PRIMARY RENAL ANGIOSARCOMA - A CASE-REPORT AND REVIEW OF THE LITERATURE

Primary renal angiosarcoma is very rare, To our knowledge, only 15 cas es have been reported to date, A 77-year-old Japanese man with a unila teral kidney presented with massive hematuria followed by renal failur e, A renal tumor was suspected and a left nephrectomy was performed, T he histopathological diagnosis was angiosarcoma of the kidney, A hemor rhagic tumor measuring 10 x 5 cm and clotted blood was found in the me dullary area. The atypical tumor cells had a sinusoidal and solid appe arance, and showed immunohistochemically positive reactions for some o f the endothelial markers, The patient died about 21 months after the nephrectomy and the autopsy revealed massive metastases to the liver a nd retroperitoneum. One of the differential diagnoses of the case was angiomyolipoma, because the tumor cells were relatively bland in their histological appearance with entrapped fat cells in the pelvic area, Fifteen case reports with titles that included the term 'hemangiosarco ma/angiosarcoma', 'hemangioendothelioma/endothelioma' or 'vascular sar coma' of the kidney were reviewed and compared to the present case.