弥漫性肺脑膜瘤样结节一例并文献复习

Diffuse pulmonary meningotheliomatosis: a case report and literature review

目的分析微小肺脑膜瘤样结节(MPMN)中的特殊类型弥漫性肺脑膜瘤样结节(DPM)的临床、影像学、病理所见,提高临床医生对DPM的认识。方法回顾性分析北京大学第三医院呼吸与危重症医学科2016年8月收治的1例DPM患者的临床资料,并进行相关文献复习。以"肺脑膜瘤样结节"、"微小肺脑膜瘤样结节"为关键词检索中国知网、万方数据库,以"minute pulmonary meningothelial-like nodules"、"diffuse pulmonary meningotheliomatosis"为关键词检索PubMed数据库,检索2017年11月1日之前的所有中文和英文文献。共检索到文献25篇,其中6篇(均为英文文献)单独以"diffuse pulmonary meningotheliomatosis"为关键词检索获得,均为病例报告,共有10例;余19篇关于MPMN研究的文献(中文1篇,英文18篇)主要探讨非DPM类型的病例,包括病例报告13篇,病理或基因分析研究6篇。结果患者女,68岁,因"间断咳嗽、咳痰3年余,加重3周"入院。胸部高分辨率CT示双肺弥漫性微小结节影,呈随机分布,口服"莫西沙星"治疗2周病变无好转。电视胸腔镜肺活检病理显示肺组织内中等大小上皮样细胞团呈巢状或旋涡样改变,免疫组织化学染色显示细胞表达孕激素受体、波形蛋白及上皮膜抗原,符合微小肺脑膜瘤样结节特点,未发现其他肺实质病变,结合影像特点最终诊断为DPM。未行特殊治疗,随诊1年后复查胸部CT病变无明显加重。DPM是MPMN的罕见类型。检索文献及本例在内共11例DPM患者,男1例,女10例,年龄51~75岁,平均(64±8)岁。8例否认吸烟史或职业接触史;7例表现为干咳或气短,伴或不伴乏力;8例肺功能轻度障碍。所有患者胸部CT均表现为双肺弥漫性多发结节影或网状结节影、实性或磨玻璃密度影,部分结节有空腔样改变,呈随机分布,直径多<5mm;除1例合并肺栓塞外,其余10例胸部CT未发现其他病变。11例均为病理确诊,活检方式为外科肺活检8例,经支气管镜肺活检2例,胸部CT引导下针吸活检1例。随访3例,随访时间为12~92个月,肺部病变均无明显加重。结论DPM是MPMN的罕见类型,临床表现缺乏特异性,影像学表现为双肺弥漫性随机分布的微小结节影,病理检查有助于早期确诊。

Objective To analyze the clinical features, diagnosis and differential diagnosis of diffuse pulmonary meningotheliomatosis (DPM), a rare subtype of minute pulmonary meningothelial-like nodules (MPMN), so as to improve the understanding of this disorder. Methods The clinical data of a patient diagnosed as DPM admitted in department of Respiratory Medicine, Peking University Third Hospital in August 2016 were reported and the related literatures were reviewed. With "pulmonary meningothelial-like nodules" , "minute pulmonary meningothelial-like nodules" or "diffuse pulmonary meningotheliomatosis" as the search terms, and the search time before November 1st 2017 for Wanfangdata, China National Knowledge Infrastructure (CNKI), and PubMed.Twenty-five articles were retrieved, among which 6 English (all case reports) articles were found with the search term "diffuse pulmonary meningotheliomatosis" involving 10 cases, and the other 19 articles (1 Chinese, 18 English) about MPMN mainly discussed non-DPM cases,including 13 case reports and 6 pathological/genetic studies. Results A 68-year old female was admitted to the hospital because of intermittent cough and expectoration for more than 3 years and aggravation for 3 weeks. Her pulmonary CT showed diffuse minute nodules distributed randomly throughout both lungs, and did not improve after treatment with oral Moxifloxacin for 2 weeks. A video-assisted thoracoscopic biopsy was performed. The specimens showed MPMN and no other lesions, which confirmed the final diagnosis of DPM. She was followed without any medication therapy and her pulmonary CT showed stabilization of the lesions one year later. DPM was considered as a rare type of MPMN. A total of 11 cases were analyzed, including 10 cases reported in the literature. The patients included 1man and 10 women, aged 51 to 75 years, with an average age of (64±8) years. Among the 11 patients, 8 denied a history of smoking or of occupational exposure, 7 presented with dyspnea, shortness of breath, or fatigue, and 8 had mild abnormalities of pulmonary function test. The pulmonary CT of all the patients showed randomly distributed, diffuse bilateral small solid or ground-glass nodules, with the diameters less than 5 millimeters, some of which were cavitated. Only 1 patient had pulmonary thromboembolism, while the other 10 did not have any other lung diseases. All the patients were histologically diagnosed, 8 by open lung biopsies, 2 by transbronchial lung biopsy, and 1 by CT-guided fine needle aspiration. Three patients were followed for 12-92 months and the lung lesions were all stable. Conclusions As a rare subtype of MPMN, DPM lacks specificity of clinical manifestations, and presents with diffuse bilateral small solid or ground-glass nodules, distributed randomly throughout both lungs. An early correct diagnosis depends on biopsy.