Angiosarcoma of the spleen

Angiosarcoma of the spleen is a very rare but highly malignant vascular neoplasm. So far only 140 cases have been reported. A 42-year-old patient is presented in which the radiologic imaging misled to the diagnosis of infiltrating echinococcosis, After splenectomy histological and immunohistochemical staining gave proof of metastatic angiosarcoma. The patient died three months later as a consequence of multiple organ dysfunction syndrome, The literature is reviewed in regard to clinical features, diagnosis and therapy.