摘要目的:提高对肺上皮样血管内皮瘤（P-EHE）的诊疗水平。方法:收集2009年1月至2020年7月就诊福建省福州肺科医院确诊为P-EHE的16例患者，其中男8例，女8例，年龄24~66岁，发病中位年龄47.75岁。总结分析影像学表现、病理特征、治疗方案、生存情况等临床资料。结果:16例患者中多数（9/16）经体检发现，部分（7/16）出现咳嗽、咳痰、气促、咯血、胸痛等呼吸系统症状。多数患者（11/16）肿瘤局限在肺部，1例出现骨转移，1例发生淋巴结转移，4例发生胸膜转移。12例患者经外科胸腔镜获得病理组织并确诊，胸部CT影像表现为双肺多发结节为主，结节直径大多数<2.0 cm，并可见钙化；孤立结节及块影表现较少，胸膜转移可表现为胸膜增厚、胸腔积液。病理镜下可见界限清楚的嗜酸小结节，结节中可见不规则状排列的巢团状结构；结节中央细胞少，外周细胞丰富，呈乳头状伸入肺泡腔内；肿瘤细胞呈上皮样，异型性小，个别瘤细胞胞质内可见空泡和红细胞；瘤细胞表达血管内皮细胞标记CD34、CD31、FⅧ、VIM相关抗原。随访0.5~11年，死亡4例，失访2例，其余患者情况良好，中位OS 4.58年。结论:P-EHE是一种罕见的肺低度恶性肿瘤，临床表现无特异性，结合胸部影像及病理免疫组化可诊断。目前尚无标准的治疗方案，多数预后良好。

abstractsObjective:To improve the diagnosis and treatment of pulmonary epithelioid hemangioendothelioma (P-EHE).Methods:Sixteen patients diagnosed with P-EHE in Fuzhou Pulmonary Hospital of Fujian Province from January 2009 to July 2020 were collected. Their gender, age, imaging findings, pathological characteristics, treatment protocols, survival and other clinical data were summarized and analyzed.Results:The ratio of male to female among the 16 patients was 1∶1; and the average age of onset was 47.75 years. Most cases of PEHE (9/16) were found by physical examination, while some cases developed respiratory symptoms such as cough, sputum, shortness of breath, hemoptysis, chest pain, etc. (7/16). In most patients the lesions were localized to the lungs (11/16), while bone metastasis (1/16), lymph node metastasis (1/16), and pleural metastasis (4/16) also occurred. The pathological tissues were obtained mainly through surgical thoracoscopy. Chest CT images showed multiple nodules in both lungs, with most of the nodules less than 2.0 cm in diameter, and calcifications were seen, while solitary nodules and masses were rare, and pleural metastases could be manifested as pleural thickening and pleural effusion. The pathological findings were well-defined eosinophilic nodules with irregularly arranged nest-like structures. Those eosinophilic nodules had few central cells and abundant peripheral cells, which extended into the alveolar cavity like papillae. The tumor cells were epithelioid with small atypia, and vacuoles and red blood cells could be seen in the cytoplasm of individual tumor cells. Immunohistochemically, the tumor cells were positive to CD34, CD31, Factor Ⅷ andvimentin (VIM). Follow-up of 0.5 to 11 years showed that four patients died, two lost to follow-up, and the rest of the patients were in good condition, with a median overall survival (OS) of 4.58 years. Conclusions:PEHE is a rare low-grade lung tumor with no specific clinical manifestations. It can be diagnosed with chest imaging and pathological immunohistochemistry. Moreover, there is currently no standard treatment for PEHE, and most patients have a good prognosis.