Angiosarcomas of primary gynecologic origin : a clinicopathologic review and quantitative analysis of survival

Objective: Angiosarcomas are aggressive, malignant soft tissue neoplasms of endothelial origin and occur rarely in the female genital tract. There is lack of consensus on risk factors for poor outcome and optimal treatment. To this end, we performed a clinicopathologic review and survival analysis. Methods: We report a case of a woman with an angiosarcoma of the vagina. Published English literature was reviewed for angiosarcomas of the vulva, vagina, uterus, and ovary. Survival was evaluated by using Kaplan-Meier analysis and the effect of clinical and demographic variables on survival by using Cox regression analysis. Results: A total of 51 patients were identified with a median age of 47 years (range, 17-87 years). Two of the patients had an angiosarcoma of the vulva; 2 had an angiosarcoma of the vagina; 18 had an angiosarcoma of the uterus, and 29 had an angiosarcoma of the ovary. Five-year overall survival was 27% (SE, 8%). Most patients presented with locoregional disease, having surgery as their primary intervention. Overall, adjuvant therapy significantly improved survival (hazards ratio, 0.17; 95% confidence interval, 0.05-0.59; adjusted for age and tumor size). Adjuvant treatment consisted of radiotherapy for angiosarcomas of the vulva, vagina, and uterus and chemotherapy for ovarian angiosarcomas. Subgroup analysis of the female genital tract site was hampered by the small number of cases. Conclusions: This review supports the use of surgical and adjuvant radiotherapy for angiosarcomas of the vulva, vagina, and uterus. Cytoreductive surgery and adjuvant chemotherapy remain the primary treatment of angiosarcomas of the ovary