Wilms' nephroblastoma is one of the most common childhood malignancies. It typically presents in children under 5 years of age, with a median age of 3 years old.

Associations

• Beckwith-Wiedemann syndrome
• as part of WAGR syndrome with Aniridia, Genitourinary malformations, mental Retardation
• hemihypertrophy
• around one-third of cases are associated with a loss-of-function mutation in the WT1 gene on chromosome 11

Features

• Abdominal mass that doesn't cross the midline (but may be bilateral in up to 5% of cases)
• Abdominal distension
• Haematuria
• Hypertension
• Normally otherwise asymptomatic unless tumour has grown so large that it is causing pain or infiltrating/disrupting other abdominal structures

Referral

• children with an unexplained enlarged abdominal mass in children - possible Wilm's tumour - arrange paediatric review with 48 hours

Investigations

• In patients with suspected nephroblastoma CT chest, abdomen and pelvis is indicated in order to identify the extent and spread of disease