纤维素性纵隔炎

纤维素性纵隔炎呼吸与危重症医学科陈文慧5/9/20241纤维素性纵隔炎病史、查体女性，74岁，汉族反复咳嗽、咳痰，喘息10余年，加重7天一直诊断为慢性阻塞性肺疾病近一年来活动耐力下降否认结核病史，吸烟史20年，20支/天查体：口唇轻度紫绀，球结膜无水肿，颈静脉充盈。桶状胸，双肺呼吸音减低，呼气相延长，双下肺可闻及呼气相干性啰音，5/9/20242纤维素性纵隔炎辅助检查血气分析（未吸氧）：pH7.517,PaCO229.3mmHg，PaO252.2mmHg。血气分析（吸氧2L/min）：pH7.493,PaCO232.4mmHg，PaO260.8mmHg。阻塞性通气功能障碍（FEV1/FVC：71.34%，FEV1实/预：85.7%）心脏彩超：肺动脉高压（重度），TI法估测肺动脉收缩压105mmHg，5/9/20243纤维素性纵隔炎影像学5/9/20244纤维素性纵隔炎概念纵膈中无细胞成分的胶原和纤维组织的良性增生上腔静脉、肺动脉、肺静脉、气管、食管分类局限：肺门、气管旁及心包旁；局限、钙化肿块；与组织胞浆菌或结核感染有关广泛：非钙化肿块；广泛区域受侵；可能与腹膜后纤维化有关5/9/20245纤维素性纵隔炎病因硬化性纵隔炎/纵膈纤维化纵膈非特异性纤维炎症性病变多数与组织胞浆菌感染引起的异常免疫反应有关；遗传易感性多发生于美国组织胞浆菌的流行区域组织胞浆菌抗原的皮肤反应阳性肉芽肿性炎部分病例组织标本中可找到组织胞浆菌5/9/20246纤维素性纵隔炎纤维素性纵隔炎与纵膈肉芽肿纵膈肉芽肿增大、粘连的淋巴结含干酪或纤维性物质有完整包膜，不引起周围组织的入侵无症状或压迫上腔静脉或食管引起的症状肉芽肿的破裂引起局部或广泛纵隔纤维化34%肉芽肿进展为纤维素性纵隔炎5/9/20247纤维素性纵隔炎纵膈肉芽肿Figure1.

Mediastinalgranulomaduetohistoplasmosisina30-year-oldmanwithchestpain.(a)Posteroanteriorchestradiographshowsacalcifiedrightparatrachealmass(arrowhead).(b)CTscan(mediastinalwindow)showsthefocalparatrachealmasswithalow-attenuationcenterandextensivecalcification(arrowhead).Notethemasseffectonthetrachea(T).Anoninvasive,well-encapsulatedmasscontainingviableHcapsulatumorganismswasfoundatresection.5/9/20248纤维素性纵隔炎相关疾病结核、曲霉菌、毛霉菌、芽生菌、隐球菌自身免疫性疾病白塞病风湿热放疗外伤何杰金淋巴瘤非特异性炎症性病变腹膜后纤维化硬化性胆管炎眼眶假瘤5/9/20249纤维素性纵隔炎病例1-影像学（58岁男性，咳嗽6个月）(a)Computedtomographic(CT)scan(lungwindow)showsaninfiltrating,soft-tissuerighthilarmassextendingintotherightlowerlobealongbronchovascularbundles.(b)CTscan(mediastinalwindow)showsthesoft-tissuemass(arrowhead)andextensivecalcificationintherighthilumandsubcarinalregion.5/9/202410纤维素性纵隔炎病例1-大体病理(c)Photographofthecutsurfaceoftheresectedspecimenshowsdensewhitefibroustissueintherighthilumextendingintothelungparenchymaalongbronchovascularbundles(arrows).5/9/202411纤维素性纵隔炎病理学表现Fibrosingmediastinitis.Medium-powerphotomicrograph(originalmagnification,×25;hematoxylin-eosinstain)demonstratesfibroustissue(arrows)infiltratingmediastinaladiposetissue.

Fibrosingmediastinitis.High-powerphotomicrograph(originalmagnification,×100;hematoxylin-eosinstain)demonstratespaucicellular,eosinophilicmaturecollagen,findingstypicaloffibrosing

5/9/202412纤维素性纵隔炎鉴别诊断局限性纤维化-组织胞浆菌病、结核恶性肿瘤何杰金淋巴瘤非何杰金氏淋巴瘤胸膜纤维性肿瘤—CD34、bcl-2胸膜间皮瘤-keratin转移癌的纤维炎症反应胸腺瘤胸腺类癌纤维瘤病肉瘤5/9/202413纤维素性纵隔炎组织胞浆菌病(a)Medium-powerphotomicrograph(originalmagnification,×50;hematoxylin-eosinstain)ofahilarlymphnodespecimenshowsacaseatinggranulomacomposedofarimofepithelioidhistocytes(arrowheads)surroundingcentralcaseousnecrosis(N).Noteperipherallymphoidinfiltrate(L).(b)Oilimmersionphotomicrograph(originalmagnification,×500;Grocottmethenamine-silverstain)showssmalloval-shapedyeastsofHcapsulatum.Notetherarebuddingforms(arrowhead).5/9/202414纤维素性纵隔炎非何杰金氏淋巴瘤(a)High-powerphotomicrograph(originalmagnification,×400;hematoxylin-eosinstain)demonstrateshypercellularatypicallymphoidinfiltrate.(b)High-powerphotomicrograph(originalmagnification,×400)ofaspecimenstainedwithimmunohistochemistryforCD20(B-cellmarker)showspositivecytoplasmicstaining5/9/202415纤维素性纵隔炎临床表现-15/9/202416纤维素性纵隔炎临床表现-2上腔静脉上腔静脉综合症中央气道咳嗽、呼吸困难反复肺炎肺不张肺静脉假二尖瓣狭窄症状劳力性呼吸困难咯血肺动脉高压、肺心病肺动脉肺动脉高压（少见）5/9/202417纤维素性纵隔炎治疗和预后死亡原因咯血、肺心病、反复肺部感染治疗抗真菌治疗激素手术并发症治疗球囊扩张激光治疗血管内或气道内支架5/9/202418纤维素性纵隔炎治疗(a)CTscan(mediastinalwindow)showsawiremeshstentintheleftmainbronchus,calcifiedadenopathy(arrow)intheaortopulmonarywindow,andasubcarinalsoft-tissuemass(arrowhead).∗=esophagus.(b)CTscan(lungwindow)showsanoutpouching(arrowhead)oftheanterioresophageallumenadjacenttothestentthatwasconfirmedtorepresentabronchoesophagealfistulaatabariumswallowexamination(notshown).Notelingularconsolidation,whichmostlikelyrepresentspneumonia.5/9/202419纤维素性纵隔炎ABCD5/9/202420纤维素性纵隔炎影像学表现5/9/202421纤维素性纵隔炎影像学表现-1(a)33岁女性，胸痛，前纵隔肿物(b)43岁女性，慢性咳嗽、呼吸困难，中、后纵膈肿物5/9/202422纤维素性纵隔炎影像学表现-25/9/202423纤维素性纵隔炎影像学表现(3)-55岁男性，咳嗽、咯血(a)Posteroanteriorchestradiographshowsalefthilarmass.(b)CoronalT1-weighted(imagedemonstratesthelefthilarmass(arrowheads)ofheterogeneouslow-to-intermediatesignalintensityobstructingtheleftmainbronchus5/9/202424纤维素性纵隔炎影像学表现(4)-44岁女性，反复肺炎(a)CTscan(mediastinalwindow)demonstratesacalcifiedrighthilarandmediastinalmassobstructingtherightupperlobebronchus.Notetherightpleuralthickening(arrows),patentbronchusintermedius(arrowhead),andenlargedazygousvein(a).R=rightpulmonaryartery,S=superiorvenacava.(b)AxialT1-weighted(680/20)MRimage

showsaninfiltrativehilarmassofintermediatesignalintensitynarrowingtherightupperlobebronchus(arrow).Notethenarrowedbutpatentsuperiorvenacava(arrowhead)5/9/202425纤维素性纵隔炎影像学表现(5)-22岁男性影像学特征(3)-(a)Contrast-enhancedCTscan(mediastinalwindow)showsasoft-tissueattenuationmassdiffuselyinfiltratingthemediastinum.Noteencasementandnarrowingoftheleftmainbronchus(∗)(b)Contrast-enhancedCTscan(mediastinalwindow)obtainedatamorecaudallevelshowsthesubcarinalmass(M),encasementoftheleftmaincoronaryartery(arrow),andnarrowingoftheleftsuperiorpulmonaryvein(S).Not