Bilateral Wilms’ tumour in a developing country: a descriptive study
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To present our experience of 20 children with bilateral Wilms’ tumour seen in a resource-challenged environment over a 10-year period.
All patients with a diagnosis of bilateral synchronous Wilms’ tumour were identified and recruited.
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 %.
Despite significant co-morbidity and advanced disease, bilateral Wilms’ tumour is a treatable disease in a resource-constrained environment.
KeywordsBilateral Wilms’ tumour Developing country Histopathology
- 2.Davidoff AM, Giel DW, Jones DP, Jenkins JJ, Krasin MJ, Hoffer FA, Williams MA, Dome JS (2008) The feasibility and outcome of nephron-sparing surgery for children with bilateral Wilms’ tumor: the St. Jude Children’s Research Hospital experience: 1999–2006. Cancer 112(9):2060–2070PubMedCrossRefGoogle Scholar
- 3.http://www.childrensrights.org.za/index.php/documents/provincial-profiles/kzn. Accessed 25 Feb 2013