原发性脑膜Rosai-Dorfman 病的临床诊断

目的 探讨颅内Rosai-Dorfman disease(RDD)的临床特点、病理诊断和鉴别诊断.方法 对1 例颅内RDD进行组织形态学和免疫组织化学分析,并复习相关文献.结果 病变以硬膜为基础,与脑实质分界清楚,可见较多组织细胞,淋巴细胞和浆细胞浸润伴纤维化,其中巨大组织细胞胞浆内可见多少不等的淋巴细胞,免疫组化显示,这些组织细胞S-100 蛋白和CD68 标记阳性,散在分布.结论 颅内RDD非常罕见,病理改变为在炎性细胞背景中散在胞浆含多个淋巴细胞的巨大组织细胞,以S-100 阳性,CD68 阳性,CD1a 阴性为特征.

Clinical diagnosis of primary intracranial Rosai-Dorfman disease

Objective To investigate the clinical features, pathological diagnosis and differential diagnosis of primary intracranial Rosai-Dorfman disease （RDD）. Methods One case of primary intracranial RDD was evaluated by analyzing pathologic morphology and immunostaining, along with literature review. Results The lesion was well circumscribed, composed of large histocytes in a plasma-cell-rich ground. These histocytes had an abundant cytoplasm containing some lymphocytes, and were stained by s-100 protein and CD68. Conclusions Primary intracranial RDD is very rare. Other similar lesion should be excluded before coming to this conclusion by morphological and immunohistochemical analysis.