CAJ | 학술논문

目的加强对肺上皮样血管内皮瘤(pulmonary epithelioid hemangioendothelioma,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,尤其是CD31、CD34.结论 PEH少见,无特异性临床表现,诊断困难,极易误诊.
목적 가강대폐상피양혈관내피류(pulmonary epithelioid hemangioendothelioma,PEH)적인식,제고학진솔.방법 분석복단대학부속중산의원수치적1례경지기관경폐활검학진위PEH환자적림상자료병복습상관문헌.결과 환자남,61세,인“반복해수4월여”입원.흉부CT시량폐다발결절영,종격림파결종대.PET-CT시좌폐상협당대사이상증고적결절,종격다발당대사이상증고적종대림파결.경지기관경폐활검병리진단위PEH.문헌현시PEH병인불청,림상증상교소차경미;폐부영상학특정성표현위량폐다발성소결절,가유마파리양개변、소협간격증후、개화등표현;병이학주요특정시류세포정소상배렬,포질내가견공포,부분세포정인계양;면역조화시내피세포원성표기물양성,우기시CD31、CD34.결론 PEH소견,무특이성림상표현,진단곤난,겁역오진.
Objective To enhance the recognition of pulmonary epithelioid hemangioendothelioma and heighten the ratio of diagnosis.Methods A case diagnosed with pulmonary epithelioid hemangioendothelioma by transbronchial biopsy was described and the related literature was reviewed.Results A 6l year old male patient was admitted to the hospital because of dry cough for over four months.CT scan showed multiple bilateral pulmonary nodules and the enlarged mediastinal lymph nodes.PET 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 PEH was not clear and most patients were asymptomatic.The characteristic feature of PEH on chest CT was the presence of multiple 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 hemangioendothelioma is a rare tumor of the lung with non-specific clinical manifestations,difficult to diagnose,and easily misdiagnosed.