[Primary angiosarcoma of the spleen: report of one case]

Primary angiosarcoma of the spleen is rare and almost always fatal. The pathogenesis is unknown. It has an aggressive behavior and frequently presents with hematological abnormalities or metastatic disease. We report a 49 year-old male that presented with spleen and lymph node enlargement. He was subjected to a splenectomy and the biopsy disclosed an angiosarcoma of the spleen. Metastases were detected in the lung and bones and the patient was considered beyond any therapeutic option, dying fifteen months later.