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目的 探讨胸膜上皮样血管内皮瘤的病因、临床表现、诊断、鉴别诊断、治疗及预后.方法 回顾性分析北京协和医院呼吸科收治的1例胸膜上皮样血管内皮瘤患者的临床资料并进行相关文献复习.以“胸膜上皮样血管内皮瘤”为检索词检索万方数据库和中国期刊网全文数据库(CNKI),以“pleural epithelioid hemangioendothelioma”为检索词检索PubMed、Embase、Ovid及Cochrane数据库,检索时间为1975年1月至2013年12月.结果 本例为40岁男性,临床表现为胸痛,胸部CT提示左侧胸膜增厚及胸腔积液,胸膜病理可见上皮样瘤细胞,免疫组织化学染色CD31、CD34、波形蛋白阳性.在以上数据库共检索到68篇文献,剔除50篇非相关文献,共检索到18篇相关文献共29例胸膜上皮样血管内皮瘤病例,包括本例在内共30例,男20例,女10例,年龄31 ～ 82岁,平均50.3岁.该病病因不清,临床表现为胸痛、咳嗽及呼吸困难等,影像学主要表现为胸腔积液、胸膜增厚或肿块;组织学主要表现为上皮样瘤细胞;血管内皮源性免疫标志物阳性.平均生存时间为8.2个月.结论 胸膜上皮样血管内皮瘤罕见,病因不清.临床及影像学表现无特异性,诊断及鉴别诊断主要依靠病理,需与腺癌、血管肉瘤、间皮瘤、肺上皮样血管内皮瘤等鉴别.目前尚无有效治疗措施,与肺上皮样血管内皮瘤相比,本病预后差.“,”Objective To explore the etiology,clinical manifestation,diagnosis,differential diagnosis,management and prognosis of pleural epithelioid hemangioendothelioma.Methods The clinical data of a patient with pleural epithelioid hemangioend othelioma admitted to Peking Union Medical College Hospital were retrospectively analyzed and the related literature was reviewed.We searched databases PubMed,Embase,Ovid,Cochrane,Wanfang and Chinese National Knowledge infrastructure (CNKI) using the keyword “pleural epithelioid hemangioendothelioma” by December 2013.Results The patient was a 40-year-old male presented with chest pain,and his chest CT scan revealed thickening of the left pleura and left pleural effusion.Biopsy of the pleura showed epithelioid tumor cells,and immunohistochemistry was positive for CD31,CD34 and vimentin.From January 1975 to December 2013,18 related articles were retrieved and 29 cases of pleural epithelioid hemangioendothelioma were reported.Among all 30 cases,there were 20 males and 10 females,ranging from 31 to 82 years old,and the average age was 50.3 years old.The etiology of the disease remained unknown.Chest pain,cough,and dyspnea were the common symptoms.Computed tomography usually revealed pleural effusion and pleural thickening or mass.Histological examinations revealed mainly epithelioid cells.Immunohistochemical stains were positive for vascular endothelial markers.The mean survival time was 8.2 months.Conclusions Pleural epithelioid hemangioendothelioma is rare and the etiology is unknown.Clinical and imaging manifestations are not specific,and diagnosis is relied on histological findings.It should be differentiated from adenocarcinoma,hemangiosarcoma,mesothelioma and pulmonary epithelioid hemangioendothelioma.There is no effective treatment and its prognosis is poorer than its pulmonary counterpart.