[National registry of retinoblastoma in Japan (1975-1982). The Committee for the National Registry of Retinoblastoma].

Clinical, pathological and follow-up data on 1,147 cases of retinoblastoma registered in Japan from 1975 to 1982 were statistically analysed. The cumulative survival rate of the 1,147 cases, calculated by the Kaplan-Meier method, was 93.0% at 5 years and 90.3% at 10 years. The survival rate of the 757 unilateral cases was 93.3% at 5 years and 92.3% at 10 years. For the 390 bilateral cases, however, the survival rate was 92.2% at 5 years and 86.7% at 10 years. A Cox multivariate analysis indicated that, among various clinical and pathological findings in the patients, extraocular invasion of the tumor is the most significant risk factor predictive of tumor death. The survival rate of those with extraocular invasion subsequently treated by local radiation and/or systemic chemotherapy was significantly better than the rate of those with extraocular invasion who did not receive aftercare. The cumulative incidence rate of second cancers in 409 cases of hereditary retinoblastoma was 4.8% at 10 years, 9.8% at 15 years and 15.7% at 20 years. The occurrence of a second cancer was the main cause of death in the hereditary cases after 10 years.