颅内原发性Rosai-Dorfman病2例并文献回顾  

Intracranial Primary Rosai Dorfman Disease:2 Cases and Literature Review

作　　者：于春燕 肖平[1] 陈翠芬[1] 赵海燕[2] Yu Chun-yan;Xiao Ping;Chen Cui-fen;Zhao Hai-yan(Department of Medical Imaging,Long Gang District Central Hospital,Shenzhen 518100,Guangdong Province,China;Department of Pathology,Long Gang District Central Hospital,Shenzhen 518100,Guangdong Province,China)

机构地区：[1]广东省深圳市龙岗中心医院医学影像科,广东深圳518100 [2]广东省深圳市龙岗中心医院病理科,广东深圳518100

出　　处：《罕少疾病杂志》2023年第8期17-18,23,共3页Journal of Rare and Uncommon Diseases

摘　　要：目的探讨颅内原发性Rosai-Dorfman病(RDD)的影像学表现及病理学特点,提高对该病的认识。方法对2例经病理证实的颅内原发性RDD患者影像学及病理学特点进行回顾性分析,并对相关文献进行复习。结果2例患者均行CT平扫及头颅MRI平扫加增强检查。病例1位于左侧桥小脑角区,病例2位于两侧丘脑顶部,右侧为主。两例CT均表现为稍高密度肿块,MRI表现T1呈等信号,T2呈等/低信号,增强明显强化,周围伴血管源性水肿。病例1中病灶包绕左侧椎动脉,术中显示肿块侵犯了左侧椎动脉壁。两例病理显示见较多组织细胞,淋巴细胞及浆细胞,病例2可见明显巨大组织细胞浆内可见大小不等的淋巴细胞,即淋巴细胞“伸入”现象;两例免疫组化均显示这些组织细胞S-100蛋白和CD68标记阳性。结论颅内原发性RDD病罕见,易误诊,发生于脑实质的病变更为罕见,相对特异性的影像学表现结合免疫组化检查有助于鉴别诊断。Objective:To explore the imaging and pathological features of primary intracranial Rosai-Dorfman disease(RDD)and improve the understanding of the disease.Methods:the imaging and pathological features of 2 patients with intracranial primary RDD confirmed by pathology were analyzed retrospectively,and the related literatures were reviewed.Results:CT and MRI were performed in both patients.Case 1 was located in the left cerebellopontine angle area,and case 2 was located at the top of the thalamus on both sides,mainly on the right side.The CT manifested as highdensity masses slightly of both cases.On MRI,T1 was isointense,T2 was isointense/hypointense,and the enhancement was significant,with angiogenic edema surrounding.In case 1,the lesion surrounded the left vertebral artery,and the mass invaded the left vertebral artery wall.The pathology of two cases showed that there were many tissue cells,lymphocytes and plasma cells.In case 2,there were obviously large tissue cells and lymphocytes of different sizes were seen in the cytoplasm,that is,the phenomenon of emperipolesis of lymphocytes;immunohistochemistry in both cases showed that the S-100 protein and CD68 marker of these tissue cells were positive.Conclusion:intracranial primary RDD disease is rare and misdiagnosed easily,and the lesions occurred in the brain parenchyma even rarer.The relatively specific imaging findings combined with immunohistochemistry are helpful for differential diagnosis.

关 键 词：ROSAI-DORFMAN病 中枢神经系统 影像学 病理学 

分 类 号：R445.2[医药卫生—影像医学与核医学]