摘要
目的研究胚胎发育不良性神经上皮瘤(DNT)的临床、影像及病理学特征。方法回顾分析11例DNT患者的临床和影像学资料,对术中切除标本进行病理形态学观察,并对患者进行长期随访。结果临床以难治性癫痫发作为主要表现,11例中有9例在20岁之前发病。磁共振成像(MRI)示皮质或皮质下低T1、高T2信号影,边界清晰,呈囊状或脑回样。7例显示强化。6例行术中皮质脑电图(ECOG)检查,发现5例瘤灶切除后仍有部分痼样放电存在。组织学由神经元和神经胶质异源性成分混合构成,1例为简单型,7例为复杂型,3例为非特异型。8例标本充足的病例有7例伴皮质发育不良改变。癫痫控制结果Ⅰ级9例,Ⅱ级2例,无肿瘤复发。结论DNT为WHOⅠ级的良性肿瘤,应结合临床、影像和病理学特征确诊DNT,准确的诊断有重要的治疗和预后判断价值。
Objective To study the clinical, radiological and pathological characteristics of dysembryoplastic neuroepithelial tumor （DNT）. Methods Neurology, neuroimaging, neurosurgery and pathology databases of 11 patients with DNT were retrospectively analyzed, and long-term follow-up was made. Results Patients presented with drug-resistant partial seizures. Nine of them began before 20 years old. On MRI, all lesions located in cortical and subcortical white matter and showed a hypointense mass in T1-weight images, hyperintense in T2-weighted with well delineated boundary. Most lesions were of multicystic appearance. 7 of 9 cases showed contrast enhancement. On intraoperative electrocorticography 5 of 6 cases still had some epileptiform discharge after tumor resection. Histopathologically, the lesions were consisted of neuronal and glial heterogeneity, with 1 case of simple form, 7 cases of complex form and 3 cases of non-specific form. Variable degree of cortical dysplasia was found in 7 out of 8 specimens with sufficient tissue available. Long-term follow-up revealed that 9 cases had class Ⅰpostoperative seizure and 2 cases class Ⅱ. Conclusions DNT is a benign tumor （WHO Ⅰ）. The correct diagnosis requires thorough understanding of the clinical, radiological and pathological features. The diagnosis of DNT has great prognostic and therapeutic value.
出处
《中华神经科杂志》
CAS
CSCD
北大核心
2006年第8期525-528,共4页
Chinese Journal of Neurology
关键词
癫(癎)
神经外胚瘤
原始
中枢神经系统肿瘤
大脑皮质
Epilepsy
Neuroectodermal tumors, primitive
Central nervous system neoplasms
Central cortex
