中枢神经系统Rosai-Dorfman病1例并文献复习

Central nervous system Rosai-Dorfman disease(report of one case and review of literature)

目的探讨中枢神经系统Rosai-Dorfman病(窦组织细胞增生伴巨大淋巴结病)的临床特点、影像学表现、病理诊断及治疗方法。方法回顾分析1例中枢神经系统Rosai-Dorfman病患者的临床资料,并复习相关文献。结果本例患者男,57岁,临床表现为间断性头晕4个月;MRI检查示,右侧颞部结节信号影,增强扫描病灶强化明显,基底宽与颅底骨板相连,可见"脑膜尾征"。病灶切除手术中见病灶位于右侧中颅窝底,呈匍匐状,质地坚韧累及颅底硬脑膜;手术全切病灶和受累硬脑膜。术后病理检查诊断为Rosai-Dorfman病。术后随访3个月未见病灶复发。结论中枢神经系统Rosai-Dorfman病较为罕见,影像学检查误诊率高,病理检查具有重要诊断作用,手术切除病灶可取得良好的疗效。

Objective To investigate the clinical features,imaging findings,pathological diagnosis and treatment of Rosai Dorfman disease of the central nervous system.Methods The clinical data of a patient with Rosai Dorfman disease of central nervous system were analyzed retrospectively,and the related literature was reviewed.Results A 57 year old male presented with intermittent dizziness for 4 months.MRI examination showed that a nodule signal of the right temporal,enhanced significantly,basal width and skull base bone plate connected,visible“meningeal tail sign”.During the operation,the lesion was located at the base of the right middle cranial fossa,which was creeping,tough and involved the dura mater of the skull base.The lesion and dura were removed.Rosai Dorfman disease was diagnosed by pathological examination.No recurrence was found in the follow-up of 3 months after operation.Conclusions Rosai Dorfman disease of the central nervous system is rare,and the misdiagnosis rate of imaging examination is high.Pathological examination plays an important role in the diagnosis of Rosai Dorfman disease.