Congenital abnormalities and clinical features associated with Wilms' tumour: a comprehensive study from a centre serving a large population

Eur J Cancer. 2007 Jun;43(9):1422-9. doi: 10.1016/j.ejca.2007.03.020. Epub 2007 May 17.


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.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Cohort Studies
  • Congenital Abnormalities / genetics*
  • Female
  • Follow-Up Studies
  • Genetic Techniques
  • Humans
  • Infant
  • Kidney Neoplasms / complications*
  • Kidney Neoplasms / genetics
  • Male
  • Pedigree
  • Wilms Tumor / complications*
  • Wilms Tumor / genetics