[Primary angiosarcoma of the spleen. Apropos of a new case]

The authors report a new case of primary angiosarcoma of the spleen and, after a review of the literature, they discuss its clinical, diagnostic and therapeutic problems. Primary angiosarcoma of the spleen is a very rare tumor. The diagnosis should be suspected in the case of a patient with splenomegaly and unexplained anemia, with no evidence of lymphoma, leukemia or myelofibrosis. In 30% of cases, the tumor presents in the form of spontaneous rupture of the spleen. The prognosis is very poor, as it is a highly malignant tumor, even more so in the presence of early metastases with or without spontaneous rupture of the organ. Splenectomy prior to rupture could increase the survival. Patients with or without metastatic disease may be treated by combination chemotherapy, which still remains empirical and palliative.