Wilms' tumor: An update

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.