Bilateral Wilms' tumour in a developing country: a descriptive study.

PURPOSE

To present our experience of 20 children with bilateral Wilms' tumour seen in a resource-challenged environment over a 10-year period.

METHOD

All patients with a diagnosis of bilateral synchronous Wilms' tumour were identified and recruited.

RESULTS

Study patients represented 11 % of a cohort of 177 new patients with Wilms' tumour seen over the same period. Three patients had a syndromic predisposition to Wilms' tumour. Metastatic disease was seen at presentation in four patients (20 %) and three children presented with unilateral tumour rupture. One patient presented with paraplegia and one with obstruction of the duodenum. All children received neoadjuvant chemotherapy. One HIV-infected child died of IRIS after neoadjuvant treatment, but before surgery. One child died of progressive disease after unilateral nephrectomy. Nephron-sparing surgery was performed in 22 kidneys and 15 kidneys were removed in toto. Following enucleation of tumours, three children had positive margins. Discordant histopathology was seen in 53 % of patients. Overall survival at 2 years is 85 %.

CONCLUSION

Despite significant co-morbidity and advanced disease, bilateral Wilms' tumour is a treatable disease in a resource-constrained environment.