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Clear cell sarcoma of kidney (CCSK) is an aggressive renal neoplasm. We report two boys aged three and half, and three years with CCSK, one of whom had a disease free survival of four years and eight months. These patients were managed with surgery, chemotherapy and radiotherapy. One of the patients discontinued treatment early and lost to follow up. Aggressive multimodality therapy is the keystone to improved outcome.

Clear Cell Sarcoma of the Kidney: Report of Two Cases