Survival rate and risk factors for patients with retinoblastoma in Japan. The Committee for the National Registry of Retinoblastoma.

Clinical, pathological and follow-up data on the 1,147 cases of retinoblastoma registered in Japan from 1975 to 1982 were statistically analyzed. The cumulative survival rate of the total 1,147 cases was 93.0% at 5 years and 90.3% at 10 years, calculated by the Kaplan-Meier method. The survival rate for the 757 unilateral cases was 93.3% at 5 years and 92.3% at 10 years. For the 390 bilateral cases, however, the rate was 92.2% at 5 years and 86.7% at 10 years. The Cox multivariate analysis indicated that, among the various clinical and pathological findings in the patients, extraocular invasion by the tumor was the most significant risk factor which is predictive of death due to tumor. The survival rate for patients with extraocular invasion was significantly better in cases undergoing subsequent local radiation and/or systemic chemotherapy than in cases without such aftercare. The cumulative incidence rate of second neoplasms 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 neoplasm was the main cause of death in the hereditary cases 10 years after the first onset.