Multimodality therapy for advanced clear cell sarcoma of the kidney in a young adult

We report a clear cell sarcoma of the kidney (CCSK) in a young adult with multiple liver and bone metastases who was treated with surgery, chemotherapy and radiation. Combination chemotherapy consisting of vinblastine, actinomycin D, cyclophosphamide, pepleomycin, cisplatin (modified VAB-6 regimen) and arterial infusion of mitomycin C and doxorubicin into the hepatic artery resulted in the complete disappearance of the liver metastasis. Although systemic and localized combination chemotherapies were very effective against the liver metastasis, the clinical course was that of high-grade malignancy, resulting in death with brain metastasis 32 months after tumor resection. Differing from typical Wilms tumor in children, the treatment of CCSK has been a subject of controversy, particularly in the advanced and adult type. Nevertheless, we consider that the multimodal treatment has an important role in the stabilization of metastatic lesions even at an advanced stage in the adult type of CCSK.