Wilms Tumor.

Wilms tumor (WT) is the most common pediatric renal malignancy, characterized by triphasic histology and strong links to disrupted kidney development. Advances in molecular profiling and international collaboration have refined risk stratification using histology, stage, and biomarkers. Treatment combines surgery, chemotherapy, and radiation, with protocol differences between Children's Oncology Group (upfront nephrectomy) and International Society of Pediatric Oncology (preoperative chemotherapy). Nephron-sparing surgery is prioritized for bilateral disease to preserve renal function. Despite excellent survival for favorable histology WT, diffuse anaplasia and adverse molecular features require intensified therapy. Emerging trials aim to reduce toxicity while maintaining cure rates.