子宫体恶性血管周上皮样细胞肿瘤的临床病理分析  

Malignant perivascular epithelioid cell tumors of the uterine body:a clinicopathological features

作　　者：李倩茹 李众 马紫瑜 金华 邵立伟 李菲菲 刘爱军 LI Qian-ru;LI Zhong;MA Zi-yu;JIN Hua;SHAO Li-wei;LI Fei-fei;LIU Ai-jun(Department of Pathology,the Seventh Medical Center of PLA General Hospital,Bejing 100700,China;Department of Pathology,the First Medical Center of PLA General Hospital,Beijing 100853,China)

机构地区：[1]中国人民解放军总医院第七医学中心病理科,北京100700 [2]中国人民解放军总医院第一医学中心病理科,北京100853

出　　处：《诊断病理学杂志》2024年第5期378-382,共5页Chinese Journal of Diagnostic Pathology

摘　　要：目的探讨子宫体恶性血管周上皮样细胞肿瘤(perivascular epithelioid cell tumor,PEComa)的临床及病理特征,旨在加深认识该疾病。方法收集解放军总医院第一、第七医学中心5例及文献报道12例子宫体恶性PEComa的相关资料,分析总结该疾病的诊断、鉴别诊断及预后相关因素。结果患者发病年龄23~67岁,主要表现为子宫异常出血和(或)盆腔肿块,个别在体检时意外发现肿瘤。肿瘤由上皮样和/或梭形细胞组成,瘤细胞常围绕薄壁血管呈放射状排列,细胞异型性明显,核分裂易见,常见肿瘤坏死,可见侵犯血管。肿瘤特异性表达肌源性和黑色素标记。所有病例均接受至少全子宫切除等不同手术方式,7例术后接受化疗。随访5~72个月,4例(其中2例接受化疗)术后复发/转移、死亡。结论子宫体恶性PEComa诊断基于病理组织形态及免疫表型。手术切除为主要治疗方案,而辅助治疗效果不明确。长期随访是及早发现疾病进展及治疗的关键。Objective To study the clinical and pathological features of malignant perivascular epithelioid cell tumor(PEComa)of the uterine body in order to understand the disease better.Methods The data of 5 cases of malignant PEComa of uterine body in our hospital and 12 cases reported in literature were collected.We analyzed and summarize the diagnosis,differential diagnosis and prognostic factors of the disease.Results The age of patients ranged from 23 to 67 years.The main manifestations were abnormal uterine bleeding and(or)pelvic mass,occasionally in the physical examination of accidental discovery of tumors.The tumor was composed of epithelioid and/or spindle cells,which were often radially arranged around thin-walled blood vessels and were characterized by cell atypia,mitosis,tumor necrosis,and vascular invasion.Tumor cells specifically expressed myogenic and melanin markers.All patients received at least total hysterectomy,and 7 patients received postoperative chemotherapy.Follow-up ranged from 5 to 72 months.4 cases recurred,metastasized and died after operation,2 of them received chemotherapy.Conclusion The diagnosis of malignant PEComa of the uterine body is based on histopathology and immunohistochemistry.Tumor resection is the main treatment,but the effect of adjuvant therapy is not clear.Long-term follow-up is the key to early detection of disease progression and treatment.

关 键 词：子宫 恶性血管周上皮样细胞肿瘤 病理诊断 预后 鉴别诊断 

分 类 号：R737.33[医药卫生—肿瘤]