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Wilms' tumor: An update
Hemant B Tongaonkar1, Sajid S Qureshi, Purna A Kurkure
1Department of Surgical Oncology, Urologic Oncology Service and Paediatric Oncology Service, Tata Memorial Hospital, Mumbai, India.
Insights
Wilms
Area of Science:
- Pediatric Oncology
- Nephrology
Background:
- Wilms' tumor (WT) is the most common kidney cancer in children, typically affecting those under five.
- Diagnosis often involves identifying an abdominal mass via CT scans.
- Multimodality treatment has significantly improved outcomes over the past 50 years.
Purpose of the Study:
- To review the evolution and current status of Wilms' tumor management.
- To highlight the impact of cooperative group trials (NWTSG, SIOP) on treatment protocols.
- To discuss advancements in chemotherapy, radiation, and management of relapsed cases.
Main Methods:
- Review of treatment protocols and evidence from randomized trials by NWTSG and SIOP.
- Analysis of modifications in chemotherapy regimens, dosage, and duration.
- Evaluation of the role of radiation therapy and treatment strategies for relapsed WT.
Main Results:
- Treatment protocols have been refined, leading to dramatic improvements in survival rates.
- Divergent approaches exist: NWTSG (surgery first) vs. SIOP (chemotherapy first).
- Excellent survival is achievable with modern protocols, though challenges remain with unfavorable histology and relapsed disease.
Conclusions:
- Modern multimodal management, guided by cooperative group research, offers excellent survival for most pediatric Wilms' tumor patients.
- Ongoing refinement of chemotherapy and radiation, alongside novel treatments for recurrence, continues to improve outcomes.
- Wilms' tumor treatment exemplifies successful collaborative research in pediatric oncology.
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.