脊索瘤样脑膜瘤的临床与病理特点

目的 探讨脊索瘤样脑膜瘤的临床与病理特点.方法 应用组织病理学、组织化学以及免疫组织化学方法对1例脊索瘤样脑膜瘤患者进行观察,结合文献分析其临床与病理特点.结果 脊索瘤样脑膜瘤在临床上可以伴有或不伴有Castleman综合征.以黏液样基质中有呈分叶状或条索状排列的肿瘤细胞为病理学特点,细胞质呈淡染或嗜酸性染色,部分细胞呈液滴状,分布于黏液样基质中,类似脊索瘤样细胞;肿瘤组织中亦可见由典型脑膜皮细胞型脑膜瘤细胞组成的区域.组织化学方法过碘酸雪夫染色呈阳性反应.免疫组织化学染色显示,肿瘤细胞上皮膜抗原、波形蛋白及表皮生长因子受体表达阳性,细胞角蛋白、S-100蛋白呈灶性阳性反应,癌胚抗原表达阴性,Ki-67抗原标记指数＜1%,35%肿瘤细胞p53蛋白表达阳性.结论 脊索瘤样脑膜瘤为临床罕见的特殊类型脑膜瘤,通过临床病史、组织病理学、组织化学和免疫组织化学检测可以明确诊断.预后尚有待进一步观察.     

三脑室脊索瘤样脑膜瘤1例报告及文献复习

目的：探讨三脑室脊索瘤样脑膜瘤的临床特点。方法：回顾性分析1例三脑室脊索瘤样脑膜瘤患者的临床表现、影像学资料、手术方式和病理检查结果,并结合复习相关文献。结果：患者入院时精神状态较差,白天睡眠次数增多。术前头颅MRI示鞍上三脑室区域团块状异常信号灶,约2.5 cm×2.5 cm×2.12 cm大小,T1W呈等低信号,T2W呈高低混杂信号,增强后见病灶均匀强化明显。考虑：①三脑室脑膜瘤;②淋巴瘤;③生殖细胞瘤。手术经纵裂胼胝体入路作肿瘤部分切除。病理检查为三脑室脊索瘤样脑膜瘤（WHOⅠ～Ⅱ级）。结论：成年人三脑室脊索瘤样脑膜瘤少见,确诊需病理证实,其生物学特征有待更细致和深入的临床研究。     

颅底脊索瘤临床与病理分析

目的研究颅底脊索瘤临床与病理特征.方法 8例颅底脊索瘤作临床及免疫组织化学分析.结果 5例为颅底脊索瘤,3例为软骨样脊索瘤.结论该瘤为低度恶性肿瘤,常局部复发,预后较好.免疫组织化学染色是诊断的重要工具.     

第三脑室脊索样胶质瘤临床病理（附1例报道及文献复习）

第三脑室脊索样胶质瘤(chordoid glioma of the third ven- tricle,CGTV)是新近报道的一种少见的中枢神经系统肿瘤。本文报告1例,结合复习文献对其临床病理学特点、免疫组织化学特征、诊断及鉴别诊断作进一步探讨。     

第三脑室脊索样胶质瘤1例报告

脊索样胶质瘤足一种罕见的脑肿瘤,多位于第三脑室。因为与脑膜瘤相似,最早被认为是脊索样脑膜瘤的一种变异类型：发生于第三脑室、具有独特的神经胶质酸性蛋白（glial fibrillary acidic protein,GFAP）阳性反应的脑膜瘤。随着对该肿瘤免疫表型的进一步认识,     

脊索样脑膜瘤

脊索样脑膜瘤是脑膜瘤的亚型之一,WHOⅡ级,临床少见,发病年龄略早于良性脑膜瘤,无明显男女性别差异,儿童患者可合并Castleman综合征.该病术前诊断困难,需要与良性脑膜瘤、脊索瘤、软骨肉瘤、脊索样胶质瘤相鉴别.脊索样脑膜瘤首选手术治疗,然而手术全切除困难,术后易复发,尤其是非全切除术后、脊索样成分较高和Ki-67指数高的患者,术后需辅助放射治疗及长期随访.     

脊索瘤样脑膜瘤1例报道及文献复习

目的 探讨脊索瘤样脑膜瘤的诊断、治疗。方法 回顾性分析1例右侧颅内蝶骨嵴起源的脊索瘤样脑膜瘤的临床资料,并结合文献进行分析。结果 MRI和CT与一般类型的脑膜瘤一样具有典型脑膜尾征,但是CT密度（低密度）和MRI信号（长T1、长T2信号）特点不同于常见类型。手术全切肿瘤,术后12个月颅脑MRI复查显示肿瘤未复发。结论 脊索瘤样脑膜瘤是具有脊索瘤样肿瘤特点的脑膜瘤,其影像特征及病理基础均不同于一般类型的脑膜瘤,要注意与其他脊索瘤样肿瘤相鉴别。手术全切肿瘤是主要治疗方法,但是易复发,注意长期随访。     

颅内脊索瘤临床与病理研究现状

脊索瘤是一种较为少见的肿瘤,呈缓慢的侵袭性生长,可以发生在颅底斜坡、鞍区,也可发生在脊椎骶尾部。在人类胚胎发育的第四周时即出现脊索结构1,随着胚胎的发育,脊索逐渐退化,但位于颅底蝶枕部及脊椎骶尾部的部分脊索组织不完全退化而残留下来,以往认为该肿瘤来源于胚胎残余。有作者认为这种残留的组织在一定条件的作用下,如刺激和创伤2或激素水平增高,如人-β绒毛膜促性腺激素3.4等因素的影响可能发生肿瘤性增殖而形成脊索瘤。Ribbert于1894年最早提出了脊索瘤的诊断,1903年Grahl首次对颅底脊索瘤进行了描述。本文将颅内脊索瘤的临床特点…     

颅内脊索瘤型脑膜瘤的临床病理特征及预后分析

目的探讨脊索瘤型脑膜瘤的临床、病理特征及预后特点。方法回顾性分析22例行手术切除并经病理证实为脊索瘤型脑膜瘤患者的临床资料,应用相应抗体重新染色标记并进行临床疗效随访。结果 22例患者中,男性9例、女性13例;平均年龄46.5岁。均未见明确有Castleman综合征表现;病变均系单发,多数位于小脑幕上。病理检查示脊索样基质占20%~80%,淋巴细胞浸润以B细胞为主,细胞增殖标记物(MIB-1)标记指数为2%~10%,平均2.5%。19例患者达全切,3例患者达次全切除。所有患者均获随访,平均随访时间44.0个月,4例患者复发;次全切除与复发相关;复发患者MIB-1标记指数与非复发患者比较差异无统计学意义。结论脊索瘤型脑膜瘤是非典型脑膜瘤的一个少见亚型,具有复发风险,女性略多见;患者缺乏明确的系统性疾病表现,小脑幕上是好发部位,手术切除程度与患者预后相关,MIB-1标记指数预测复发的意义尚难肯定。     

Clinical characteristics and treatment outcomes of pigmented tumors in central nervous system: Focusing on melanocytic tumors

Pigmented tumors are rare neoplasm of central nervous system. Melanocytic tumor, including primary and metastatic lesions, is the most common type. Owing to the rarity, the differential diagnosis of pigmented tumors and clinical management of melanocytic tumor remain challenge. Therefore, focusing on melanocytic tumors, the clinical, radiological, histopathological features and treatment outcomes were presented and analyzed in this study. We identified 22 melanocytic tumors, 2 melanotic medulloblastomas, 2 melanotic ependymomas and 1 melanotic schwannoma. Compared with metastatic melanocytic tumors (MMTs), primary melanocytic tumors (PMTs) were characterized by younger age (36.11 ± 17.96 vs. 51.69 ± 12.58 years, p = 0.0262), lower possibility to be multiple lesions (11.1%vs. 61.5%, p = 0.0306), higher proportion of hypointensity on T2-weighted images (66.7% vs. 15.4%, p = 0.0260) and higher frequency in black appearance (77.8% vs. 23.1%, p = 0.0247). During the follow-up, 4 PMTs and 11 MMTs (71.4%) experienced tumor progression. PMTs had better prognosis than MMTs that progression-free survival (PFS) rate of PMT was 50.0% but decreased to 23.1% for MMTs at 12 months (p = 0.0123). Cox proportional hazards regression revealed that multiplicity of tumor was an independent predictor for PFS. None of patient with multiple tumors was in PFS after 12 months’ follow-up whereas PFS rate was 40.5% for single tumor (p = 0.0002). In conclusion, radiological appearances, especially hypointensity on T2-weighted images, might be an indication for PMT. MMTs are more likely to be multiple intraparenchymal masses in elder patients located in supratentorial region. Current treatments included operation, radiotherapy and chemotherapy are not competent to control tumor progression and other therapeutic modalities are urgently needed.     

免疫组化与电镜在诊断神经系统疑难肿瘤中的应用

目的 分析评估免疫组化和电镜技术在疑难神经系统肿瘤病例诊断中的应用。方法 采用免疫组化和电镜观察对4例神经系统肿瘤进行研究，免疫组化应用的抗体包括了神经系统肿瘤诊断中最重要的几种抗体。结果 通过显微镜观察HE染色切片，并结合免疫组化、电镜结果，根据WHO神经系统肿瘤2000年分类，明确了病理诊断，包括中枢神经系统的幕上原始神经外胚叶肿瘤(PNET)、婴幼儿促纤维增生性星形细胞瘤、脊索瘤样脑膜瘤和原发于外周神经的神经纤维瘤。结论 免疫组化和电镜的应用在神经系统肿瘤诊断和鉴别诊断中具有重要的意义。     

原发性中枢神经系统淋巴瘤影像学及病理学特征

目的探讨原发性中枢神经系统淋巴瘤(PCNSL)的临床特征,提高诊断准确率。方法回顾性总结分析自1995年1月至2005年11月经手术、活检、术后病理及免疫组化检查证实的16例PCNSL患者的临床资料,分析其病变部位、影像学表现、组织病理学特征、诊断及治疗。结果单发肿瘤10例,多发肿瘤6例;11例位于幕上,3例位于小脑,1例位于咽旁间隙并累及内听道、桥小脑角,1例位于椎管内,其中位于后颅窝5例,基底节、脑室周围白质和小脑蚓部易受累,具有一定的影像学和病理学特征;全部病例均行手术,见肿瘤多呈肉红色或灰白色,质地较软,血供丰富,边界较清,无包膜。结论PCNSL的影像学和组织病理学有一定特征,有助于提高诊断准确率。     

中枢神经系统脱髓鞘假瘤

CT及MRI技术的普及为疾病的临床诊断提供了更为直观的依据.水肿及占位效应被认为是区分中枢神经系统肿瘤与非肿瘤性病变的可靠依据之一,然而在临床工作中,我们经常还会遇到一类特殊表现的炎性脱髓鞘病,其临床及影像学表现与中枢神经系统原发性肿瘤如胶质瘤、淋巴瘤等十分相似,故常误诊而行手术治疗,给患者带来不必要的损伤.<阿克曼外科病理学>称该类病变为脱髓鞘性假瘤或肿胀性脱髓鞘性病变,现结合文献对其综述如下.     

Neuroendocrine markers in central nervous system neuronal tumors (gangliocytoma and ganglioglioma)

Summary We studied five cases of central nervous system neuronal tumor, one gangliocytoma and four gangliogliomas, both ultrastructurally and immuno-histochemically, using antibodies to neuroendocrine markers including tyrosine hydroxylase (TH), serotonin (5HT), somatostatin (SOM), met-enkephalin (MEK), leu-enkephalin (LEK), substance P (SP), gastrin, vasopressin, oxytocin, vasoactive intestinal polypeptide, adrenocorticotropic hormone and calcitonin. In all cases, the presence of dense-core vesicles (60–250 nm) in the neuronal elements was the characteristic ultrastructural finding. Synapses were observed in two cases. Immunohistochemically, variable numbers of neuronal cells showed positive staining for SOM in five cases, TH, MEK and LEK in three cases, and 5HT and SP in one case each. The others were negative. Positive immunoreactivity for multiple markers was shown in all cases. SOM, TH, 5HT and SP were present in the small- to medium-sized cells, while MEK and LEK were almost exclusively confined to the large cells. Our study clearly indicated that these tumors contained neuronal cells which were not homogeneous with regard to neuroendocrine markers.     

Rhabdoid tumors of the central nervous system

Rhabdoid tumors of the central nervous system are rare malignancies with a still almost uniformly fatal outcome. There is still no proven curative therapy available. We report our experience with nine patients with central nervous system rhabdoid tumors. Gross complete surgical removal of the tumor was achieved in six patients. Seven patients received intensive chemotherapy. Four of these were treated in addition with both neuroaxis radiotherapy and a local boost directed to the tumor region, while two patients received local radiotherapy only. The therapy was reasonably well tolerated in most cases. Despite the aggressive therapy, eight of the nine patients died from progressive tumor disease, and one patient died from hemorrhagic brain stem lesions of unknown etiology. The mean survival time was 10 months after diagnosis. Conventional treatment, although aggressive, cannot change the fatal prognosis of central nervous system rhabdoid tumors. As these neoplasms are so rare, a coordinated register would probably be a good idea, offering a means of learning more about the tumor’s biology and possible strategies of treatment. Received: 7 September 1999     

貌似肿瘤的中枢神经系统脱髓鞘病

目的总结貌似肿瘤的中枢神经系统炎性脱髓鞘病病例,分析其临床、影像学及病理特点,归纳临床误诊原因并探讨诊断及鉴别诊断要点.方法分析经术后病理证实的14例临床资料,包括病史、发病情况、临床症状、体征、手术中肉眼所见,影像学包括CT/MRI表现,病理包括HE染色,Luxolfastblue(Lfb)髓鞘染色,Bodian神经纤维染色,还进行了CD68,胶质纤维酸性蛋白(GFAP)及神经丝蛋白(NF)等免疫组化染色.结果中枢神经系统脱髓鞘病临床上可出现头痛、癫等症状,急性期CT/MRI可以出现水肿等占位效应、增强后可以有环形强化,冰冻切片可以观察到胶质细胞增生等.结论中枢神经系统脱髓鞘病可以有肿瘤样的非典型性表现,临床容易造成误诊.应在临床、影像学及病理学等多方面进行鉴别诊断.     

CD34 immunoreactivity in nervous system tumors

CD34 is a sialylated transmembrane glyco-protein of unknown function that is present in myeloid progenitor cells, endothelial cells, and some fibroblastrelated mesenchymal cells. However, its tissue distribution is still incompletely characterized. In this study we evaluated the distribution of CD34 antigen in tumors of the central and peripheral nervous system. For comparison the tumors were also stained for CD31, also known as platelet-endothelium cell adhesion molecule (PECAM-1), a transmembrane glycoprotein so far considered to be endothelium specific beyond its reactivity with certain hematopoietic cells. Neurofibromas showed consistently high numbers of CD34-positive spindle cells, whereas peripheral and acoustic schwannomas were negative. A subset of meningiomas (15%) showed CD34-positive tumor cells, and some were also weakly positive for CD31. Gliomas were negative. Meningeal hemangiopericytomas were consistently CD34 positive, but CD31 negative. These results indicate a moderately widespread distribution of the CD34 antigen in nervous system tumors, and necessitate caution in making conclusions regarding endothelial cell differentiation of nervous system tumors on the basis of CD34 immunoreactivity.     

Distribution of metallothionein in the human central nervous system

The distribution of metallothionein (MT), a metal-binding protein, was examined immunohistochemically in the normal human brain and spinal cord. Paraffinembedded brain tissue from three patients who had died from a non-neurological disease and were free of histopathological central nervous system alterations were processed. The results of the present study demonstrate that MT is readily detectable in a subgroup of astrocytes in the normal human brain. MT staining is most intense on grey matter astrocytes that bear short stout processes and which probably represent protoplasmic astrocytes. Using anti-MT and anti-glial fibrillary acidic protein immunostaining, we could demonstrate two subpopulations of astrocytes that were mutually exclusive. The functional significance of MT-expression in protoplasmic astrocytes is not entirely clear. Metal detoxification is only one of the many postulated functions of MT. The finding that staining for MT permits subtyping of astrocytes may be of great importance in glia research and surgical pathology of the human brain. © 1993 Wiley-Liss, Inc.     

Multiple sclerosis and malignant neoplasms in the central nervous system

Summary There is some evidence that the incidence of malignant neoplasms in the central nervous system of patients with multiple sclerosis (MS) is more infrequent than among the general population. The examination of 3 MS cases with different type of neoplasms in the central nervous system revealed mainly inactive demyelinated plaques. These plaques and the neoplastic lesions were dispersed diffusely and coincidentally all over the brain and spinal cord. The benign course of MS in all three cases is discussed in relation to the malignant diseases.Supported by the Deutsche Forschungsgemeinschaft, Schwerpunktprogramm Ätiologie und Pathogenese der Multiplen Sklerose und verwandter Erkrankungen