• Around 90 children a year are diagnosed with renal tumours

• 90% are Wilms tumours (also known as nephroblastoma)

• The other 10% of renal tumours are formed of more aggressive tumours 

  • malignant rhabdoid tumours

  • clear cell sarcoma of the kidney

  • renal cell carcinoma

 

Wilms tumours are embryonal tumours, and are usually unilateral, but can occur bilaterally.

They usually affect children under the age of 5 with a peak incidence of 1-3 years of age.

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Unilateral tumours are equally common in males and females, but bilateral tumours are more common in girls (1:2 ratio). 

 

Although the causes of Wilms tumour are unknown, up to one in six children also have a congenital malformation syndrome as well including:

• Denys–Drash syndrome

• Beckwith-Weidemann syndrome

• WAGR syndrome (Wilms tumour predisposition, aniridia, genitourinary anomalies, retardation).

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Chemotherapy and surgery, usually nephrectomy, are the mainstay of treatment.

Higher risk tumours receive more aggressive chemotherapy with or without radiation too.

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Five-year survival averages at 90%, depending on staging and histology. However, 5% of Wilms’ tumours have unfavourable ‘anaplastic’ histology giving them much worse outcomes. 

 

Bilateral disease has a worse prognosis than unilateral and children with other types of renal tumour have a poorer prognosis.

 

Malignant rhabdoid tumours, whilst rare, have few survivors.

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Information Resources

Parent information leaflet:

My Child has a kidney tumour - produced by the Children's Cancer and Leukaemia Group and 

                                                  Bethany's Wish

My Child's Wilms' Tumour has come back: treatment for relapsed Wilms' - produced by the

                                              Children's Cancer and Leukaemia Group and Bethany's Wish

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