title: Second and subsequent tumours among 1927 retinoblastoma patients diagnosed in Britain 1951-2004.
abstract: BACKGROUND
  Retinoblastoma is an eye tumour of childhood that occurs in heritable and non-heritable forms. In the heritable form, there is a predisposition to the development of non-ocular subsequent primary tumours (SPTs).
  
  
  METHODS
  This study included 1927 retinoblastoma patients diagnosed in Britain from 1951 to 2004. Ascertainment was through the (UK) National Registry of Childhood Tumours; cases were followed-up for the occurrence of SPTs. Standardised incidence ratios (SIRs) were calculated.
  
  
  RESULTS
  We identified 169 SPTs in 152 patients. The SIR analysis included 145 SPTs with cancer registrations from the years 1971 to 2009. These tumours occurred in 132 patients: 112 of the 781 heritable and 20 of the 1075 (presumed) non-heritable cases under surveillance at the start of this period developed at least one registered SPT. The SIRs for all tumours combined were 13.7 (95% confidence interval 11.3-16.5) in heritable cases and 1.5 (0.9-2.3) in non-heritable cases. The main types of SPT in the heritable cases were leiomyosarcoma, (31 cases; SIR 1018.7 (692.2-1446.0)), osteosarcoma (26 cases; SIR 444.6 (290.4-651.4)), and skin melanoma (12 cases; SIR 18.6 (9.6-32.4)).
  
  
  CONCLUSIONS
  The risk of SPTs in heritable retinoblastoma is extremely high. This has important implications for the clinical follow-up and counselling of survivors and their families.
authors.affiliation: Childhood Cancer Research Group, New Richards Building, University of Oxford, Old Road Campus, Headington, Oxford OX3 7LG, 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: P A
authors.initials: PA
authors.lastname: Brownbill
authors.forename: K J
authors.initials: KJ
authors.lastname: Bunch
authors.forename: N L
authors.initials: NL
authors.lastname: Diggens
authors.forename: G J
authors.initials: GJ
authors.lastname: Draper
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: C A
authors.initials: CA
authors.lastname: Stiller
authors.forename: T J
authors.initials: TJ
authors.lastname: Vincent
authors.forename: M F G
authors.initials: MF
authors.lastname: Murphy
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D020022:Genetic Predisposition to Disease / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D007223:Infant; D007231:Infant, Newborn; D008297:Male; D008875:Middle Aged; D016609:Neoplasms, Second Primary / Q000453:epidemiology* / Q000235:genetics; D012042:Registries; D019572:Retinal Neoplasms / Q000453:epidemiology* / Q000235:genetics; D012175:Retinoblastoma / Q000453:epidemiology* / Q000235:genetics; D017741:Survivors / Q000706:statistics & numerical data; D013997:Time Factors; D006113:United Kingdom / Q000453:epidemiology; D055815:Young Adult