Aim-The aim of this study was to determine the (time trends in) incidence and survival of hereditary (familial and sporadic) and non-hereditary retinoblastoma for male and female patients born in the Netherlands between 1862 and 1995. Method-The national retinoblastoma register was updated and now consists of 955 patients. The missing dates of death were obtained from the municipal registers and the Central Bureau of Genealogy in The Hague. Mortality was compared with the Dutch vital statistics. Results-From 1862 to 1995 no significant diVerences in incidence for retinoblastoma were found in the hereditary subgroups. Further, no significant differences between males and females were found, both overall and in the hereditary subgroups. The average incidence of retinoblastoma increased untill 1944, probably due to incompleteness of the register, and stabilised after 1945 (1 per 17 000 live births). From 1900 to 1995 the standardised mortality ratio increased for hereditary retinoblastoma patients from 2.9 to 9.0 and decreased for non-hereditary retinoblastoma patients from 1.9 to 1.0. Conclusion-Although survival for retinoblastoma was significantly better after 1945 than before, in comparison with the Dutch population the mortality between 1900 and 1990 increased for the hereditary and decreased for the non-hereditary retinoblastoma patients. (Br J Ophthalmol 1997;81:559-562)

Purpose: The aim of this survey was to review the different studies regarding the occurrence of second primary tumors (s PT) among survivors of retinoblastoma. Methods: Ovid (Medline, Current contents life, Psychlit, Embase) was searched for the years 1966 -1995 using the mesh headings: 'retinoblastoma', 'second primary neoplasms', and 'multiple primary neoplasms'. The inclusion criteria were: the study should involve 50 patients or more and should not be limited to one specific SPT. A checklist with criteria regarding the study design and the results was applied to each study. Results: Eleven studies were identified which met the inclusion criteria. Thirtyfive different types of S P T (Ntotal=z43) were reported. Most of them were osteosarcomas (37.0%), followed by melanomas (7.4%), soft-tissue sarcomas (6.9%), brain tumors (4.5%), fibrosarcomas (3.3%), chondrosarcomas (3.3%), and sarcomas (3.3%). Less frequently reported were leukemias (2.4%), sebaceous cell carcinomas (1.6%), and non-Hodgkin lymphomas (1.6). Pineoblastoma, which in fact is a trilateral retinoblastoma and not an S P T , was found in 2.4%. Despite the differences, all 11 studies showed a higher incidence of S P T compared to the general population. Only 4 studies were judged to be free from selection bias, reporting a cumulative incidence of S P T of 8.4% 18 years after diagnosis, 15.7% at the age of 20 years, 19% at the age of 35 years, and a relative risk of 15.4 for s P T, respectively. Conclusion: s P T is a serious problem for the survivors of hereditary retinoblastoma and its importance shoud be recognized in (genetic) counseling of patients.

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