A developmental context for multiple genetic alterations in Wilms' tumor

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19 Scopus citations


Wilms' tumor has served as an example of Knudson's two-hit hypothesis of recessive tumor genes, but the genetics has proven to be surprisingly complex. WT1, a tumor suppressor gene on 11p13, is mutated in only a small fraction of Wilms' tumors, and a second chromosomal region, 11p15, harbors a second Wilms' tumor gene also involved in other cancers. In addition, loss of genomic imprinting, or parental origin-specific gene expression of at least two genes, appears to be an early step in Wilms' tumorigenesis and common cancers. Finally, genes on other chromosomes also play a role. I propose a model of Wilms' tumorigenesis in which multiple genetic alterations act within a specific developmental context, accounting for the epidemiological and pathological heterogeneity of Wilms' tumor, as well as the tissue specificity of the tumor types arising from alterations in these genes.

Original languageEnglish (US)
Pages (from-to)7-12
Number of pages6
JournalJournal of cell science
Issue numberSUPPL. 18
StatePublished - 1994


  • 11p15 tumor suppressor gene
  • Genomic imprinting
  • H19
  • IGF2
  • WT1
  • Wilms' tumor

ASJC Scopus subject areas

  • Cell Biology


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