颅内原发性Rosai-Dorfman病1例报道及文献回顾  

Primary Intracranial Rosai-Dorfman Disease:Case Report and Literature Review

作　　者：冼丽芳 文剑明[1,2] 叶玉清[1] 邓小雯 

机构地区：[1]澳门镜湖医院病理科 [2]中山大学第一附属医院病理科,广东广州510080 [3]澳门镜湖医院影像中心

出　　处：《中国神经肿瘤杂志》2009年第3期196-199,共4页Chinese Journal of Neuro-Oncology

摘　　要：背景与目的:Rosai-Dorfman病(Rosai-Dorfman disease,RDD)是一种少见的良性组织细胞增生性疾病,多位于淋巴结内,颅内RDD极少见。本文探讨颅内RDD的病理、诊断、鉴别诊断及治疗。方法:对1例颅内RDD病例进行病理形态观察和免疫组化检测,并对文献报道进行回顾。结果:本例患者病灶位于右侧额、颞部和基底池,肿物基底位于硬膜。病理和免疫组化见病变组织由数量不等的成熟淋巴细胞、浆细胞及S-100、CD68标记阳性的组织细胞构成,组织细胞内有吞噬淋巴细胞或淋巴细胞"伸入现象"。结论:原发性颅内窦组织细胞增生症是一种原发于中枢神经系统的罕见的良性组织细胞增生性疾病,缺乏特异性临床表现,确诊依赖病理学检查。因本例在颅内有多个病灶,手术难以全部切除,采用保守治疗包括使用激素效果良好。BACKGROUND ＆ OBJECTIVE： Rosai-Dorfman disease （RDD） is an uncommon, benign histiocytic proliferative disorder, which commonly affects the lymph nodes. Intracranial RDD is rare. In this study, we discussed the pathology, diagnosis, differential diagnosis and treatment for primary intracranial RDD. METHODS： A case of intracerebral RDD was studied by histopathological observation and immunohistochemical staining. Related literature was also reviewed. RESULTS： The lesions in our case were located in the right frontaltemporal region and based on dura. Pathological and immunohistochemical examinations showed the lesion consisted of variable numbers of mature lymphocytes, plasma cells and histiocytes, and the latter were positive for S-100 protein and CD68. A histocyfie phagocytosis of lymphoeytes, so-called emperipolesis of lymphocytes was observed. CONCLUSION： Intraeranial Rosai-Dorfman disease is a rather rare nonmalignant histiocytosis disease primarily involving central nervous system. As special clinical representation was absent, the final diagnosis relies on pathological exantination. Due to multiple intracranial lesions, it is difficult to remove the tumor entirely in the case we reported, but a good outcome was achieved by conserved therapies including using corticosteroids.

关 键 词：ROSAI-DORFMAN病 颅内 诊断与治疗 

分 类 号：R739.41[医药卫生—肿瘤]