目的提高对肺上皮样血管内皮瘤(PEH)的认识,提高诊断率。方法结合2013年8月诊治的1例PEH患者的临床资料和相关文献复习,分析该病的病因、临床特征、诊断方法、鉴别疾病、治疗及预后。结果 PEH病因不清,临床症状轻微且无特异性,影像学特异性表现为肺内多发结节内钙化病变,病理学检查主要特征是单细胞原始管腔结构,免疫组织化学常表达血管内皮标志物。遗传学分析目前较有限。目前尚无有效治疗措施,预后差。结论 PEH是一种低度恶性肿瘤,好发于年轻女性,临床表现无特异性且变异大,易误诊,因此临床中应强调病理学检查。 Objective To improve the understanding of pulmonary epithelioid hemangioendothelioma (PEH) and improve the diagnosis rate. Methods According to the clinical data and literature review of one PEH treated in August 2013, the etiology, clinical features, diagnosis, diagnosis, treatment, and prognosis of PEH were analyzed. Results The etiology of PEH was unclear. The clinical symptoms were mild and nonspecific. The imaging findings were multiple nodular calcifications in the lung. The primary features of the pathological examination were the single lumen structure of the cells. Immunohistochemistry often expressed vascular endothelial markers Things. Genetic analysis is currently limited. There is no effective treatment, the prognosis is poor. Conclusions PEH is a low-grade malignant tumor that occurs in young women. The clinical manifestations of PEH are nonspecific and vary widely. Therefore, PEH should be emphasized in pathology.