*GROW, School for Oncology and Developmental Biology, Departments of †Obstetrics and Gynecology, ‡Internal Medicine, §Epidemiology, and ∥Pathology, Maastricht University Medical Center, Maastricht; and ¶Department of Pathology, Atrium Medisch Centrum Parkstad, Heerlen, the Netherlands.
Int J Gynecol Cancer. 2014 Jan;24(1):4-12. doi: 10.1097/IGC.0000000000000020.
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.
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.
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.
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.
血管肉瘤是一种源自内皮细胞的侵袭性恶性软组织肿瘤，在女性生殖道中罕见发生。目前对于影响预后的风险因素和最佳治疗方法尚未达成共识。为此，我们进行了临床病理回顾和生存分析。
我们报告了 1 例阴道血管肉瘤患者。检索了发表的英文文献，包括外阴、阴道、子宫和卵巢的血管肉瘤病例。采用 Kaplan-Meier 分析评估生存情况，采用 Cox 回归分析评估临床和人口统计学变量对生存的影响。
共确定了 51 例患者，中位年龄为 47 岁（范围，17-87 岁）。其中 2 例患者为外阴血管肉瘤，2 例为阴道血管肉瘤，18 例为子宫血管肉瘤，29 例为卵巢血管肉瘤。5 年总生存率为 27%（SE，8%）。大多数患者表现为局部区域疾病，主要治疗方法为手术。总体而言，辅助治疗显著提高了生存率（风险比，0.17；95%置信区间，0.05-0.59；校正年龄和肿瘤大小后）。辅助治疗包括外阴、阴道和子宫血管肉瘤的放疗和卵巢血管肉瘤的化疗。由于病例数量较少，女性生殖道部位的亚组分析受到限制。
本综述支持对外阴、阴道和子宫血管肉瘤采用手术和辅助放疗，而减瘤手术和辅助化疗仍然是卵巢血管肉瘤的主要治疗方法。
