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

Pediatr Surg Int. 2013 May;29(5):419-23. doi: 10.1007/s00383-013-3287-7. Epub 2013 Mar 2.

Abstract

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.

MeSH terms

  • Chemotherapy, Adjuvant
  • Child
  • Child, Preschool
  • Developing Countries
  • Female
  • Humans
  • Infant
  • Kidney Neoplasms / mortality
  • Kidney Neoplasms / pathology
  • Kidney Neoplasms / surgery*
  • Male
  • Neoplasms, Multiple Primary / mortality
  • Neoplasms, Multiple Primary / pathology
  • Neoplasms, Multiple Primary / surgery*
  • Postoperative Complications / epidemiology
  • Survival Analysis
  • Wilms Tumor / mortality
  • Wilms Tumor / secondary
  • Wilms Tumor / surgery*