儿童胚胎发育不良性神经上皮肿瘤的临床特点及外科治疗策略研究

Clinical characteristics and surgical treatment strategy of pediatric disembryoplastic neuroepithelial tumor

目的总结儿童胚胎发育不良性神经上皮肿瘤(disembryoplastic neuroepithelial tumor,DNT)的临床特点,探讨其外科治疗策略。方法以2015年1月至2020年6月在广州市妇女儿童医疗中心神经外科接受手术治疗的14例儿童DNT患者为研究对象,男11例,女3例,年龄4个月至13岁,平均4.64岁。均以癫痫发作起病,其中局灶性发作9例,全面性发作5例。病程1周至3年,平均10.46个月。对14例患者的病程时间、癫痫发作类型、影像学表现、手术方案、肿瘤切除程度、术后病理结果及疗效进行总结分析。结果肿瘤位置:颞叶9例,额叶3例,顶叶和枕叶各1例。患者均接受详细的癫痫外科术前评估及手术治疗。术中均行皮层脑电监测(electroencephalography,ECoG)以协助判断致痫灶范围,其中肿瘤+周围致痫皮层切除术12例,单纯肿瘤切除术1例,肿瘤大部分切除术1例。术后出现颅内感染4例,对侧肢体肌力下降1例,无一例死亡。术后病理结果提示单纯型DNT 5例,复杂型DNT 9例。14例术后均得到有效随访,随访时间7个月至5年,平均2.14年。按Engel分级判断术后疗效,其中EngelⅠ级13例(92.86%),Ⅲ级1例(7.14%)。结论DNT是儿童癫痫常见病因,通常药物治疗效果不佳,外科治疗安全有效,癫痫外科治疗理念及术中ECoG有利于判断致痫灶范围,改善术后癫痫控制效果。

Objective To summarize the clinical features of disembryoplastic neuroepithelial tumor(DNT)and discuss its surgical strategies.Methods From January 2015 to June 2020,a total of 14 DNT children undergoing operations at Department of Neurosurgery,Guangzhou Women&Children s Medical Center were retrospectively analyzed.There were 11 boys and 3 girls with an average age of 4.64(1/3-13)years.Epileptic seizures were focal(n=9)and general(n=5).The average course of disease was 10.46 moths(1 week to 3 years).Gender,age,duration of disease,seizure type,imaging studies,surgical approach,extent of tumor resection,postoperative pathology and efficacy were thoroughly analyzed.Results Imaging showed that tumor involved the lobes of temporal(n=9),frontal(n=3)and parietal(n=1).Detailed preoperative evaluations were performed.Cortical electroencephalography(ECoG)monitoring was performed intraoperatively for assisting in the determination of epileptic foci(n=12),simple tumor resection(n=1)and major tumor resection(n=1).There were postoperative intracranial infection(n=4),lowered strength of contralateral limb muscle and survival(n=14).Postoperative pathological examinations indicated simple DNT(n=5)and complex DNT(n=9).The average follow-up period was 2.14(7/12-5)years.According to the Engel classification scheme of postoperative curative effect,the Engel levels wereⅠ(n=13,92.86%)andⅣ(n=1,7.14%).Conclusion DNT is a common cause of childhood epilepsy and drug treatment is often ineffective.Surgery is both safe and effective.The concept of surgery for epilepsy and intraoperative ECoG are helpful to determine the range of epileptic focus and improve the control outcomes of postoperative epilepsy.