[Primary renal angiosarcoma. Uncommon manifestation of a rare malignancy]

This article reports a case of primary renal angiosarcoma, a very rare and aggressive malignancy, in a 59-year-old male patient. The mean overall survival time is limited to a few months if the diagnosis is made when clinical symptoms are present but chances of a cure can be increased with surgical resection of smaller incidental findings. Due to the lack of a standard therapy, systemic treatment is based on the therapy of other soft tissue sarcomas. The role of adjuvant medical treatment particularly in angiosarcoma remains poorly studied but using a doxorubicin-based chemotherapy regimen, a survival benefit can be achieved.