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Condition report · Cancer

Hereditary Wilms Tumor

Inherited predisposition to Wilms tumour, a childhood kidney cancer, including that caused by variants in WT1 or REST.

A condition report looks for rare variants known to cause the conditions it covers.

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The condition we analyse

Hereditary Wilms tumour

Inherited susceptibility to Wilms tumour (nephroblastoma), including WT1- and REST-related forms.

What a diagnosis changes

Regular kidney ultrasound in early childhood allows tumours to be found early; WT1 carriers may also need kidney-function and gonadal checks.

Genes analysed

Also known as

familial Wilms tumor; familial Wilms tumour; hereditary kidney adenosarcoma; hereditary nephroblastoma; hereditary renal adenosarcoma; WT1

Includes 7 related subtypes ↓
  • Wilms tumor 1
  • Wilms tumor 2
  • Wilms tumor 3
  • Wilms tumor 4
  • Wilms tumor 5
  • Wilms tumor 6
  • Wilms tumor 7
MONDO:0003321MedGen 146190

Limitations

  • Deletions at 11p13 (WAGR) and imprinting changes at 11p15 (as in Beckwith-Wiedemann syndrome) are not reliably detected by this test.
  • Some cases are mosaic, so a variant present in only some cells may be missed.

Other cancer conditions

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