Wilms' tumour

Keywords: Nephroblastoma

Key Information

Appropriate Tests

Associated syndromes

1% of patients with Wilms' tumour (nephroblastoma) 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 mesangial sclerosis.

 

WAGR

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