Uterine angiosarcomas: a morphologic and immunohistochemical study of four cases

The clinical, gross, microscopic, and immunohistochemical features of four examples of exceedingly rare uterine angiosarcomas reviewed at the Armed Forces Institute of Pathology between 1970 and 1997 are presented. One of the cases described has been reported previously. Based on our findings and a review of the literature, uterine angiosarcomas are aggressive lesions that occur predominately in peri- and postmenopausal women with uterine bleeding and anemia. Grossly, the lesions are of large size at initial presentation with deep myometrial extension. Histologically, the lesions demonstrate characteristic features of angiosarcoma and, when associated with a benign smooth muscle proliferation within the uterus, tend to demonstrate an epithelioid morphology. The immunohistochemical reactivity with CD31, factor VIII, and CD34 and lack of reactivity with smooth muscle actin, keratin, and estrogen receptor confirm the vascular nature of these lesions and exclude the differential diagnostic considerations of carcinosarcoma (MMMT), leiomyosarcoma, adenosarcoma, and hemangiopericytoma. The overall survival of these lesions is poor; the majority of women die of disease within 1 year of diagnosis.