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
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).
(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).
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
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.
Fibrosing mediastinitis. High-power photomicrograph (original magnification, ×100; hematoxylin-eosin stain) demonstrates paucicellular, eosinophilic
mature collagen, findings typical of fibrosing
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
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
病理学表现
Fibrosing mediastinitis. Medium-power photomicrograph (original magnification, ×25; hematoxylin-eosin stain) demonstrates fibrous tissue (arrows) infiltrating mediastinal adipose tissue.
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.
(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
辅助检查
血气分析（未吸氧）：pH7.517, PaCO229.3 mmHg，PaO252.2mmHg。
血气分析（吸氧2L/min）：pH7.493, PaCO232.4 mmHg，PaO260.8mmHg。
阻塞性通气功能障碍（FEV1/FVC：71.34%， FEV1实/预：85.7%）
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，
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Βιβλιοθήκη 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