Hu Zhen,Chen Wei,Xie Yanping,et al.Clinical characteristics and surgical treatment strategy of pediatric disembryoplastic neuroepithelial tumor[J].Journal of Clinical Pediatric Surgery,2021,20(05):415-419.[doi:10.12260/lcxewkzz.2021.05.004]
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
- Title:
- Clinical characteristics and surgical treatment strategy of pediatric disembryoplastic neuroepithelial tumor
- 关键词:
- 脑肿瘤; 神经上皮肿瘤/外科学; 癫痫/外科学; 治疗结果; 儿童
- Keywords:
- Brain Neoplasms; Disembryoplastic Neuroepithelial Tumor/SU; Epilepsy/SU; Treatment Outcome; Child
- 分类号:
- R739.4;R726.1;R742.1
- 摘要:
- 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。
- Abstract:
- 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 were Ⅰ (n=13,92.86%) and Ⅳ (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.
参考文献/References:
1 Blumcke I,Spreafico R,Haaker G,et al.histopathological findings in brain tissue obtained during epilepsy surgery[J].N Engl J Med,2017,377(17):1648-1656.DOI:10.1056/NEJMoa1703784.
2 Luyken C,Blümcke I,Fimmers R,et al.The spectrum of long-term epilepsy-associated tumors:long-term seizure and tumor outcome and neurosurgical aspects[J].Epilepsia,2003,44(6):822-830.DOI:10.1046/j.1528-1157.2003.56102.x.
3 Thom M,Blümcke I,Aronica E.Long-term epilepsy-associated tumors[J].Brain Pathol,2012,22(3):350-379.DOI:10.1111/j.1750-3639.2012.00582.x.
4 Scheffer IE,Berkovic S,Capovilla G,et al.ILAE classification of the epilepsies:Position paper of the ILAE Commission for Classification and Terminology[J].Epilepsia,2017,58(4):512-521.DOI:10.1111/epi.13709.
5 Engel J.Early versus late surgery for intractable seizures[J].Adv Exp Med Biol,2002,497:99-105.DOI:10.1007/978-1-4615-1335-3_11.
6 Daumas-Duport C,Scheithauer BW,Chodkiewicz JP,et al.Dysembryoplastic neuroepithelial tumor:a surgically curable tumor of young patients with intractable partial seizures.Report of thirty-nine cases[J].Neurosurgery,1988,23(5):545-556.DOI:10.1227/00006123-198811000-00002.
7 方庆和,刘世忠,庄楠,等.胚胎发育不良性神经上皮肿瘤附6例病例分析[J].医学影像学杂志,2013,23(5):669-671.DOI:677.10.3969/j.issn.1006-9011.2013.05.005. Fang QH,Liu SZ,Zhuang N,et al.Dysembryoplastic neuroepithelial tumor:a report of 6 cases[J].Journal of Medical Imaging,2013,23(5):669-671.DOI:677.10.3969/j.issn.1006-9011.2013.05.005.
8 Stanescu Cosson R,Varlet P,Beuvon F,et al.Dysembryoplastic neuroepithelial tumors:CT,MR findings and imaging follow-up:a study of 53 cases[J].J Neuroradiol,2001,28(4):230-240.
9 郭强,张伟,朱丹.儿童致痫性低级别肿瘤的手术策略与切除范围设计[J].临床小儿外科杂志,2019,18(9):719-722.DOI:10.3969/j.issn.1671-6353.2019.09.002. Guo Q,Zhang W,Zhu D.Surgical strategies and scope of resecting epileptic low-grade tumors in children[J].J Clin Ped Sur,2019,18(9):719-722.DOI:10.3969/j.issn.1671-6353.2019.09.002.
10 Onishi S,Amatya VJ,Kolakshyapati M,et al.T2-FLAIR mismatch sign in dysembryoplastic neuroepithelial tumor[J].Eur J Radiol,2020,126(5):108924.DOI:10.1016/j.ejrad.2020.108924.
11 李方成,李军亮.小儿脑血管疾病的诊断要点及外科治疗理念[J].临床小儿外科杂志,2019,18(9):713-718.DOI:10.3969/j.issn.1671-6353.2019.09.001. Li FC,Li JL.Key points and concepts in the diagnosis and treatment of cerebrovascular diseases in children[J].J Clin Ped Sur,2019,18(9):713-718.DOI:10.3969/j.issn.1671-6353.2019.09.001.
12 刘雪青,沈艳玲,杨文圣.胚胎发育不良性神经上皮肿瘤4例临床病理观察[J].诊断病理学杂志,2019,26(9):582-586.DOI:10.3969/j.issn.1007-8096.2019.09.008. Liu XQ,Shen YL,Yang WS.Dysembryoplastic neuroepithelial tumor:a clinicopathological analysis of four cases[J].Chinese Journal of Diagnostic Pathology,2019,26(9):582-586.DOI:10.3969/j.issn.1007-8096.2019.09.008.
13 Chassoux F,Landré E,Mellerio C,et al.Dysembryoplastic neuroepithelial tumors:epileptogenicity related to histologic subtypes[J].Clin Neurophysiol,2013,124(6):1068-1078.DOI:10.1016/j.clinph.2012.11.015.
14 Vecht C,Royer-Perron L,Houillier C,et al.Seizures and anticonvulsants in brain tumours:frequency,mechanisms and anti-epileptic management[J].Curr Pharm Des,2017,23(42):6464-6487.DOI:10.2174/1381612823666171027130003.
15 Fallah A,Weil AG,Sur S,et al.Epilepsy surgery related to pediatric brain tumors:Miami Children’s Hospital experience[J].J Neurosurg Pediatr,2015,16(6):675-680.DOI:10.3171/2015.4.PEDS14476.
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备注/Memo
收稿日期:2021-01-03。
基金项目:国家自然科学基金(编号:81703011)
通讯作者:许新科,Email:sjwkxxk@163.com