肺上皮样血管内皮瘤的临床和病理特征

Clinical and pathological features of pulmonary epithelioid hemangioendothelioma

目的加强对肺上皮样血管内皮瘤（pulmmtoryepithelioidhemangioendothelioma，PEH）的认识，提高确诊率。方法分析复旦大学附属中山医院收治的1例经支气管镜肺活检确诊为PEH患者的临床资料并复习相关文献。结果患者男，61岁，因“反复咳嗽4月余”入院。胸部CT示两肺多发结节影，纵隔淋巴结肿大。PET—CT示左肺上叶糖代谢异常增高的结节，纵隔多发糖代谢异常增高的肿大淋巴结。经支气管镜肺活检病理诊断为PEH。文献显示PEH病因不清，临床症状较少且轻微；肺部影像学特征性表现为两肺多发性小结节，可有磨玻璃样改变、小叶间隔增厚、钙化等表现；病理学主要特征是瘤细胞呈巢状排列，胞质内可见空泡，部分细胞呈印戒样；免疫组化示内皮细胞源性标记物阳性，尤其足CD31、CD34。结论PEH少见，无特异性临床表现，诊断困难，极易误诊。

Objective To enhance the recognition of and heighten the ratio of diagnosis. hemangioendothelioma by transbronchial Methods biopsy was A case described hemangioendot heliorna Results A 61 year old male patient was admitted to the hospital because of dry cough for over four months. CT scan showed muhiple bilateral pulmonary nodules and the enlarged mediastinal lymph nodes. PFT CT showed the nodule on left upper lobe and mediastinal lymph nodes with increased glucose metabolism. The final diagnosis was made by transbronchial biopsy pathology. Literatures showed that the etiology of PFH was not clear and most patients were asymptomatic. The characteristic feature of PEH on chest （＇T was the presence of muhiple bilateral perivascular nodules, ground-glass opacity, interlobular septal thickening,and calcification. The pathological characteristics typically included tumor cells arranging in nests and intracytoplasmic vacuoles. Some cells showed a signet ring-like shape. Immunohistochemistry showed that endothelium derived markers were positive, especially CD31, CD34. Conclusions Pulmonary epithelioid hemangloendothelioma is a rare tumor of the lung with non specific clinical manifestations, difficult to diagnose,and easily misdiagnosed.