Retinoblastoma: survival, age at detection and comparison 1914-1958, 1958-1983

Abstract

A retrospective analysis of the records of 1531 retinoblastoma patients (809 male, 720 female, sex of two not known) was carried out to ascertain life table survivals. Of patients seen between 1914 and 1983 survival from metastatic retinoblastoma was the same for unilateral and bilateral patients. Metastatic retinoblastoma occurred within four years from diagnosis of the unilaterally affected patients and within nine years from diagnosis of the bilaterally affected patients. Survival thereafter remained at 76%. Because of the development of second non-ocular tumors in survivors of bilateral retinoblastoma there was a dramatic difference between survival of the patient with unilateral and bilateral disease. Within five years after diagnosis of bilateral retinoblastoma more children die from their second tumor than retinoblastoma itself. By 25 years after diagnosis more than 50% of the bilaterally affected children are dead and by 35 years 59% are dead. There was no difference between survival of boys and girls. Although bilateral patients were seen at significantly lower ages at diagnosis, they did not have a lower mortality rate from metastatic retinoblastoma. Despite the fact that bilaterally affected children with a family history were diagnosed at a younger age than those without a family history (11 vs. 15 mos.) mortality from metastatic retinoblastoma was the same. Similarly, even though unilateral patients with a family history were diagnosed younger than those without a family history (5 vs. 25 mos.) mortality from metastatic retinoblastoma was the same. There has been little change in the age at detection for retinoblastoma in the pre- versus post-1958 series and no change in survival rate for bilateral or unilateral patients.(ABSTRACT TRUNCATED AT 250 WORDS)