脱髓鞘假瘤五例临床分析

Clinical analysis of five cases of demyelinating pseudotumor

目的回顾分析5例经病理学证实的脱髓鞘假瘤患者的临床表现、影像学和病理学特点、治疗及预后。方法与结果共5例经术后病理学证实的脱髓鞘假瘤患者,均为男性,临床主要表现为肢体无力(3例),头晕和记忆力减退(2例),癫发作(1例);头部MRI显示占位效应,呈T_1WI低信号、T_2WI和FLAIR成像高信号,增强扫描病灶多呈强化征象,其中3例为开环状强化;手术全切除(4例)或部分切除(1例)病变;术后病理学提示炎性脱髓鞘改变;1例复发后死亡,4例预后较好。结论脱髓鞘假瘤是表现为占位效应的炎性脱髓鞘病变,应注意与中枢神经系统肿瘤相鉴别,以避免不必要的手术治疗和放射治疗。

Objective To study the clinical manifestations,imaging and pathological features,treatment and prognosis of 5 patients diagnosed as demyelinating pseudotumor（DPT） by pathology.Methods The data of clinical features,radiological and histological examination of 5 patients with DPT were retrospectively analyzed,and relevant literatures were reviewed. Results All patients were male.Main symptoms included limb weakness in 3 cases,dizziness and impaired memory in 2 cases,seizures in one case. Head MRI examination revealed space-occupying lesions with hypointense signal on T_1WI,hyperintense signal on T_2WI and FLAIR. Enhanced MRI showed obvious enhancement of lesions（openring enhancement in 3 cases）. The lesion were totally（4 cases） or partially（one case） removed,and postoperative histological examination showed inflammatory demyelination. One case died after twice relapse,and others had favorable prognosis. Conclusions DPT is an inflammatory demyelination with mass effect. It should be differentiated from central nervous system tumors in order to avoid unnecessary resection or radiotherapy.