Korean Journal of Nephrology 1997;16(3):620-624.

Clear Cell Sarcoma of the Kidney 1예

박선영 , 설소영 , 송민섭 , 정우영 , 이순용

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 aMominal distention and palpable huge aMominal 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.