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Treatment & Prognosis: Rhabdoid tumor of kidney is generally treated with a combination of surgery, radiation, and chemotherapy. The overall prognosis is poor. Widespread metastases to lymph node, liver, and lungs are common at the time of diagnosis. Less than 20% of patients present at stage 1. The 5-yr survival rates are 15%-25%. Several investigational drugs targeting SMARCB1-related epigenetic dysregulation are undergoing clinical trials.