Retinoblastoma in Great Britain 1963-2002

Br J Ophthalmol. 2009 Jan;93(1):33-7. doi: 10.1136/bjo.2008.139618. Epub 2008 Oct 6.

Abstract

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).

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Age Distribution
  • Child
  • Child, Preschool
  • Female
  • Genetic Counseling
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Registries / statistics & numerical data
  • Retinal Neoplasms / epidemiology*
  • Retinal Neoplasms / pathology
  • Retinoblastoma / epidemiology*
  • Retinoblastoma / pathology
  • Sex Distribution
  • Time Factors
  • United Kingdom / epidemiology