Wilms' tumour: connecting tumorigenesis and organ development in the kidney

Nat Rev Cancer. 2005 Sep;5(9):699-712. doi: 10.1038/nrc1696.


Wilms' tumour, or nephroblastoma, is a common childhood tumour that is intimately linked to early kidney development and is often associated with persistent embryonic renal tissue and other kidney abnormalities. WT1, the first gene found to be inactivated in Wilms' tumour, encodes a transcription factor that functions as both a tumour suppressor and a critical regulator of renal organogenesis. Our understanding of the roles of WT1 in tumour formation and organogenesis have advanced in parallel, providing a striking example of the intersection between tumour biology, cellular differentiation and normal organogenesis.

Publication types

  • Research Support, N.I.H., Extramural
  • Research Support, U.S. Gov't, P.H.S.
  • Review

MeSH terms

  • Animals
  • Child
  • Child, Preschool
  • Genes, Wilms Tumor*
  • Humans
  • Kidney / embryology*
  • Kidney Neoplasms / genetics*
  • Wilms Tumor / genetics*