胚胎发育不良性神经上皮肿瘤3例报道及文献复习

目的 探讨胚胎发育不良性神经上皮肿瘤（DNT）的临床特征、影像学特征、病理特点、治疗及预后。方法 回顾性分析手术治疗的3例DNT病人的临床资料,并结合文献复习。结果 3例均以癫痫发作起病。手术均全切肿瘤及致痫灶,未留有神经功能障碍。术后随访6个月,均无癫痫发作。结论 DNT属于良性肿瘤,手术全切除预后良好,术后需要密切影像学随访     

18例罕见胚胎发育不良性神经上皮肿瘤的治疗

目的探讨胚胎发育不良性神经上皮肿瘤（dysembryoplastic neuroepithelial tumor，DNT）的临床治疗特点。方法对2001年至2007年治疗18例DNT病人的临床症状，影像学、电生理和病理资料进行回顾性分析。结果18例DNT病人表现癫痫发作，发作形式与部位有关，14例行脑电图描记，其中10例行术中皮层脑电图描记切除病灶，术后随访1月～6年，3例仍有癫痫发作，15例癫痫发作消失，无肿瘤复发。结论DNT属良性肿瘤，手术效果良好，行术中脑电图描记可有效切除癫痫灶。     

胚胎发育不良性神经上皮肿瘤临床及病理分析

目的探讨胚胎发育不良性神经上皮肿瘤（DNT）的临床及病理特点.方法对近年来收治的5例DNT的临床症状和体征、影像学、电生理及病理学资料进行了回顾性分析.结果DNT典型的临床表现为难治性癫痫,绝大多数于20岁以前发病,男性多于女性.病变多位于幕上,影像学检查与其他低级别胶质瘤难以鉴别.5例均于全麻下行开颅肿瘤全切除术,术后癫痫发作消失,病理均证实为DNT.随访3个月-5年,无肿瘤复发.结论DNT是手术可治愈的良性病变,预后良好,无需放疗和化疗.     

胚胎发育不良性神经上皮肿瘤的诊断与治疗

目的探讨胚胎发育不良性神经上皮肿瘤（DNT）的临床特点及治疗方法。方法回顾性分析2008年2月至2012年10月经病理学证实的11例DNT病人的临床资料。11例病变均位于幕上,均行手术治疗。术后均服用丙戊酸钠6个月至1年,未行放、化疗。结果 11例DNT病人中10例以癫痫起病。11例术后随访1~5.5年;10例癫痫患者中,8例癫痫发作消失,2例减少。结论 DNT是一种良性病变,确诊需病理学检查,临床及影像学特征可辅助诊断;该病手术效果好,术后不需放疗及化疗。     

胚胎发育不良性神经上皮肿瘤的手术治疗

目的探讨胚胎发育不良性神经上皮肿瘤（DNT）的手术方法及其疗效。方法 2007年6月至2013年6月显微手术治疗DNT患者17例,手术方式采用在皮层脑电图指引下行肿瘤加致痫灶扩大切除术。术后至少随访1年,根据Engel预后分级评估手术效果。结果所有患者肿瘤均得到全切,病理学均证实为DNT,其中10例合并局灶性脑皮质发育不良（FCD）,术后未出现永久性功能障碍。患者术后随访1~4.5年,无肿瘤复发,17例中无癫痫发作（EngelⅠ级）的13例（76.5%）,仍有少量癫痫发作（EngelⅡ级）4例（23.5%）。结论 DNT是一种少见的以癫痫为主要临床表现的良性肿瘤,常伴有FCD,多发展为药物难治性癫痫,在皮层脑电图监测下行肿瘤加致痫灶扩大切除可以获得良好的癫痫控制率。     

颞叶与非颞叶胚胎发育不良性神经上皮肿瘤的对比分析

目的探讨颞叶和非颞叶胚胎发育不良性神经上皮肿瘤的不同临床特点并探究影响预后的相关因素。方法收集2010年1月至2019年1月期间就诊于我院的33例确诊为胚胎发育不良性神经上皮肿瘤的患者的临床资料进行回顾性分析。根据肿瘤发生部位将患者分为颞叶组和非颞叶组,对患者的一般资料、影像学特点、病理学特点以及预后进行统计对比分析。结果 5例(15. 15%)患者术后仍有癫痫发作,且均为颞叶组患者。统计学分析提示术后癫痫症状控制不佳的患者起病年龄更大、患病时间更长(P 0. 05)。非颞叶组患者的预后更好(P=0. 018)。结论病灶部位、起病年龄以及患病时间与胚胎发育不良性神经上皮肿瘤患者的预后密切相关。     

胚胎发育不良性神经上皮肿瘤

病例资料 病史 患学男性,11岁,癫瘌病史7年,药物控制不佳而就诊。     

胚胎发育不良性神经上皮肿瘤的临床诊断与治疗

目的探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床特征及治疗方法,以提高临床诊治水平。方法对2006年至2009年经手术及病理证实的11例DNT病人的临床症状、影像学特点和病理学表现进行回顾性分析。结果 11例DNT病人典型临床症状均为癫痫发作,病变均位于幕上,均行显微手术治疗。术后随访1月~2年,9例癫痫发作消失,2例仍有癫痫发作;1例肿瘤复发。结论 DNT是一种良性病变,最终需病理诊断定性,氢质子磁共振波谱检查有辅助其诊断的作用。该病显微手术治疗效果良好,术后不需放疗及化疗。     

小脑蚓部胚胎发育不良性神经上皮肿瘤1例

1 病历摘要（图1） 男,7岁。因头痛伴间断恶心、呕吐4个月,加重3d入院。体格检查：轻度颈强,水平眼震,指鼻笨拙,跟膝胫反应（＋）,闭目难立征（＋）。头颅MRI示：小脑蚓部占位性病变,第四脑室及导水管受压,幕上脑积水；病灶T1像呈低信号，T1呈高信号，增强后病变不均匀强化。[第一段]     

胚胎发育不良性神经上皮肿瘤相关癫痫的研究进展

胚胎发育不良性神经上皮肿瘤(dysembryoplastic neuroepithelial tumors,DNET)是中枢神经系统少见的神经元和神经胶质混合性肿瘤,属于良性肿瘤,具有特定影像学及病理学表现.DNET病人大多为药物难治性癫痫,治疗方式首选手术治疗,术后癫痫控制效果好.本文从DNET临床表现、影像学表现、...     

Dysembryoplastic neuroepithelial tumor: report of a case without typical glioneuronal elements

We studied a temporal lobe lesion found in a 44-year-old woman with a 25-year history of intractable complex partial seizures. Histologically, the lesion contained several nodular areas differing in cellular composition within the disarranged uncal cortex; in each area, neuronal and small round cells, mature ganglion cells and fibrillary astrocytic cells predominated. Ultrastructurally, the small round cells demonstrated neuronal, astrocytic or oligodendrocytic features. The astrocytic cell processes were occasionally covered by basal laminae. The mature ganglion cells had well-developed rough endoplasmic reticulum, many mitochondria and lipofuscin granules in their cytoplasm. No dense-cored vesicles were evident. We diagnosed this temporal lobe lesion as a dysembryoplastic neuroepithelial tumor (DNT), although no areas of typical glioneuronal elements with mucinous matrix were evident. This case suggests that a glioneuronal element is not always a constant feature of DNT, and that although mature ganglion cell nodules may be present, they may differ from those of gangliocytomas and gangliogliomas in lacking intracytoplasmic dense-cored vesicles. The presence of occasional astrocytic cell processes covered by basal laminae strongly suggests that some of the constituent astrocytic cells were of subpial astrocytic origin.     

Dysembryoplastic neuroepithelial tumors in two children with neurofibromatosis type 1

Dysembryoplastic neuroepithelial tumors (DNT) occur mainly in children and are always clinically associated with intractable complex partial seizures. In the first report, which included 39 cases, the patients had no neurological deficit and no stigmata of phacomatosis. In contrast, we observed a DNT in 2 children with a neurofibromatosis type 1. The first patient developed intractable complex partial seizures at age 9 years and was operated at the age of 13 years. Neuroimaging study showed multifocal involvement with three separated lesions in the frontal, parietal and temporal lobes. The second patient was a 16-year-old boy with 5-year history of severe and refractory epilepsy. Magnetic resonance imaging identified a right temporal lesion and the patient underwent a right temporal lobectomy. This unusual association of two cases of DNT with neurofibromatosis type 1 raises the question of whether this association is specific or fortuitous.     

Dysembryoplastic neuroepithelial tumor in the insular cortex. Three dimensional magnetoencephalographic localization of epileptic discharges

Abstract

A case of dysembryoplastic neuroepithelial tumor in the left insular cortex manifesting with intractable seizures is presented. The multichannel magnetoencephalography (MEG) localized equivalent current dipoles of epileptic discharges at the cerebral cortex adjacent to the tumor. The total removal of the tumor resulted in complete disappearance of seizures. This case demonstrated that multichannel MEG was useful in the three-dimensional localization of irritative zone in the concealed cortices such as inside the sylvian fissure. [Neural Res 1998; 20: 433–438]     

Dysembryoplastic neuroepithelial tumors in pediatric patients

Objective: Dysembryoplastic neuroepithelial tumors (DNTs) are benign cortical tumors that are frequently associated with the medically intractable focal epilepsy. In this study, the authors delineate the clinical characteristics of DNTs in children and evaluate the role of cortical dysplasia (CD) in the epileptogenicity to find out the optimum surgical strategy. Methods: A retrospective analysis was performed for clinical data of children with DNT, who underwent surgery between 1996 and 2006. The adopted surgical methods were uniform according to the tumor location and included intraoperative electrocorticography (ECoG)-guided resection. The prognostic factors were evaluated for the two prognostic group categorized by the seizure outcome at one year after surgery. Results: Of 22 patients, the overall seizure free rate was 90.9% and the other two patients belonged to Engel class II during the mean follow-up period of 44.1 months. There was no worsening of the seizure after one year of surgery. Associated CD was found in 18 cases (81.8%) and in the 80% (8 of 10 cases) of the additionally resected areas according to the electrophysiologic studies. Conclusions: The CD associated with DNT appears to have its own epileptogenicity. Therefore, complete removal of the CD with tumor itself is important for patient outcome. A thorough surgical approach can be accomplished by comprehensive presurgical evaluations and extensive surgery with the aid of the intraoperative ECoG or intracranial recording.     

胚胎发育不良性神经上皮瘤

目的探讨胚胎发育不良性神经上皮瘤(DNT)的诊断和治疗。方法分析总结我院治疗的4例DNT患者的临床和病理资料。结果所有患者以癫疒间发作为临床表现;MRI上为T1低信号,T2高信号病灶,无水肿,无占位效应;手术治疗后癫疒间发作控制满意;病理可见特异的多发性瘤结节和胶质神经细胞。结论DNT是一种良性病变,手术治疗效果良好,本病的准确诊断对其治疗有重要意义。     

Dysembryoplastic neuroepithelial tumor

Dysembryoplastic neuroepithelial tumor (DNT) is a mixed neuronal-glial tumor associated with a history of partial complex seizures. This uncommon lesion must be disting-uished from other infiltrating gliomas with similar histo-logical features. DNT frequently affects the temporal and frontal lobes of adolescents and young adults. On neuroimaging, the tumor is well demarcated and located in the superficial layers of the cortex. The presence of intracortical nodules is a positive sign indicating DNT. DNT can be divided into complex and simple forms. Complex forms comprise specific glioneuronal elements, glial nodules and/or cortical dysplasia while the simple forms may be composed of glioneuronal elements only. Glioneuronal elements consist of oligodendroglial-like cells which often exhibit alveolar and microcystic patterns. In addition, mature neurons of varying size may be present within the mucinous matrix. Surgical resection is usually sufficient for the treatment of DNT. The heterogeneous histology of DNT supports the hypothesis that it is derived from cells in the subpial granular layer.     

胚胎发育不良性神经上皮瘤诊治体会

目的 探讨胚胎发育不良性神经上皮瘤（DNT）的诊断及治疗方法。方法 回顾性分析4例DNT病人的临床资料。结果 4例病人年龄13～20岁，平均17岁，以部分癫痫发作为主要临床表现，CT表现为低密度，强化不明显，MRI示T1低信号，T2高信号。瘤周无水肿，占位效应不明显，强化不明显，4例均行肿瘤全切术，随访10-24个月，癫痫未再发作，肿瘤无复发。结论 DNT是一种良性病变，其病史特点和影像学资料对术前诊断有帮助。手术切除肿瘤疗效良好。     

胚胎发育不良性神经上皮肿瘤致癫痫的手术治疗

目的 探讨胚胎发育不良性神经上皮肿瘤(DNT)致癫痫患者的手术策略.方法 回顾性分析自2008年1月至2013年8月首都医科大学宣武医院神经外科手术治疗14例DNT患者的经验,术前常规通过MRI及视频脑电图(VEEG)等检查对肿瘤及致痫灶进行评估,术中行皮层脑电监测(EcoG)对致痫灶定位,手术方式:肿瘤病灶加致痫灶切除.结果 14例患者肿瘤均得到全切,手术后病理证实为DNT,未行放疗和化疗,无肿瘤复发及恶性转化.10例患者术后癫痫发作得到完全控制(Engel Ⅰ级),4例患者术后稀少发作(EngelⅡ级).结论 DNT是低级别神经元～胶质细胞肿瘤,常见于青少年,多伴药物难治性癫痫,除切除肿瘤外,积极处理肿瘤外致痫灶可以很好地控制癫痫.     

胚胎发育不良性神经上皮肿瘤临床病理分析(附5例报告)

目的探讨胚胎发育不良性神经上皮肿瘤(DNT)的临床病理特征、影像学特点、治疗及预后。方法应用光镜及免疫组织化学染色对5例DNT进行观察分析。结果主要临床症状为顽固性癫痫发作,神经系统检查无阳性体征。MRI病变呈囊实性改变,无强化、无瘤周水肿及占位效应。肿瘤组织形态类似,由神经胶质-神经元成分构成,周围有少突胶质细胞样细胞(OLC),其组织学特点是瘤组织黏液变明显,多见网状微囊变,单个神经元漂浮在微囊的黏液样基质中。免疫组织化学染色示神经元及部分OLC突触素、神经核抗原(NeuN)及S-100阳性,OLC胶质纤维酸性蛋白染色阴性,Ki-67低增殖活性。4例随访2~7年,术后均无复发。结论 DNT属良性肿瘤(WHOⅠ级),手术切除即可治愈。结合临床表现、影像学及病理形态学和免疫组织化学结果DNT才确诊。