields results similar to others, more aggressive combined chemotherapy is necessary for the improvement in the treatment of patients with advanced retinoblastoma. Late referral may be responsible for the high incidence of advanced c ases.