误诊为转移癌的肺朗格汉斯组织细胞增多症两例报告并文献复习  

Pulmonary Langerhans Cell Histiocytosis Mimicking Metastasis of Cancer: Two Cases Report and Literature Review

作　　者：赵婷婷[1] 夏伟 赵琪[1] 马苗[1] 李燕[1] 苗立云[1] 孙琦[3] 张英为[1] 

机构地区：[1]南京大学医学院附属鼓楼医院呼吸科,江苏南京210008 [2]湖北省宜昌市第二人民医院呼吸科,湖北宜昌443000 [3]南京大学医学院附属鼓楼医院病理科,江苏南京210008

出　　处：《中国呼吸与危重监护杂志》2016年第3期250-255,共6页Chinese Journal of Respiratory and Critical Care Medicine

摘　　要：目的探讨具有转移癌影像学特点的肺朗格汉斯组织细胞增多症(PLCH)的临床表现、影像学特征及相关鉴别诊断,以提高对该病的关注和认知。方法回顾性分析2例影像学表现(HRCT及PET/CT)似转移癌的PLCH患者的临床资料,并以"朗格汉斯组织细胞增多症"为关键词在万方数据库、中国知网以及Pubmed数据库中检索相关文献,筛选PLCH误诊的文献,分析其临床表现和胸部影像学特点。结果 2例患者临床表现均主要为咳嗽、咳痰,其PET/CT均提示肺部病灶葡萄糖摄取率增高。1例胸部HRCT表现为双肺弥漫结节影及囊状影,纵隔窗间右侧第4肋骨有局灶性破坏,经外科胸腔镜肋骨活检确诊;1例患者胸部HRCT表现为弥漫结节影,经外科胸腔镜肺活检确诊。2例患者的肺活检病理均提示病灶中多量朗格汉斯组织细胞(LC)及嗜酸粒细胞浸润;LC表达CD1a。共检索到误诊相关文献7篇,均为个案报告。分别误诊为抑郁症、嗜酸粒细胞性肺炎、自身免疫性疾病继发的间质性肺炎、恶性肿瘤。结论当患者胸部影像学表现为转移癌伴或不伴其他器官损害时,在鉴别诊断时需考虑到PLCH可能,尽快完善病理学诊断,有利于患者及早治疗,改善预后。Objective To investigate the clinical,radiographic characteristics and differential diagnosis of pulmonary Langerhans cell histiocytosis（ PLCH） mimicking metastasis of cancer in radiography.Methods Clinical data of 2 patients with PLCH manifesting as metastatic cancer on HRCT and PET / CT were retrospectively analyzed. Patients reported as PLCH on Wan Fang Database,China Knowledge Resource Integrated Database and Pubmed were reviewed to screen misdiagnosis literature and further analyzed the clinical and radiographic characteristics. Results Two cases both presented with cough and sputum.18F-FDG PET / CT showed increased18F-FDG up-take in both nodules in the lungs. One patient presented with multiple nodules,diffuse multiple cystic changes in lungs and osteoclasia in the right 4thrib on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of rib biopsy. The other patient presented with diffuse multiple nodules on HRCT who was diagnosed by a video-assisted thoracoscopic biopsy of lung biopsy. The pathological characteristics of both biopsy specimen demonstrated infiltration by Langerhans cells（ LC） and eosinophils. The LC were positive for CD1 a. Literature review found seven PLCH cases who were misdignosed as depression,eosinophilic pneumonia,interstitial lung disease involvement of autoimmune disorders and malignant tumor. Conclusion When clinician faced with a patient suspected as metastatic cancer by HRCT and PET / CT,it is reasonable to consider PLCH as a differential diagnosis and obtain the pathological information as soon as possible so that better prognosis can be achieved through early intervention.

关 键 词：肺朗格汉斯组织细胞增多症 转移癌 误诊 HRCT PET/CT 

分 类 号：R563[医药卫生—呼吸系统]