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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, imaging and pathological features of embryonic dysplastic neuroepithelioma (DNT). Methods The clinical and imaging data of 11 patients with DNT were retrospectively analyzed. The pathological changes of the resected specimens were observed and the patients were followed up for a long time. Results Clinical refractory seizures as the main performance, 11 cases of 9 cases before the age of 20 onset. Magnetic resonance imaging (MRI) showed cortical or subcortical low T1, high T2 signal shadow, the boundary is clear, cystic or brain-like. Seven cases showed enhancement. Six cases underwent intraoperative cortical electroencephalography (ECOG) examination and found that after resection of the tumor in 5 cases, there were still some discharges. Histology consisted of a mixture of neuronal and glial heterologous components, one simple, seven complicated and three non-specific. Seven of eight patients with adequate specimens had cortical dysplasia. Epilepsy control results Ⅰ grade in 9 cases, Ⅱ grade in 2 cases, no tumor recurrence. Conclusion DNT is a grade I benign tumor of the WHO. DNT should be diagnosed in combination with clinical, imaging and pathological features. Accurate diagnosis has important therapeutic and prognostic value.