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
Original language | English |
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Pages (from-to) | 33-37 |
Number of pages | 4 |
Journal | British Journal Of Ophthalmology |
Volume | 93 |
Issue number | 1 |
DOIs | |
Publication status | Published - Jan 2009 |
Keywords
- Adolescent
- Age Distribution
- Child
- Child, Preschool
- Female
- Genetic Counseling
- Great Britain/epidemiology
- Humans
- Infant
- Infant, Newborn
- Male
- Registries/statistics & numerical data
- Retinal Neoplasms/ epidemiology/pathology
- Retinoblastoma/ epidemiology/pathology
- Sex Distribution
- Time Factors