Wilms' tumour

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 (most common presenting feature)
• painless haematuria
• flank pain
• other features: anorexia, fever
• unilateral in 95% of cases
• metastases are found in 20% of patients (most commonly lung)

Referral

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

Management

• nephrectomy
• chemotherapy
• radiotherapy if advanced disease