中枢神经系统 Rosai-Dorfman 病的 MRI 表现

目的：总结中枢神经系统 Rosai-Dorfman 病(RDD)的临床及影像学特点,增加对该病的系统性认识,提高对该病的影像诊断水平。方法结合文献回顾性分析3例病理确诊的中枢神经系统 RDD 患者的临床表现和影像特点。结果2例发生于颅内,1例发生于颅外。病灶均呈等 T2、等 T1或稍高 T1信号且均可见明显强化。2例单发病灶,1例位于上矢状窦旁,1例位于颈部脊髓,均靠近硬膜。另 1例为多发病灶,位于脑室内。结论基于脑膜或者脊膜生长的独立或多发病灶,特别是形态或分布以中线为轴对称生长与在脑内静脉系统周围的病灶,应考虑到中枢神经系统 RDD 的可能。

MRI features of Rosai-Dorfman disease in CNS

Objective To summarize the clinical and imaging characteristics of primary Rosai-Dorfman disease (RDD)in central nervous system in order to understand well its features and improve the diagnosis.Methods The clinical and imaging features in 3 patients with RDD in CNS proved by pathology were retrospectively analyzed in combination with the related literatures.Results The lesions in 2 patients occurred in brain and other lesion in 1 occurred outside the brain.Iso-or slight hyperintensity on T1 WI and marked enhancement of the lesion were found.Single lesion was found in 2 patients near the dura mater and located near the superior sagittal sinus or cervical spinal cord.Multiple lesions in 1 patient were located in the lateral ventricle.Conclusion It’s suggested that single or multiple lesions in dural or spinal meninges located in the midline or near the intracephalic venous system would be considered as RDD in CNS.