Wilms Tumor (WT) or Nephroblastoma is the most common renal tumor of childhood. It is an embryonal tumor developing from the remnants of the immature kidney. The classic pathologic finding is a triphasic pattern which includes varying proportions of three cell types: blastemal, stromal, and epithelial. Over the last few decades, work performed by cooperative groups such as the National Wilms Tumor Study (NWTS) and the International Society of Paediatric Oncology (SIOP) has led to dramatic improvement in survival, which approaches 90% for all patients collectively. Currently, treatment for WT in North America is based largely on protocols from the Children’s Oncology Group (COG), Renal Tumors Committee. Patients are stratified into risk groups based on current clinical and molecular staging paradigms and assigned into various treatment protocols that include surgery, chemotherapy and sometimes radiotherapy. Increasing recognition of the late effects of treatment has driven the recent focus on minimizing therapy for those in low-risk groups and developing new agents and strategies for those at highest risk.
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