title: Congenital abnormalities and clinical features associated with Wilms' tumour: a comprehensive study from a centre serving a large population.
abstract: Altogether 156 children treated for Wilms' tumour (WT) between 1970 and 1998 were studied. Sixty-six children, selected only by their attendance at clinic, were carefully examined and the findings compared to those from a case note review of 90 children. Congenital abnormalities were present in 45% of the examined cohort, in 19% of the case notes review group and in 30% overall. Novel findings included the association of WT with Marshall Smith syndrome, developmental delay in 3 of 4 cases of WT (one bilateral) and 1 sibling from consanguineous Pakistani families and another sibling also had leukaemia. The possibility of rare DNA repair or cancer predisposing disorders among these 4 families requires further study. Careful examination and history taking of an unselected patient cohort revealed a higher than expected incidence of clinical abnormalities which may be overlooked if not specifically sought.
authors.affiliation: Department of Paediatric Oncology, Birmingham Children's Hospital, B4 6NH, UK.
authors.forename: A
authors.initials: A
authors.lastname: Ng
authors.forename: A
authors.initials: A
authors.lastname: Griffiths
authors.forename: T
authors.initials: T
authors.lastname: Cole
authors.forename: V
authors.initials: V
authors.lastname: Davison
authors.forename: M
authors.initials: M
authors.lastname: Griffiths
authors.forename: S
authors.initials: S
authors.lastname: Larkin
authors.forename: S E
authors.initials: SE
authors.lastname: Parkes
authors.forename: J R
authors.initials: JR
authors.lastname: Mann
authors.forename: R G
authors.initials: RG
authors.lastname: Grundy
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D000013:Congenital Abnormalities / Q000235:genetics*; D005260:Female; D005500:Follow-Up Studies; D005821:Genetic Techniques; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000150:complications* / Q000235:genetics; D008297:Male; D010375:Pedigree; D009396:Wilms Tumor / Q000150:complications* / Q000235:genetics