纤维素性纵隔炎课件

纤维素性纵隔炎 呼吸与危重症医学科 陈文慧 病史、查体 n女性，74岁，汉族 n反复咳嗽、咳痰，喘息10余年，加重7天 n一直诊断为慢性阻塞性肺疾病 n近一年来活动耐力下降 n否认结核病史，吸烟史20年，20支/天 n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， 辅助检查 n血气分析（未吸氧）：pH7.517, PaCO229.3 mmHg，PaO252.2mmHg。 n血气分析（吸氧2L/min）：pH7.493, PaCO232.4 mmHg，PaO260.8mmHg。 n阻塞性通气功能障碍（FEV1/FVC：71.34%， FEV1实/预：85.7%） n心脏彩超：肺动脉高压（重度），TI法估测肺 动脉收缩压105mmHg， 影像学 概念 n纵膈中无细胞成分的胶原和纤维组织的良性增 生 n上腔静脉、肺动脉、肺静脉、气管、食管 n分类 n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 n广泛：非钙化肿块；广泛区域受侵；可能与腹膜 后纤维化有关 病因 n硬化性纵隔炎/纵膈纤维化 n纵膈非特异性纤维炎症性病变 n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 n多发生于美国组织胞浆菌的流行区域 n组织胞浆菌抗原的皮肤反应阳性 n肉芽肿性炎 n部分病例组织标本中可找到组织胞浆菌 纤维素性纵隔炎与纵膈肉芽肿 n纵膈肉芽肿 n增大、粘连的淋巴结 n含干酪或纤维性物质 n有完整包膜，不引起周围组织的入侵 n无症状或压迫上腔静脉或食管引起的症状 n肉芽肿的破裂引起局部或广泛纵隔纤维化 n34%肉芽肿进展为纤维素性纵隔炎 纵膈肉芽肿 Figure 1. Mediastinal granuloma due to histoplasmosis in a 30-year-old man with chest pain. (a) Posteroanterior chest radiograph shows a calcified right paratracheal mass (arrowhead). (b) CT scan (mediastinal window) shows the focal paratracheal mass with a low-attenuation center and extensive calcification (arrowhead). Note the mass effect on the trachea (T). A noninvasive, well-encapsulated mass containing viable H capsulatum organisms was found at resection. 相关疾病 n结核、曲霉菌、毛霉菌、芽生菌 、隐球菌 n自身免疫性疾病 n白塞病 n风湿热 n放疗 n外伤 n何杰金淋巴瘤 n非特异性炎症性病变 n腹膜后纤维化 n硬化性胆管炎 n眼眶假瘤 病例1-影像学（58岁男性，咳嗽6个月） (a) Computed tomographic (CT) scan (lung window) shows an infiltrating, soft-tissue right hilar mass extending into the right lower lobe along bronchovascular bundles. (b) CT scan (mediastinal window) shows the soft-tissue mass (arrowhead) and extensive calcification in the right hilum and subcarinal region. 病例1-大体病理 n(c) Photograph of the cut surface of the resected specimen shows dense white fibrous tissue in the right hilum extending into the lung parenchyma along bronchovascular bundles (arrows). 病理学表现 Fibrosing mediastinitis. Medium-power photomicrograph (original magnification, 25; hematoxylin-eosin stain) demonstrates fibrous tissue (arrows) infiltrating mediastinal adipose tissue. Fibrosing mediastinitis. High-power photomicrograph (original magnification, 100; hematoxylin-eosin stain) demonstrates paucicellular, eosinophilic mature collagen, findings typical of fibrosing 鉴别诊断 n局限性纤维化-组织胞浆菌病、结核 n恶性肿瘤 n何杰金淋巴瘤 n非何杰金氏淋巴瘤 n胸膜纤维性肿瘤CD34、bcl-2 n胸膜间皮瘤-keratin n转移癌的纤维炎症反应 n胸腺瘤 n胸腺类癌 n纤维瘤病 n肉瘤 组织胞浆菌病 (a) Medium-power photomicrograph (original magnification, 50; hematoxylin-eosin stain) of a hilar lymph node specimen shows a caseating granuloma composed of a rim of epithelioid histocytes (arrowheads) surrounding central caseous necrosis (N). Note peripheral lymphoid infiltrate (L). (b) Oil immersion photomicrograph (original magnification, 500; Grocott methenamine-silver stain) shows small oval-shaped yeasts of H capsulatum. Note the rare budding forms (arrowhead). 非何杰金氏淋巴瘤 (a) High-power photomicrograph (original magnification, 400; hematoxylin-eosin stain) demonstrates hypercellular atypical lymphoid infiltrate. (b) High-power photomicrograph (original magnification, 400) of a specimen stained with immunohistochemistry for CD20 (B-cell marker) shows positive cytoplasmic staining 临床表现-1 临床表现-2 n上腔静脉 n上腔静脉综合症 n中央气道 n咳嗽、呼吸困难 n反复肺炎 n肺不张 n肺静脉 n假二尖瓣狭窄症状 n劳力性呼吸困难 n咯血 n肺动脉高压、肺心病 n肺动脉 n肺动脉高压（少见） 治疗和预后 n死亡原因 n咯血、肺心病、反复肺部感染 n治疗 n抗真菌治疗 n激素 n手术 n并发症治疗 n球囊扩张 n激光治疗 n血管内或气道内支架 治疗 (a) CT scan (mediastinal window) shows a wire mesh stent in the left main bronchus, calcified adenopathy (arrow) in the aortopulmonary window, and a subcarinal soft-tissue mass (arrowhead). = esophagus. (b) CT scan (lung window) shows an outpouching (arrowhead) of the anterior esophageal lumen adjacent to the stent that was confirmed to represent a bronchoesophageal fistula at a barium swallow examination (not shown). Note lingular consolidation, which most likely represents pneumonia. A B C D 影像学表现 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 影像学表现-2 影像学表现(3)-55岁男性，咳嗽、咯血 (a) Posteroanterior chest radiograph shows a left hilar mass. (b) Coronal T1-weighted (image demonstrates the left hilar mass (arrowheads) of heterogeneous low-to-intermediate signal intensity obstructing the left main bronchus 影像学表现(4)-44岁女性，反复肺炎 (a) CT scan (mediastinal window) demonstrates a calcified right hilar and mediastinal mass obstructing the right upper lobe bronchus. Note the right pleural thickening (arrows), patent bronchus intermedius (arrowhead), and enlarged azygous vein (a). R = right pulmonary artery, S = superior vena cava. (b) Axial T1-weighted (680/20) MR image shows an infiltrative hilar mass of intermediate signal intensity narrowing the right upper lobe bronchus (arrow). Note the narrowed but patent superior vena cava (arrowhead) 影像学表现(5)-22岁男性 n影像学特征(3)- (a) Contrast-enhanced CT scan (mediastinal window) shows a soft-tissue attenuation mass diffusely infiltrating the mediastinum. Note encasement and narrowing of the left main bronchus () (b) Contrast-enhanced CT scan (mediastinal window) obtained at a more caudal level shows the subcarinal mass (M), encasement of the left main coronary artery (arrow), and narrowing of the left superior pulmonar