儿童胚胎发育不良性神经上皮肿瘤

目的 探讨儿童胚胎发育不良性神经上皮肿瘤(dysembryo plastic neroepithelial tumor,DNT)的临床特点、诊断及手术治疗.方法 回顾性分析了2003年1月至2008年5月手术治疗的13例儿童胚胎发育不良性神经上皮肿瘤的临床和病理资料.结果 患儿以难治性癫痫为主要临床表现.本组患儿均接受了显微外科手术治疗,其中全切10例,近全切3例.磁共振成像示皮质或皮质下低T1、高T2信号,病灶边界清晰,无水肿及占位效应.肿瘤病理学检查可见特异性胶质神经元成分.随访13例患儿中2例于术后1年内出现癫痫发作,近全切的1例表现为强直性发作,全切的1例为失神性发作,均与术前发作形式相同,但发作频率明显减少(频率减少≥75%),术后随访癫痫发作控制满意.结论 DNT为良性肿瘤,手术治疗效果好.准确诊断对本病的治疗有重要意义.

Dysembryoplastic neuroepithelial tumor in children

Objective To investigate the clinical features,diagnosis and surgical management of dysembryoplastic neuroepithelial tumors(DNT) in children.Methods The clinical presentation,imaging features and pathological characteristics were analyzed retrospectively in 13 cases of dysembryoplastic neuroepithelial tumors in children.Results Patients presented with intractable partial seizures.On MRI,all lesions were located in cortical and subcortical white matter and showed a hypointense mass in T1-weight images,hyperintense in T2-weighted with well delineated boundary.And there were neither edema nor mass effect.Specific glioneuronal element was typical manifestation in pathological examination.All the patients underwent microsurgical operation.Among them total resection was performed in 10 patients,subtotal resection in 3 patients.Epilepsy was well controlled after the operationx except for two patients who developed epileptic seizure one year after operation.One had tonic seizures after total resection and one had absence epilepsy after subtotal resection.These two patients presented with similar clinical picture pre-operatively.The seizure frequency decreased post-operatively (more than 75% declined).Conclusions Dysembryoplastic neuroepithelial tumors is a benign tumor and the microsurgery is curative.The correct diagnosis is important in the treatment of DNT.