伴IgG4阳性浆细胞数量增多的脑膜Rosai-Dorfman病临床病理特点分析

Clinicopathological features of meningeal Rosai-Dorfman's disease with increased number of IgG4 positive plasma cells

关键词:  Rosai-Dorfman病  IgG4阳性浆细胞数量增多  IgG4相关性疾病

Key words:  Rosai-Dorfman diseas  increased number of IgG4 positive plasma cells  IgG4 related disease

摘要:

目的　探讨脑膜Rosai-Dorfman病临床病理特点。方法　选取大连医科大学附属第二医院神经外科脑膜原发性伴IgG4阳性浆细胞增多的Rosai-Dorfman病1例，手术治疗，切除病变组织，分析其病理学特点。结果　脑膜局限性增厚、纤维化，肿瘤组织呈“明暗相间”的病理特点，较多淋巴细胞及多量浆细胞浸润，局部淋巴滤泡形成，并见组织细胞吞噬完整的淋巴细胞及浆细胞（即淋巴细胞伸入现象），未见席纹状纤维化、闭塞性静脉炎及嗜酸性粒细胞浸润。肿瘤组织边缘呈推挤性生长，压迫周边脑组织，周边脑组织血管周围见淋巴细胞及组织细胞套袖。应用免疫组化染色显示：组织细胞CD68及S-100阳性；IgG4阳性浆细胞数量为30个/HPF，IgG4阳性浆细胞/ IgG阳性浆细胞为60%。MRI示右侧额部异常信号影，呈宽基底与邻近脑膜相连，可见硬膜尾征，邻近脑实质呈受压推挤状态，周围可见少量脑水肿影，病变范围14 mm×23 mm×16 mm，T1WI呈低信号，其内混杂高信号，T2WI呈中高信号，其内混杂少许低信号，增强扫描呈均匀强化。结论　脑膜的Rosai-Dorfman病是少见病变，需要与多种疾病鉴别，其诊断具有挑战性，并且此例伴随IgG4阳性浆细胞增多及IgG4阳性浆细胞/ IgG阳性浆细胞为60%，具有IgG4相关性疾病的一个组织学特征（即致密淋巴细胞及浆细胞浸润），需要结合组织病理、临床及血清学特点综合判断，笔者认为诊断为脑膜Rosai-Dorfman病可能伴有IgG4相关性疾病较为恰当。

Abstract:

Objective To investigate the clinical pathological features of meningeal Rosai-Dorfman disease. Methods We chose a case of meningeal Rosai-Dorfman's disease with increased number of IgG4 positive plasma cells from the second hospital of Dalian Medical University. The histological features and immunohistochemically staining features were reviewed. Results Meningeal locally thickening, fibrosis, tumor tissue was characterized of "dark and light" appearance and a large number of lymphocytes and plasma cells infiltrated. Local lymph follicle formation and histocytes phagocytosis of intact lymphocytes and plasma cells (i.e. lymphocytes emperipolesis) were observed. Tumor tissue showed a push-growth edge, oppressing peripheral brain tissue. Lymphocytes and histocytes conformed sleeves around the blood vessel in peripheral brain tissue. Immunohistochemically staining showed that histocytes were CD68 and S-100 positive, and the number of IgG4 positive plasma cells was 30 /HPF, the ratio of IgG4 positive plasma cells to IgG positive plasma cells were 60%, MRI showed the abnormal signal appeared in the right frontal region, which was connected with the adjacent meningeal (i.e. the dural tail sign).Conclusion Meningeal Rosai-dorfman disease is a rare lesion and the diagnosis is challenging. This case with the increased number of IgG4 positive plasma cell and the histological characteristic of IgG4-related disease. We think that it is better to be diagnosed with meningeal Rosai-dorfman disease probably with IgG4-related disease.