【摘要】目的:探讨肺上皮样血管内皮细胞瘤的临床及病理组织学特征。方法 应用光镜结合免疫组织化学标记对3例肺上皮样血管内皮细胞瘤进行临床病理分析，结合相关文献展开讨论。结果 3例均发生于中年女性。病理学检查大体表现是肺脏孤立性或多发性的大小不等结节。光镜下低倍显示圆到卵圆形的结节，典型的有中心硬化、细胞减少区和外周的富于细胞区。肿瘤典型地播散至邻近的细支气管和肺泡腔，细胞间的间质为粘液软骨样肿瘤背景，肿瘤细胞通常呈圆形或卵圆形，细胞内空泡常见，有时呈印戒样，这提示形成单细胞血管的趋势，单细胞原始管腔是血管内皮细胞瘤的特征。免疫组化染色结果：PEH瘤细胞CD34、CD31及FⅧ、vimentin均阳性，TTF-1、CK(pan),EMA均阴性。结论 肺上皮样血管内皮细胞瘤罕见，好发于中年女性，低到中级别的血管源性肿瘤，以手术治疗为主，辅以放疗和化疗。

    【关键词】肺肿瘤；肺上皮样血管内皮细胞瘤;病理学；免疫组织化学

    【中国分类号】R734.2【文献标识码】A【文章编号】1044-5511(2011)11-0019-02

    【Abstract】Objective: To study the clinical and pathological characteristics, diagnosis and differential diagnosis of pulmonary epithelioid hemangioendothelioma(PEH). Methods: Analyze the clinical manifestations, pathological morphology and immunohistochemical features of PEH after diagnosing three cases and reviewing some literatures. Results: Three cases were middle-aged female. The gross presentation was solitary or multiple nodules in lung. Histologically, the tumors were characterized by oval to round nodulous proliferation, mucocartilagnoid background, centrol hypercellular and sclerotic area, peripheral hypocellular area, invasion to adjacent bronchiole and alveolar. Oval to round epithelioid cells with intracytoplasmic vacuoles, signet-ringoid sometimes, suggested the formation of single-cell vascular, the characteristic of epithelioid hemangioendothelioma. The tumor cells were strongly positive for factorVⅢ, CD31, CD34 and vimentin, negative for TTF-1, CK(pan) and EMA. Conclusion: PEH is a rare low/medium-grade m alignant tumor. Surgery is the main therapy, supplemented by radiotherapy and chemotherapy. ......