Angiosarcoma of kidney: a case report and review of literature.

Received March 2008 Accepted September 2008 INTRODUCTION Angiosarcoma is a rare high-grade malignant tumor accounting for less than 2% of the soft tissue sarcomas.(1) They originate from the endothelium of the blood and lymphatic vessels and can be primary or metastatic, and localized or multicentric. Most angiosarcomas develop in the skin or soft tissue, and others, in the breast, liver, and bones.(2) Commonly, metastases occur in the liver, lungs, and bones. Those involving the kidney usually represent metastasis from the skin or the primary visceral lesions. On the contrary, primary angiosarcoma of the kidney is a very rare neoplasm with less than 30 cases reported in the literature to date. (3,4) To our knowledge, all previous reports described the origin of angiosarcoma in a normal kidney. We report a unique case of primary renal angiosarcoma arising from a multicystic kidney.