多形性黄色瘤型星形细胞瘤与癫痫1例报告并文献复习

Pleomorphic xanthoastrocytoma and epilepsy (a report of one case and review of literature)

目的探讨实质型多形性黄色瘤型星形细胞瘤(PXA)的临床、影像学表现、病理学特点及致痫机制和外科治疗方案。方法回顾性分析1例PXA患儿的临床资料。结果患儿男,10岁,临床表现为反复癫痫发作。脑电图示右颞区为主的慢波、棘-慢波发放,头颅MRI平扫见右颞中部皮质结构异常,增强扫描局部呈不均匀片状强化。术中见病变位于右侧颞中回后部,局部皮质略肿胀、颜色稍黄、质地偏韧、边界欠清,自皮质表面向深部生长。术中皮质脑电图(ECo G)监测见癫痫放电主要位于病变及其周围皮质,扩大切除病变后再次监测未见异常癫痫波发放。术后随访9个月无癫痫发作。结论 PXA是一种少见的中枢神经系统肿瘤,多以癫痫发作为首发症状。在ECo G监测下行病变扩大切除是治疗PXA的有效方法。

Objective To investigate the imaging features,pathological characteristics,epileptogenic mechanism and surgical treatment of pleomorphic xanthoastrocytoma(PXA).Methods The clinical data of 1 patient with PXA were analyzed retrospectively.Results A 10-year-old boy presented with repeated epileptic seizures as his first clinical manifestation,whose EEG monitoring showed abnormal discharges like slow and spike-slow waves mainly in the right temporal lobe regions.MRI scan showed a small lesion located in the right middle and posterior part of temporal lobe with no specific distinct patterns on T 1 and T 2-weighted imaging,and heterogeneous patchy enhancement could be found in the lesion.During the operation,the lesion was found in the posterior part of the middle temporal gyrus,which was slightly swollen,yellowish in color,tough in texture and unclear in border,and grew from the cortical surface to the deep part.Intraoperative electrocorticography(ECoG) monitoring revealed that epileptic discharges were mainly located in and around the lesion.No abnormal epileptic discharges showed after extended resection of the lesion.No seizures occurred at 9 months followed up.Conclusions PXA is a rare central nervous system neoplasm,which can cause seizures frequently.Extended resection of the lesion under ECoG monitoring can effectively treat the lesion and seizure.