title: Non-ocular tumours following retinoblastoma in Great Britain 1951 to 2004.
abstract: BACKGROUND
  Retinoblastoma occurs in both a heritable and a non-heritable form. In the heritable form, there is a predisposition to the development of non-ocular tumours.
  
  
  OBJECTIVE
  To identify the types of non-ocular tumour occurring in retinoblastoma survivors and to produce estimates of risk for these tumours.
  
  
  METHODS
  We carried out a cohort study that included 1927 cases of retinoblastoma diagnosed in Great Britain between 1951 and 2004. Cases were ascertained through the National Registry of Childhood Tumours and followed up for the occurrence of non-ocular tumours using the routine notification system based on the National Health Service Central Registers in Britain.
  
  
  RESULTS
  Of the 1927 cases, 809 were known to have the heritable form of the disease and 1118 assumed to have the non-heritable form. 102 of the heritable and 13 of those classified as non-heritable developed a non-ocular tumour. The cumulative risk of developing such a tumour 50 years after retinoblastoma diagnosis was 48.3% (95% confidence interval: 38.1 to 59.7%) in the heritable and 4.9% (1.9 to 12.4%) in the non-heritable cases. The main categories of non-ocular tumours observed in the heritable cases were soft-tissue sarcomas (36 of which 21 were leiomyosarcoma), osteosarcoma (32), carcinoma (13), brain and central nervous system tumours (10), melanoma (9), leukaemia (4) and others (4). There were a total of 108 non-ocular tumours in 102 cases.
  
  
  CONCLUSIONS
  There is a high risk of non-ocular tumours occurring in survivors of heritable retinoblastoma. These results have important implications for the clinical follow-up and counselling of survivors.
authors.affiliation: Childhood Cancer Research Group, University of Oxford, 57 Woodstock Road, Oxford OX2 6HJ, UK. angela.maccarthy@ccrg.ox.ac.uk
authors.forename: A
authors.initials: A
authors.lastname: MacCarthy
authors.forename: A M
authors.initials: AM
authors.lastname: Bayne
authors.forename: G J
authors.initials: GJ
authors.lastname: Draper
authors.forename: E M
authors.initials: EM
authors.lastname: Eatock
authors.forename: M E
authors.initials: ME
authors.lastname: Kroll
authors.forename: C A
authors.initials: CA
authors.lastname: Stiller
authors.forename: T J
authors.initials: TJ
authors.lastname: Vincent
authors.forename: M M
authors.initials: MM
authors.lastname: Hawkins
authors.forename: H C
authors.initials: HC
authors.lastname: Jenkinson
authors.forename: J E
authors.initials: JE
authors.lastname: Kingston
authors.forename: R
authors.initials: R
authors.lastname: Neale
authors.forename: M F G
authors.initials: MF
authors.lastname: Murphy
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000453:epidemiology*; D019572:Retinal Neoplasms / Q000453:epidemiology*; D012175:Retinoblastoma / Q000453:epidemiology*; D012307:Risk Factors; D040242:Risk Reduction Behavior; D017741:Survivors / Q000706:statistics & numerical data; D013997:Time Factors; D006113:United Kingdom / Q000453:epidemiology; D055815:Young Adult