特发性纵隔纤维化CT与病理表现

CT and Pathology Manifestations of Idiopathic Fibrosing Mediastinitis

目的探讨特发性纵隔纤维化(IFM)的CT与病理表现。方法回顾性分析经病理证实的IFM患者5例,均行MSCT平扫+增强扫描,观察分析其CT和病理表现。结果 5例IFM患者平均年龄40.8岁;弥漫性分布并包绕纵隔血管结构4例,局限性分布1例;"束管征"3例(上腔静脉并双侧头臂静脉狭窄1例,右肺动脉并双侧头臂静脉狭窄1例,右肺动脉并双侧肺静脉狭窄1例),无"束管征"2例;5例均无钙化;伴胸腔积液5例。病理切片示大量纤维组织增生及胶原化,伴不同程度慢性炎性细胞浸润。结论中青年纵隔内弥漫性软组织影,无钙化,伴"束管征"及胸腔积液,应考虑IFM可能;"束管征"的主要原因是纵隔内大量纤维组织及胶原增生。

Objective To analyze the CT and pathology manifestations of Idiopathic Fibrosing Mediastinitis( IFM).Methods A total of 5 patients with IFM proved by pathology were retrospectively analyzed,5 patients underwent enhanced and non-enhanced CT,according to CT findings and pathological findings. Results Average age of the of 5 patients is40. 8 years old; the findings include diffuse distribution and surrounding the mediastinal structure 4 cases,focal distribution1 case; 3 cases with ' bound tube sign'( superior vena cava and bilateral brachiocephalic vein stenosis in one case,right pulmonary artery and bilateral brachiocephalic vein stenosis in 1case,right pulmonary artery and bilateral pulmonary vein stenosis in 1case),2 cases without ' bound tube sign'; 5 cases no calcification; 5 cases with pleural effusion. Pathological photos show a large amount of fibrous tissue and collagen proliferation,accompanied by chronic inflammatory cells infiltration. Conclusion IFM should be considered in young and middle-aged patient with diffuse soft tissue in mediastinal,no calcification,with ' bound tube sign' and pleural effusion; A large amount of fibrous tissue and collagen hyperplasia. is the main reason of 'bound tube sign'.