颅内原发性Rosai-Dorfman病:附3例报告并文献复习  

Primary intracranial rosai-dorfman disease:report of 3 cases and literature review

作　　者：柴学[1] 张龙江[2] 王娟[3] 宋坤[3] 刘文[1] 蔡宗绕 

机构地区：[1]南京医科大学附属脑科医院放射科,江苏南京210009 [2]南京军区南京总医院医学影像科,江苏南京210002 [3]南京医科大学附属脑科医院病理科,江苏南京210029

出　　处：《医学影像学杂志》2013年第12期1869-1872,共4页Journal of Medical Imaging

基　　金：南京医科大学科技发展基金面上项目(2012NJMU106)

摘　　要：目的探讨颅内原发性Rosai-Dorfman病(RDD)的MRI表现及病理特点,提高对该病的认识。方法对3例经病理证实的RDD患者MRI及病理特点进行回顾性分析,并对相关文献进行复习。结果 3例患者均行头颅MRI平扫加增强检查。2例为多发病灶,1例为单发病灶,均位于脑实质外;T1WI和T2WI均显示呈等信号影,其中2例基于脑膜生长,1例位于脑室内;增强后病灶呈明显均匀强化。显微镜下见较多组织细胞,淋巴细胞及浆细胞浸润伴纤维化,其中巨大组织细胞浆内可见大小不等的淋巴细胞;免疫组化显示这些组织细胞S-100蛋白和CD68标记阳性。结论颅内Rosai-Dorfman病较少见,易误诊,免疫组化检查有助于鉴别诊断。若MR检查发现基于脑膜生长的多发或孤立性病灶,应考虑到Rosai-Dorfman病的可能。Objective To investigate the magnetic resonance imaging （MRI） manifestations and pathological features of primary intracranial Rosai-Dorfman disease （RDD）, so as to improve the knowledge of this disease. Methods 3 cases of primary intracranial RDD confirmed pathologically were evaluated by analyzing MRI and pathologic features, along with literature review. Results 3 cases perfomed by MRI plain scan and enhanced scan were located outtside the brain paren chyma, of which 2 cases were multiple lesions, 1 case was single lesiongs. T1WI and T2WI scan showed the lesions, of which 2 cases were mening growth based and 1 case was in the ventriciles. After enhancement, the lesions were showed homogeneous enhancement. Histologically, the lesions consisted of variable numbers of pale-stained histocytes with empe ripolesis were positive for S-100 protein and CD68 by immunohistochemical analysis. Conclusion Primary intracranial Ro sai-Dorfman disease is rare, which could be diagnosed by immunhistochemical examination. The possibility of central nerv ous system Rosai-Dorfman disease should be considered when single or multiple dural-based mass lesions.

关 键 词：磁共振成像 R0sai-D0r fman病 

分 类 号：R445.2[医药卫生—影像医学与核医学] R739.41[医药卫生—诊断学]