多形性黄色瘤型星形细胞瘤与癫痫1例报告并文献复习
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摘要
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.
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.
引文
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[2]曹杨,冯恩山,李培亮,等.多形性黄色瘤型星形细胞瘤一例并文献复习[J].中华神经外科杂志,2014,30:1218.
[3]冯一鸣,汪晶,柯杨,等.多形性黄色瘤型星形细胞瘤13例报道并文献复习[J].华中科技大学学报:医学版,2013,42:74.
[4]邵立伟,王辅林.间变型多形性黄色瘤型星形细胞瘤[J].中国现代神经疾病杂志,2017,17:616.
[5]Chappe CL,Scavarda D.Dysembryoplastic neuroepithelial tumors share with pleomorphic xanthoastrocytomas and gangliogliomas BRAF(V600E)mutation and expression[J].Brain Pathol,2013,23:574.
[6]Komri T.The 2016 WHO classification of tumours of the central nervous system:the maior points of revision[J].Neurol Med Chir(Tokyo),2017,57:301.
[7]Schaller B.Usefulness of positron emission tomography in diagnosis and treatment follw-up of brain tumors[J].Neurobiol Dis,2004,15:437.
[8]Reifenberger G,Kaulich K,Wiestler OD,et al.Expression of the CD34 antigen in pleomorphic xanthoastrocytomas[J].Acta Neuropathol,2003,105:358.
[9]李云林,王晓飞,张景,等.影响癫痫起始区和脑结构异常区关系的病理学因素探讨[J].中华神经外科杂志,2014,30:9.
[10]Ruda R,Bello L,Duffau H,et al.Seizures in low-grade gliomas:natural history,pathogenesis,and outcome after treatments[J].Neuro Oncol,2012,14:55.
[11]李云林,王晓飞,张景,等.中央区癫痫的唤醒手术及疗效观察[J].临床神经外科杂志,2013,10:323.
[12]Beaumont A,Whittle IR.The pathogenesis of tumour epilepsy[J].Acta Neurochir(Wien),2000,142:1.
[13]Zaatreh MM,Firlik KS,Spencer DD,et al.Temporal lobe tumoral epilepsy:characteristics and predictors of surgical outcome[J].Neurology,2003,61:636.
[14]Barba C,Coras R,Giordano F,et al.Intrinsic epileptogenicity of gangliogliomas May be Independent from co-occurring focal cortical dysplasia[J].Epilepsy Res,2011,97:208.
[15]Ida CM,Rodriguez FJ,Burger PC,et al.Pleomorphic xanthoastrocytoma:natural history and long-term follow-up[J].Brain Pathol,2015,25:575.