Primary renal Angiosarcoma is a rare neoplasm and only 24 cases have been reported in specialized literature. We describe a case of primary renal angiosarcoma in a patient presenting with hematuria, palpable abdominal mass, left flank pain and anemia. A computerized tomography of the abdomen with contrast medium showed a tumor with 15 cm diameter, in the upper pole of the left kidney, with a low-density central area, suggesting necrosis or hemorrhage. Diagnosis was given in a morphologic base and proven by an immunohistochemical study. Primary renal angiosarcoma should be included among differential diagnosis of retroperitoneal hematoma and hemorrhagic renal tumors.
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