Wilms' tumor: An update

Abstract

Wilms' tumor (WT) is the commonest pediatric renal tumor, predominantly seen in children less than five years of age. The majority of patients present with an abdominal lump and CT scan is the usual imaging modality for determining the extent of disease. With multimodality management, the results of treatment of WT have improved dramatically over the last 50 years. The treatment protocols have been devised and modified repeatedly depending on evidence from randomized trials by several cooperative groups - mainly National Wilms' Tumor Study Group (NWTSG) and the International Society of Pediatric Oncology (SIOP). The NWTSG recommends primary surgery followed by chemotherapy while SIOP advocates four weeks of chemotherapy prior to surgery. The regimen, dose and duration of chemotherapy have been repeatedly modified to reduce toxicity while maintaining efficacy. The role of radiation therapy has also been customized. Most centers have reported excellent survival rates with the modern day treatment protocols, except in patients with an unfavorable histology. The results of treatment of relapsed WT have also improved with newer drugs and combinations being used for the same.

Keywords: Chemotherapy; International Society of Pediatric Oncology; National Wilms' Tumor Study Group; Wilms' tumor; surgery; survival; toxicity.