中枢神经细胞瘤

一、资料与方法1.一般资料:本组2例,均为女性,年龄26-36岁,病程为4-18个月。2.临床表现:2例均表现为头痛、恶心及呕吐,伴视乳头水肿。1例双下肢肌力4级,肌张力正常,双侧巴彬斯基征阳性,另1例未发现神经系统阳性体征。     

中枢神经细胞瘤

中枢神经细胞瘤（CNC）是一种较少见的中枢神经系统肿瘤，1982年首次由Hasson等报道，1993年WHO中枢神经系统组织学分类中正式将其列为起源于神经元的肿瘤。病因尚不清楚，其主要特征为：（1）肿瘤常位于侧脑室；（2）多见于青壮年（20-40岁）；（3）具有特征性的影像学表现；（4）光镜下类似少枝胶质瘤或室管膜瘤；（5）免疫组化及电镜检查发现肿瘤起源于神经元；（6）肿瘤为良性病变，预后良好。迄今为止，国内外文献报道约350余例，其确切发病率尚无定论，估计占中枢神经系统肿瘤的0．1％～1％，但发病率可能高于统计数字，其原因主要是对该肿瘤认识缺乏，多误诊为其他肿瘤所致。发病无性别差异。     

中枢神经细胞瘤

中枢神经细胞瘤（CNC）是临床较为少见的中枢神经系统肿瘤,1982年,Hassoum等首次通过电子显微镜对2例脑室内肿瘤的组织学形态进行观察。其类似于少突胶质细胞瘤,肿瘤细胞缺乏非典型有丝分裂。无“Homer-Wright玫瑰花形”形成和向成熟神经节细胞演变表现,来源于神经细胞,故命名为中枢神经细胞瘤。     

中枢神经细胞瘤

中枢神经细胞瘤是近年来通过电镜和免疫组织化学检查识别出来的一种少见的肿瘤,其组织病理学特征为肿瘤细胞是由具有神经元分化特征的单一细胞群组成,肿瘤大多数为良性。最佳治疗方法是手术全切除或次全切除加放射治疗,多数愈后良好。本文通过复习文献介绍其病理及临床特点。     

中枢神经细胞瘤研究进展

中枢神经细胞瘤是一种少见的神经元来源的脑室内肿瘤，大多呈良性，预后良好。其组织学病理特征为肿瘤细胞来源相同、体积小、大小一致、排列紧密。磁共振波谱特征包括胆碱增高、肌酸和N-乙酰天门冬酸盐减少。最佳的治疗方法是手术治疗，术后辅助放疗、化疗可以提高局部控制率或患者的生存率以及降低复发率。MIB-1标记指数在提示预后上价值较大。本文就近年来其影像、病理和临床等方面的研究进展做一综述。     

中枢神经细胞瘤诊疗新进展

中枢神经细胞瘤好发于青中年,一般以慢性颅内高压症状表现为主,有特征性影像学表现,病理学上由来源相同大小均匀的密集小细胞构成,细胞质界限非常明显,肿瘤细胞核周围细胞质缺乏,形成核周空晕表现,呈煎蛋样外观。治疗以手术切除为主,辅助放疗及化疗可提高疗效。本文就中枢神经细胞瘤的流行病学、临床表现、影像学表现、病理学表现及治疗等新进展作一综述。     

中枢神经细胞瘤临床分析

目的分析颅内中枢神经细胞瘤（CNT）的临床表现特点及诊断治疗。方法回顾性分析经病理证实的28例CNT病人的临床表现、影像学特征、病理特点和治疗方式,并进行随访。结果肿瘤全切除20例,大部分切除8例。术后3个月GOS评分5分12例,4分12例,3分2例,1分2例。术后行标准放疗24例,随访12～120个月,平均38个月,GOS评分均达4-5分,无肿瘤进展和复发。结论CNT好发于侧脑室室间孔附近,具有较特征性的影像学表现,病理特征显著,手术切除加术后放疗为最佳治疗方式,总体预后较好。     

中枢神经细胞瘤相关研究进展

中枢神经细胞瘤(CNC)是位于脑室内的一种罕见的神经上皮肿瘤,目前起源尚不清楚,好发于中青年群体,其在影像学上有一定的特征性表现。但由于中枢神经细胞瘤的罕见性,目前临床上对其的认识仍然存在不足,治疗方式的选择及其预后相关的影响因素也存在着较大的争议。本文结合最近的文献报道就中枢神经细胞瘤临床及流行病学特征、影像及病理学特点、治疗方式的选择及其相关预后因素、与脑室外神经细胞瘤的关系等方面的研究进展作一综述。     

中枢神经细胞瘤的临床特征

目的 探讨中枢神经细胞瘤的临床特征,以提高对该肿瘤的认识.方法 收集本科室1995年1月至2006年12月73例中枢神经细胞瘤的临床资料并进行回顾性分析.结果 发病率约占颅内肿瘤的0.3%.男:女=1.28:1,平均发病年龄31.9岁.临床表现:颅内压增高症状53例,头昏头晕18例,肢体乏力15例,视力改变9例.肿瘤主要来源于透明隔50例.肿瘤误诊率64.4%.手术治疗中,肿瘤全切率80.8%,死亡率8.2%.随访到36例,时间6-105个月,无一例肿瘤复发或再长,术后行放疗19例.结论 中枢神经细胞瘤多见于年轻人,男性多见,临床表现主要为颅内压增高的症状.肿瘤大多来源于透明隔.本病误诊率高.手术全切是治疗的最佳选择,但手术死亡率高.全切后无需放疗.肿瘤预后良好.     

中枢神经细胞瘤一例报告

患，女，45岁。头痛，头晕2d，查体发现右侧脑室占位2d,于2002年10月入院。体格检查：体温37.1℃，脉搏82次/min，血压140/110mmHg(1mmHg=0.133kPa),既管理方式体健。神经科检查：双瞳孔等大，等圆，对光反射阳性，四肢肌力5级，无恶心、呕吐、双侧Babinski征阴性，指鼻试验阴性。实验室检查未见异常。影像学检查：（1）胞部X线片示心     

中枢神经细胞瘤和脑室外神经细胞瘤

肿瘤由形态一致伴神经元分化的圆形细胞组成,位于侧脑室室间孔区(中枢神经细胞瘤)或脑实质内(脑室外神经细胞瘤),好发于青年人,预后良好.肿瘤具有良性组织学形态,典型结构为小而成熟的肿瘤细胞,呈单一片状排列,其间被神经毡背景分隔(图1a);肿瘤细胞呈簇状排列于血管周围,形成室管膜瘤样"假菊形团"结构(图1b);肿瘤细胞形态较一致,内有圆形或卵圆形细胞核,染色质呈细斑点状,偶见核仁;某些区域以透明细胞为主,类似少突胶质细胞瘤结构(图1c);少见的组织学形态为"Homer-Wright菊形团"结构和神经节细胞.肿瘤细胞弥漫表达突触素(图2).     

Central neurocytoma

Central neurocytomas (CN) are rare intraventricular tumors with prominent neuronal differentiation. CN commonly arise in the lateral ventricles of young adults who predominantly present with raised intracranial pressure. Few studies have described the clinical, pathological, and radiological features of these tumors, and those that have are typically single case reports. Herein, we report ten patients with CN with variable clinical and pathological features and discuss the management of these tumors. Nine tumors occupied the lateral ventricle and only one was located in the sellar region. On MRI, all 10 tumors showed heterogeneous hypo-or iso-intensity on T1-weighted and hyperintensity on T2-weighted MRI. Contrast enhancement varied greatly from very slight to intense. All patients were surgically treated by macroscopic total or subtotal removal. Postoperative radiotherapy was given to six patients (four of whom had undergone subtotal resection and two of whom had undergone total resection). The surgical and histopathological data of these patients were reviewed and analyzed. No recurrences were noted although we were unable to contact two patients for follow-up. A brief review of the literature concerning differential diagnosis and therapeutic aspects of these tumors is also presented.     

Central neurocytoma

Summary The authors report two cases of a rare tumor in adults which were inserted on the fornix and caused a frontal syndrome. By light microscopy, the tumors, highly calcified, were composed of small clear cells forming dense areas in a patchy fibrillary stroma. Electron microscopy revealed a striking neuronal differentiation with numerous synapses. These tumors, for which the term neurocytomas was proposed, were compared with the other CNS neuronal tumors described in the literature.Supported by a grant from the Feglefluc, Marseille, France     

Central neurocytoma: the neuroradiological perspective

Background  Central neurocytoma (CN) represents a rare, relatively recently described primary central nervous system tumor. It ranks among intraventricular tumors due to its predominant location within the lateral brain ventricles. CN occurs mostly in young adults around the 3rd decade of life; almost a fifth of the cases are children under 18 years of age. Objectives  The authors present three cases of patients with histopathologically confirmed CN, emphasizing diagnostic imaging issues. A review of the literature concerning differential diagnosis and clinical and therapeutic aspects is also presented. Conclusion  Literature reports of CN comprise most likely case reports, small cohorts of patients, and meta-analytic studies due to the generally low incidence of this tumor. In the current paper, the authors summarize up-to-date knowledge of this rare disease on the background of their own observations. CN should be included in the list for differential diagnostics of intraventricular brain tumors, especially those located in lateral ventricles.     

Central neurocytoma: three case reports

Central neurocytoma is classically recognized as an intraventricular benign brain tumour. Extraventricular localisation is rarer. We report two intraventricular neurocytoma and another bulbar extraventricular neurocytoma. Histologically, central neurocytoma presents remarkable likeness characteristics to oligodendroglioma, but immunohistochemical study distinguishes this tumour. Imaging appearances (CT, MRI) raise the diagnosis and immunohistochemical study confirm it. The purpose of our work is to assess the value of imaging (CT, MRI) in the diagnosis of central neurocytoma.     

Central neurocytoma: immunohistochemical and ultrastructural study

Summary Eight cases of central neurocytomas were studied by immunohistochemistry and electron microscopy. Seven tumors were located in the lateral ventricles and one in the subependymal region. All but one patient had a favorable postoperative course. The tumors were composed of small uniform cells possessing amitotic round nuclei with frequent perinuclear halos, a few Homer Wright rosettes and no ganglion cells; an appearance resembling that of oligodendroglioma. Immunohistochemical studies disclosed neuron-specific enolase and Leu-7 positivity in all tumors, S-100 protein-positive cells were found in six, while glial fibrillary acidic protein —and vimentin-positive cells were confined to the blood vessels. Myelin basic protein as well as neurofilament were not detected in the tumors. Synaptophysin-positive areas were seen in one tumor. Ultrastructural examination showed distinctive neuronal tumor cells which had a cytoplasm with sparse dense-core vesicles and thin cell processes containing parallel microtubules. They were classified into three different types of tumor cells according to the extent of differentiation. The most consistent finding for histological diagnosis was the presence of typical or abortive synapses with clear and dense-core vesicles. Additionally, synaptophysin may be a specific marker for some central neurocytomas.     

Central neurocytoma: report of two cases

INTRODUCTION: Central neurocytomas are rare neuroectodermal tumors believed to arise from the subependymal matrix of the lateral ventricles. CASE REPORTS: A 26-year-old woman and a 33-year-old man each had a large, heterogeneous, contrast enhancing mass in the lateral ventricles at the foramen of Monro causing bilateral hydrocephalus. The woman died after surgery, but the man is asymptomatic after three years. HISTOPATHOLOGY: Both tumors were composed of isomorphic rounded cells positive for synaptophysin, chromogranin and NSE, while some reacted for GFAP, vimentin and S-100 protein. Electron microscopy revealed neuropil-like tissue between cells, but synapses were rare.