卵巢血管肉瘤（OA）是罕见的，英语文献中仅报道了零星病例。我们对PubMed上发表的病例进行了系统回顾，科学直接，和谷歌学者数据库，目的是描述报告的OA的临床病理特征。回顾了53篇报道60例患者的文章。在60名患者中，7例（11.6%）被诊断为继发性（转移性）卵巢血管肉瘤，53例（88.3%）被诊断为原发性卵巢血管肉瘤。卵巢血管肉瘤的平均年龄为38.3±17.8岁。卵巢血管肉瘤的平均肿瘤大小为11.9±6.1cm。在45/60（75％）中报告了腹胀。显微镜检查发现坏死28/60（46.7％），32/59（54.2%），44/60的有丝分裂图（73.3%），27/36（75%）的梭形细胞，上皮样细胞在20/36（55.5％），12/36（33.3％）患者的混合上皮样细胞和纺锤形细胞。在免疫组织化学上，CD31在41/41（100％）中呈阳性，CD34in38/39(97.4%),18/21（85.7％）患者中的因子VIII相关抗原。43/60（71.6％）患者存在转移。36/52（69.2％）进行了化疗和手术。卵巢血管肉瘤的中位随访时间为7个月(IQR1-IQR3:2-13.5个月)。有可用生存数据的50例患者中有24例（48％）存活，26/50（52％）死于疾病。生存分析（KM曲线）显示，组织病理学上存在坏死（对数秩检验；p=0.05）和不存在纺锤形细胞（对数秩检验；p=0.04）与较差的预后相关。而联合化疗和手术切除治疗与更好的生存率相关（P<0.001），因此，及时诊断和早期治疗联合化疗和手术切除可以延长OA的生存期。
Ovarian angiosarcoma (OA) is rare, with only sporadic cases reported in English literature. We performed a systematic review of cases published in the PubMed, Science Direct, and Google Scholar databases with the aim of describing the reported clinicopathological features of OA. Fifty-three articles that reported 60 patients were reviewed. Of the 60 patients, 7 (11.6 %) were diagnosed with secondary (metastatic) ovarian angiosarcoma and 53 (88.3 %) were diagnosed with primary ovarian angiosarcoma. The mean age at presentation for ovarian angiosarcoma was 38.3±17.8 years. The average tumor size for ovarian angiosarcoma was 11.9±6.1 cm. Abdominal distention was reported in 45/60 (75 %). Microscopic examination revealed necrosis in 28/60 (46.7 %), pleomorphism in 32/59 (54.2 %), mitotic figures in 44/60 (73.3 %), spindle-shaped cells in 27/36 (75 %), epithelioid-shaped cells in 20/36 (55.5 %), and mixed epithelioid and spindle-shaped cells in 12/36 (33.3 %) patients. On immunohistochemistry CD 31 was positive in 41/41 (100 %), CD 34 in 38/39 (97.4 %), and Factor VIII related antigen in 18/21 (85.7 %) patients. Metastasis was present in 43/60 (71.6 %) patients. Chemotherapy and surgery was performed in 36/52 (69.2 %). The median follow-up time for ovarian angiosarcoma was 7 months (IQR1-IQR3:2-13.5 months). 24 (48 %) of the 50 patients with available survival data were alive and 26/50 (52 %) were dead of disease. Survival analyses (KM curves) revealed that the presence of necrosis (log-rank test; p = 0.05) and absence of spindle-shaped cells (log rank test; p = 0.04) on histopathology were associated with worse outcomes, while treatment with combined chemotherapy and surgical excision was associated with better survival (P < 0.001) therefore, prompt diagnosis and early treatment with combined chemotherapy and surgical excision can prolong survival in OA.
