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目的:探讨子宫内膜间质肉瘤(ESS)的临床病理特征、诊断、治疗及预后。方法:对郑州大学第三附属医院病理科2006~2012年收集的32例子宫内膜间质肉瘤患者临床资料、组织学、免疫组化进行观察分析并随访,结合文献讨论。结果:32例患者的中位年龄49.5岁,主要临床表现为不规则阴道出血、子宫增大及子宫占位,全子宫、双侧附件切除术是主要的手术方式。镜下肿瘤细胞与增殖期子宫内膜间质细胞相似,无明显异型性,肿瘤边界可见瘤组织呈蛇状或不规则岛屿状浸润临近子宫平滑肌束,瘤组织内有较多螺旋小动脉。免疫组化结果显示32例ESS瘤细胞均表达CD10,28例瘤细胞表达Vimentin,24例表达ER,21例表达PR,仅4例表达Desmin;ESS中的螺旋小动脉均表达CD34,ESS瘤细胞Ki-67阳性指数较低。26例患者获得随访信息(16~96月),中位生存期61个月。其中1例在67月内死亡。结论:ESS在所有子宫恶性肿瘤较为罕见,诊断要结合临床表现及相关病史,并依据病理学特征及相关的免疫组织化学标志物综合判断而确诊,以外科手术为基础的综合治疗可减少患者的复发及延长生存期。
Objective: To investigate the clinicopathological features, diagnosis, treatment and prognosis of endometrial stromal sarcoma (ESS). Methods: The clinical data, histology and immunohistochemistry of 32 cases of endometrial stromal sarcoma collected from 2006 to 2012 in Department of Pathology, the Third Affiliated Hospital of Zhengzhou University were observed and analyzed, followed up with literature review. Results: The median age of 32 patients was 49.5 years. The main clinical manifestations were irregular vaginal bleeding, uterine enlargement and uterine space occupying. Hysterectomy and bilateral accessory resection were the main surgical methods. Microscopic tumor cells and proliferative endometrial stromal cells were similar, no significant atypia, the tumor boundary visible serological or irregular island-like infiltration near the uterine smooth muscle bundles, tumor tissue more spiral small arteries. The results of immunohistochemistry showed that CD10 was expressed in 32 ESS tumor cells, Vimentin was expressed in 28 tumor cells, ER was expressed in 24 cases, PR was expressed in 21 cases and Desmin was expressed in only 4 cases. Spiral arterioles in ESS both expressed CD34 and ESS tumor cells Ki-67 positive index is lower. Twenty-six patients received follow-up information (16-96 months) with a median survival of 61 months. One case died within 67 months. Conclusions: ESS is rare in all uterine malignancies. The diagnosis should be based on clinical manifestations and related medical history and be diagnosed on the basis of the pathological features and associated diagnostic judgment of immunohistochemical markers. Surgical-based combination therapy may reduce the risk of patients Recurrence and prolong survival.