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Aim: This paper describes the epidemiology and family history status of 1601 children with retinoblastoma in Great Britain diagnosed 1963-2002 and summarises the practical consequences for diagnosis and counselling of developments in molecular genetics. Methods: Incidence rates were analysed according to year of diagnosis and tumour laterality. Cases were classified as heritable or non-heritable on the basis of laterality and family history of the disease. Results: There were 998 unilateral cases, 581 bilateral and 22 of unknown Iaterality. Bilateral cases tended to be diagnosed at a younger age than unilateral. All bilateral cases are regarded as heritable, and 35% had a family history of the disease. 7% of the unilateral cases had a family history and are therefore heritable. Thus, at least (41%) of our cases are heritable. This is an underestimate, since these data on family history are incomplete. For unilateral cases aged below 1 year, the reported incidence rate increased significantly (p<0.000l) by about 2.5% per year; for the age group 1-4 years, the average increase was about 0.5% per year (not significant). [ABSTRACT FROM AUTHOR]

Aim: This paper describes the treatment and survival of 1576 children with retinoblastoma in Great Britain diagnosed 1963-2002. Methods: Survival rates were analysed according to period of diagnosis and tumour laterality. Results: Survival was calculated by calendar period of diagnosis, 1963-1 982 and 1983-2002. For both unilateral and bilateral retinoblastoma, survival improved between the two periods. The survival curves for the two periods were significantly different: for unilateral retinoblastoma p<0.00001 for bilateral p<0.01. For unilateral cases, the estimated 5-year survival rates rose from 85% for those diagnosed in 1963-1967 to 97% for those diagnosed in 1998-2002. The equivalent rates for bilateral cases were 88% and 100%. Conclusion: Survival rates were already high at the start of the study period. They increased with changes in treatment regimens. [ABSTRACT FROM AUTHOR]