肺上皮样血管内皮瘤一例并文献复习

Pulmonary Epithelioid Hemangioendothelioma:A Case Report and the Literatures Review

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 enhance the understanding of pulmonary epithelioid hemangioendothelioma （PEH）, and improve the diagnostic rate of the disease. Methods The experience of diagnosis and treatment of a case of PEH in August 2013 was reported in the present study and the related literature was reviewed. The etiology, clinical manifestations, pathological diagnosis, differential diagnosis, therapy and prognosis of this disease were described. Results The etiology of PEH remained unknown. Symptoms of PEH were usually nonspecific and mild. Chest radiograph or computed tomography usually revealed calcification in multiple nodules of half or both lungs. Primitive lumen formed by a single cell was the pathologic feature. Immunohistochemical stains showed that the malignant cells were of endothelial type. Genetic analysis was relatively limited currently. There was no effective treatment for this disease and the prognosis was poor. Conclusions PEH is a low potential malignancy occurring exclusively in young women. Symptoms of PEH are usually nonspecific and variable which can easily lead to misdiagnosis. So, pathological diagnosis should be emphasized.