Wilms' tumour (Nephroblastoma)

Last updated: Saturday, 14, August, 2010
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Associated syndromes

1% of patients with Wilms' tumours have a family history of the same tumour. About 10% of Wilms' tumours occur in association with one of several syndromes. 

A variety of genetic abnormalities can predispose to the development of this tumour. 

Molecular genetics may assist in identifying infants at high risk.

Beckwith-Wiedemann

  • Omphalocoele
  • hemihypertrophy
  • macroglossia
  • visceromegaly

Individuals may develop:

  • Wilms' tumour;
  • hepatoblastoma;
  • rhabdomyosarcoma;
  • neuroblastoma; or
  • pancreatoblastoma.

Perlman

  • Wilms' tumour, gigantism and mental retardation.

Denys-Drash

  • Wilms' tumour, pseudohermaphoditism and renal mesangeal sclerosis.

WAGR

  • Wilms' tumour, aniridia, genitourinary malformations, and mental retardation.