Wilms tumour

Wilms tumour, also called nephroblastoma, is a tumour of the metanephric blastema. It’s the most common renal tumour in childhood. It mostly presents before the age of 5. 5% of cases are bilateral.

WAGR syndrome

Wilms tumour may be part of a syndrome called WAGR syndrome, characterised by Wilms tumor, aniridia, genitourinary anomalies, and retardation (intellectual disability).

Clinical features

It commonly presents as a painless, incidentally discovered abdominal mass, sometimes with haematuria. Unlike in neuroblastoma, this abdominal mass does not cross the midline.

Diagnosis and evaluation

Imaging shows the mass, but for children ultrasound is usually the first choice over CT/MRI. Imaging shows a characteristic renal mass. As part of the preoperative evaluation, it’s important to assess for lung metastases and the function of the contralateral kidney.

Management

Treatment is usually by neoadjuvant chemotherapy followed by surgery (nephrectomy). ‎