多中心型Castleman病的CT表现特征及其病理学基础

The CT and pathology characteristics of multicentric Castleman's disease

目的:探讨多中心型Castleman病(MCD)的CT表现特征及其病理学基础。方法:回顾性分析经手术病理证实的9例MCD患者的CT表现及病理资料。所有患者均行CT平扫和增强扫描。结果:9例患者中1例为透明血管型,8例为浆细胞型,且其中1例为多中心型CD继发大B细胞淋巴瘤。增大淋巴结位于颈部7例,腋窝8例,胸部7例,腹部6例,腹股沟区6例,平扫呈软组织密度,增强扫描呈明显均匀强化。2例浆细胞型可见肺内病变,其中1例以双肺多发磨玻璃病灶及薄壁空腔为主,1例以弥漫性分布的小结节和多发小叶间隔增厚为主。所有患者中伴脾脏增大4例,伴肝脏及脾脏增大1例,以其中1例继发大B细胞淋巴瘤者脾脏增大明显,最大径≥15.0cm。结论:MCD的CT表现以全身多部位的多发淋巴结增大为主,平扫呈软组织密度,增强扫描呈明显均匀强化,其肺内表现为肺间质性病变,浆细胞型MCD伴脾脏明显增大时有继发淋巴瘤的可能。

Objective： To evaluated the CT imaging characteristics of multicentric Castleman＇s disease （MCD） and correlated with pathology. Methods： The CT findings of 9 patients with surgery and pathology proven MCD were analyzed retrospectively. All patients underwent plain and enhanced CT. Results：Of the 9 cases,there were hyaline-vascular type （HV-CD） in one case and plasma cell type （PC-CD） in 8 cases, one of the PC-CD was secondary to large B cell lymphoma. The location of en- larged lymph nodes included neck （n=7）,axillary （n= 8）,thoracic （n= 7） ,abdomen （n= 6） and in- guinal region （n=6）. On plain CT images,all nodes showed homogeneous soft tissue density,and were markedly,homogeneously enhanced after contrast administration. Two patients with PC-CD showed pulmonary lesions including multiple ground-glass opacities and thin-wall cystic lesions in one and dif- fuse small nodules associated with multiple interlobular septal thickening in the other. Four patients had splenomegaly, one had hepatomegaly as well as splenomegaly. Marked splenomegaly was seen in the patient with secondary large B cell lymphoma with the median diameter larger than 15.0cm. Con- clusion： MCD usually demonstrated as multiple lymph nodes enlargement with soft tissue density and marked homogeneous enhancement, its pulmonary lesions were characterized as interstitial lung dis- ease,when marked splenomegaly is assessed, secondary lymphoma might probably be associated.