胚胎发育不良性神经上皮肿瘤的诊断与治疗  

Diagnosis and treatment of dysembryoplastic neuroepithelial tumor

作　　者：许威[1] 杨天权 韩勇 王杭州[1] 陈民[1] 王勇强[1] Xu Wei;Yang Tianquan;Han Yong;Wang Hangzhou;Chen Min;Wang Yongqiang(Department of Neurosurgery,Affiliated Children's Hospital,Soochow University,Suzhou 215000,China)

机构地区：[1]苏州大学附属儿童医院神经外科,苏州215000

出　　处：《临床小儿外科杂志》2023年第4期383-386,共4页Journal of Clinical Pediatric Surgery

基　　金：苏州市科技发展计划(民生科技)项目(SYSD2018116)

摘　　要：目的结合文献探讨胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumor,DNET)的临床特点及治疗策略。方法回顾性分析苏州大学附属儿童医院神经外科2017年12月至2018年5月收治的5例胚胎发育不良性神经上皮肿瘤患儿临床资料,结合文献复习,总结胚胎发育不良性神经上皮肿瘤的临床表现、影像学特点、分类、治疗方法和预后。结果5例患儿中4例表现为癫痫发作,1例表现为头痛、嗜睡等脑积水症状。术前CT和磁共振检查诊断为胚胎发育不良性神经上皮肿瘤,按影像学特点分类并经手术及病理检查确诊。根据影像学特点,5例中DNETⅠ型1例,Ⅱ型3例,Ⅲ型1例。1例Ⅰ型患儿行单纯额叶病灶切除术;2例Ⅱ型患儿行颞叶病灶+前内侧颞叶切除术;1例Ⅱ型患儿病灶侵犯功能区及基底节区,予病灶大部切除术;1例Ⅲ型患儿病灶位于脑室内,行脑室镜下肿瘤切除术。术后随访8~14个月,5例患儿Engel分级均为Ⅰ级,1例脑积水患儿术后症状改善。结论DNET患儿常表现为癫痫,也可表现为脑积水等症状,临床表现与影像学分型相关,大部分患儿经单纯肿瘤切除或包含肿瘤的脑叶切除可获得良好治疗效果。Objective To explore the clinical characteristics and treatments of dysembryoplastic neuroepithelial tumor(DNET).Methods From December 2017 to May 2018,5 DNET children were reviewed.Etiologies,imaging features,classifications,treatments and prognoses were recorded.Results There were epileptic seizures(n=4)and headache,lethargy and other hydrocephalus symptoms(n=1).Computed tomography(CT)and magnetic resonance imaging(MRI)were performed.Based upon imaging characteristics,they were divided into typeⅠ(n=1),typeⅡ(n=3)and typeⅢ(n=1).Type 1 underwent gyrus resection while type 3 resection of lesion in ventricle through ventriculoscopy.Type 2 underwent resection of temporal lobe lesion plus anterior medial temporal lobe(n=1)and subtotal resection of lesion due to an invasion of functional areas and basal ganglia(n=1).Conclusion Associated with imaging types,DNET is frequently manifested as epilepsy and hydrocephalus.Tumor resection alone or extended excision tumors and epileptogenic lobe often yield decent outcomes.

关 键 词：胚胎发育不良性神经上皮肿瘤 诊断 治疗 治疗结果 

分 类 号：R739.4[医药卫生—肿瘤]