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Risk stratification for wilms tumor: current approach and future directions
Jeffrey S Dome1, Elizabeth J Perlman1, Norbert Graf1
1From the Division of Oncology, Children's National Medical Center, Washington DC; Northwestern University Feinberg School of Medicine, Ann & Robert H. Lurie Children's Hospital of Chicago, Chicago, IL; Department of Pediatric Oncology and Hematology, Saarland University, Homburg, Germany.
Wilms tumor survival rates now exceed 90% due to advancements in treatment and risk-directed therapy. Improved prognostic markers allow for reduced treatment intensity, leading to healthier survivors of this pediatric cancer.
Area of Science:
- Pediatric Oncology
- Cancer Genomics
- Clinical Trials
Background:
- Wilms tumor (nephroblastoma) treatment has significantly evolved through cooperative group studies.
- Modern multimodal therapy has improved survival rates to approximately 90% for Wilms tumor patients.
- Therapy reduction, guided by prognostic markers, enhances survivor health outcomes.
Purpose of the Study:
- To review the evolution of Wilms tumor treatment strategies.
- To highlight the role of prognostic markers in risk-directed therapy.
- To outline current and future risk stratification approaches in major pediatric oncology protocols.
Main Methods:
- Analysis of outcomes from serial cooperative group studies.
- Identification and application of clinical and biologic prognostic factors.
- Comparison of risk stratification in Children's Oncology Group (COG) and International Society of Pediatric Oncology (SIOP) protocols.
Main Results:
- Overall survival for Wilms tumor has reached 90% with reduced therapy intensity for many subgroups.
- Prognostic factors have advanced from simple staging to complex molecular markers.
- COG and SIOP protocols utilize diverse factors like histology, stage, molecular markers, and treatment response.
Conclusions:
- Wilms tumor management exemplifies successful cooperative group research leading to high survival and improved quality of life.
- Risk-directed therapy, informed by sophisticated prognostic markers, is crucial for optimizing Wilms tumor treatment.
- Ongoing research aims to refine risk classification by incorporating novel molecular and genomic data for even more personalized treatment strategies.
