胚胎发育不良性神经上皮肿瘤(二例报告及文献复习)

Dysembryoplastic neuroepithelial tumor(2 cases report and review of literature)

目的探讨胚胎发育不良性神经上皮肿瘤的临床特点、诊断及治疗。方法报告2例表现为癫痫的患儿(男女各1例),介绍其临床特征、神经影像学特点、手术治疗及预后。结果2例均为儿童,男女各1例,癫痫发作5～6年,MRI显示额叶长T1长T2信号,无强化,无占位效应,局部颅骨受压变薄,肿瘤近全切除后效果良好。随诊3～6个月,无复发,无癫痫发作。结论胚胎发育不良性神经上皮肿瘤是手术切除肿瘤可治愈的良性病变,术后不需放疗及化疗。

Objective To discuss the clinical features, diagnosis, treatment and prognosis of dysembryoplastic neuroepithelial tumor (DNT). Methods 2 cases of DNTs were reported. Results One boy and one girl presented with epilepsy for 5-6 years, MR showed a mass located in the frontal lobe with hypointense signal intensity on T1-weighted images and hyperintense signal on T2-weighted images without enhancement and mass effect, combined with deformity of the overlying calvarium. The DNTs were subtotally removed. The patients had been seizures free for 3 to 6 months and without recurrence. Conclusions DNT is a surgically curable benign tumor, radiotherapy and chemotherapy are contraindicated.