肺上皮样血管内皮瘤(附1例报告及文献复习)

Clinical analysis of pulmonary epithelioid hemangioendothelioma-One case report and literature review

目的提高对肺上皮样血管内皮瘤的诊断和治疗。方法通过对1例肺上皮样血管内皮瘤患者的诊治,就本病的临床特点、诊断方法以及治疗和预后等情况进行全面的分析和讨论。结果肺上皮样血管内皮瘤是一种罕见的肺部肿瘤,病因不明,以干咳为主,影像学表现主要为双肺弥漫性小结节影,病理学检查特征是单细胞原始管腔结构、免疫组织化学提示内皮细胞源性,恶性程度并不低,疗效差。结论肺上皮样血管内皮瘤是一种罕见的肺部恶性肿瘤,临床无特异性,极易误诊,目前尚无确切的治疗方法,预后差,临床医生应该提高对其的认识程度。

Objective To improve the understanding of the clinical features and diagnostic value of pulmonary epithelioid hemangioendothelioma. Methods By ease-study method and literature review, clinical features, methods of diagnosis, treatment and prognosis of pulmonary epithelioid hemangioendothelioma were discussed in details in the present study. Results Pulmonary epithelioid hemangioendothelioma was a rare tumor. Moreover, its pathogensis remained unknown. Cough was a major symptom of the disease, however, it was usually mild. Chest radiograph or computed tomography usually indicated multiple bilateral pulmonary nodules. Primitive lumen formed by a single cell was the pathologic feature of the disease, lmmunohistoehemiseal stain showed that the malignant cells were of the endothelial type. The prognosis was poor. Conclusions Pulmonary epithelioid hemangioendothelioma is a rare malignant lung tumor. It is often misdiagnosed as other pulmonary disease. There is no effective treatment at present. Physician should improve the knowledge about and understanding of the disease.