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 laterality. 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<0.0001) by about 2.5% per year; for the age group 1–4 years, the average increase was about 0.5% per year (not significant).
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Competing interests: None.
Funding: The Childhood Cancer Research Group receives Core Programme funding from the Department of Health and the Scottish Ministers. The funding agencies had no role in the design, conduct, reporting, or decision to publish the study. The views expressed here are those of the authors and not necessarily those of the Department of Health and the Scottish Ministers. We are grateful to the Childhood Eye Cancer Trust for financial support for Childhood Cancer Research Group retinoblastoma studies.
Ethics approval: Ethics approval was provided by Oxfordshire Research Ethics Committee.