Angiosarcoma of the Uterus: Report of 2 New Cases With Deviant Clinicopathologic Features and Review of the Literature

A few cases of uterine angiosarcoma have been detailed in the literature: 2 new cases are herein described featuring some unusual clinical or phenotypic differences compared with previously published cases, such as occurrence in premenopausal age or a poorly differentiated histology. The patients were 35 and 81 years old, and both presented with extrauterine spread and evidence of distant metastases. Grossly, they were fleshy, hemorrhagic, and necrotic. Microscopically, they were made up of poorly differentiated, epithelioid, or spindle cells. In one case, the neoplastic growth deceitfully recalled a poorly differentiated leiomyosarcoma showing focal rudimentary endothelial differentiation. The other index case was characterized by a more pronounced vascular pattern, the neoplasm was composed of spindle cells arranged in loose channels. A diffuse immunopositivity for CD31, CD34, and factor VIII–related antigen was detected. Tumor cells were negative for other tested antigens including keratins, desmin, actins, and H-caldesmon. One patient died shortly during the follow-up, whereas the other is alive with evidence of disseminated disease. Occurrence in childbearing age or a deviant histologic pattern, as documented in this report, may be added to the clinicopathologic spectrum of uterine angiosarcoma.