Primary Uterine Angiosarcoma

OBJECTIVE: The aim of this study was to report the first case of primary uterine angiosarcoma described in a Hispanic American woman and to review the literature on uterine angiosarcomas. We review characteristic presenting symptoms, gross and microscopic pathologic findings, and treatment outcomes where available. METHODS: A case report is presented with a review of the English language literature via a Medline search. The key phrases used in the search were uterine angiosarcoma, hemangiosarcoma, hemangioendothelioma, and primary uterine neoplasm. RESULTS: Since the first report in 1902, there have been 19 reported cases of primary uterine angiosarcoma considered valid. Many early cases are questioned due to the lack of ultrastructural and immunohistochemical evidence available in later cases. Seventy-four percent (14 of 19) of these patients are perimenopausal with a mean age of 55 years (range 17-76 years). The common presenting findings are a pelvic mass, menorrhagia, and weight loss. Varying regimens of surgery, chemotherapy, and radiation have been utilized with limited success. CONCLUSIONS: Primary uterine angiosarcomas tend to exhibit a highly malignant behavior. The predominant prognostic factor seems to be the size of the tumor at diagnosis and the presence of extrapelvic disease. Recurrence occurs on average at 8.2 months. Of evaluable patients (n = 14), at 12 months the survival was only 43%. Although radiation and chemotherapy are options being utilized, no consensus exists for optimal therapy given the few cases from which to draw conclusions. Regardless of treatment, outcome is usually poor.