CAJ | 학술논문

目的总结原发于中枢神经系统中的Rosai-Dorfman病的临床特点,探讨其诊治方法及预后.方法结合文献回顾分析2010 ～ 2012年收治的3例原发于中枢神经系统中的Rosai-Dorfman病例临床特点、影像、病理学特点、治疗方案及预后.结果 1例患者颅内多发,最大病灶予以全切,其余分阶段予以类固醇及放射治疗,回访1年示手术病灶无复发,余病灶无明显改变;1例颅底多发且呈匍匐样生长,最大病灶部分切除,余病灶随访观察1年略增大;1例颅内单发,完整切除后回访2年无复发.结论原发于中枢神经系统中的Rosai-Dorfman病罕见,影像与脑膜瘤相似;病理特点是淋巴细胞和浆细胞伸入到体积较大胞浆丰富的组织细胞中;手术治疗为首选方案,类固醇及放射治疗疗效不确定.
목적 총결원발우중추신경계통중적Rosai-Dorfman병적림상특점,탐토기진치방법급예후.방법 결합문헌회고분석2010 ～ 2012년수치적3례원발우중추신경계통중적Rosai-Dorfman병례림상특점、영상、병이학특점、치료방안급예후.결과 1례환자로내다발,최대병조여이전절,기여분계단여이류고순급방사치료,회방1년시수술병조무복발,여병조무명현개변;1례로저다발차정포복양생장,최대병조부분절제,여병조수방관찰1년략증대;1례로내단발,완정절제후회방2년무복발.결론 원발우중추신경계통중적Rosai-Dorfman병한견,영상여뇌막류상사;병리특점시림파세포화장세포신입도체적교대포장봉부적조직세포중;수술치료위수선방안,류고순급방사치료료효불학정.
Objective To summarize the clinical characteristics of Rosai-Dorfran disease primarily involved in central nervous system and to explore the diagnosis and treatment.Methods Retrospectively evaluated the clinical,imaging and pathological characteristics as well as treatment and prognosis of three cases of Rosai-Dorfman disease primarily involved in central nervous system and reviewed the literature simultaneously.Results In case one,the largest lesion of multiple intracranial lesions was totally resected,followed by steroid administration and radiotherapy for rest lesions.with no regrowth at 1 year follow up.tIn case 2,multiple intracranial lesions lived in sheetgrowth,and the largest lesion was subtotally resected and the residual showed small growthat 1-year following up.In case 3,the isolate lesion received total resection and didn't recur during the 2-years followup.Conclusions Rosai-Dorfman disease primarily involved in central nervous system is rare,with similar imaging characteristics to those of meningiomas,and the pathological features indicated as lymphocytes and plasma cells reaching tissue cells with large volume and abundant cytoplasm.Operation is the frontlinetreatment,while the effects by steroid administration and radiotherapy are not reliable.