肺上皮样血管内皮细胞瘤1例并文献复习

Pulmonary epithelioid hemangioendothelioma：a case report and review of the literature

目的 加强对肺上皮样血管内皮细胞瘤（PEH）的认识,提高诊断率.方法 结合我院诊治的1例PEH患者的临床资料和文献复习,分析该病的病因、临床特点、病理诊断、鉴别诊断、治疗及预后.结果 PEH病因不清,临床症状较少且轻微,多为咳嗽、咳痰、少量咯血；影像学检查主要表现为两肺多发性结节影,病理学检查主要特征是单细胞原始管腔结构；免疫组织化学检查提示其为内皮细胞源性.目前尚无有效治疗措施,预后介于良、恶性肿瘤之间.结论 PEH是一种低度恶性肿瘤,好发于中年女性,临床表现无特异性,易误诊,需肺活检才能诊断.

Objective To describe the clinical features of pulmonary epithelioid hemangioendothelioma （PEH） and heighten the ratio of diagnosis. Methods With a case report and review of the relat- ed literature, the etiology, clinical manifestations, pathology diagnosis, differential diagnosis, management and prognosis of pulmonary epithelioid hemangioendothelioma were described. Results The etiology of this rare disease remained unknown. Symptoms were scanty and usually mild. Chest radiograph or computed tomography usually revealed multiple bilateral pulmonary nodules. Primitive lumen formed by a single cell was the pathologic feature. Immunohistochemical stains showed that the malignant ceils were of the en- dothelial type. There was no effective treatment for this disease and its prognosis was unpredictable. Conclusion PEH is a rare kind of low malignant tumor that easily occur in women. It has no specific clinic features and often misdiagnosed as other pulmonary diseases.