A Case of Clear Cell Sarcoma of the Kidney / 대한신장학회잡지

ABSTRACT

Clear cell sarcoma of the kidney(CCSK) is one of the rare malignant renal tumors in childhood and is different from Wilms tumor by its pathologic features, clinical manifestations and propensity to metastasize to the skeletal system and brain. We report a case of CCSK on the right kidney in a 14-month-old girl presented with abdominal distention and palpable huge abdominal mass on physical examination, followed by metastasis to the rib on the left upper thorax. After right nephrectomy, chemotherapy with National Wilms Tumor Study DD regimen(vincristine+dactinomycin+adriamycin) was continued without any evidence of relapse until the last follow-up, 1 year after beginning chemotherapy. A brief review of related literatures was made.