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.     

青少年多形性低级别神经上皮肿瘤的临床病理及分子病理特征分析

目的 分析青少年多形性低级别神经上皮肿瘤（PLNTY）的临床病理及分子病理特点。 方法 选取 2021 年 5 月— 2023 年 5 月于首都医科大学附属北京天坛医院接受手术治疗的 16 例 PLNTY 患者为研究对象,收集患者临床及影像学资料。采用苏木精 - 伊红染色、免疫组织化学染色及基因测序 检测肿瘤组织形态学及分子遗传学变化,电话随访及查阅影像学资料了解患者的症状改善情况、有无 放化疗及肿瘤复发情况。结果 16 例 PLNTY 患者中位发病年龄 24 岁,男女比例相当（1∶1）;5 例（5/16） 呈急性起病（起病时间＜ 3 个月）;临床症状以不同程度的癫痫发作为主;8 例（8/16）肿瘤位于颞叶,5 例 （5/16）位于顶枕叶。增强磁共振成像扫描显示,11例（11/16）无明显强化,3例（3/16）出现轻度不均匀强化, 2 例（2/16）明显不均匀强化。14 例（14/16）患者术后癫痫症状改善,其中 1 例复发。组织病理学结果显 示肿瘤呈浸润性生长,位于皮层及皮层下白质,可见多形性和少突样细胞形态,并见枝芽状细小血管, 12 例（12/16）伴钙化;其中 1 例复发再次手术切除的标本中呈现恶性转化,表现为局部细胞密度增高, 可见核分裂象、微血管增生和栅栏样坏死。16 例肿瘤免疫组织化学染色结果显示,胶质纤维酸性蛋白 （GFAP）、少突胶质细胞转录因子 2（Olig2）及 CD34 弥漫阳性,突触素呈阴性表达,其中 14 例 ki67 增殖指 数≤ 4%,恶性转化病例的 Ki67 增殖指数为 20%。7 例 PLNTY 二代测序结果显示,以BRAF或FGFR分子 变异为主,其中 3 例（3/7）存在 O6- 甲基鸟嘌呤 -DNA 甲基转移酶启动子（MGMT）甲基化;1 例原发及复发 的病例中均检测到FGFR3：：TACC3融合,伴 TERT 启动子突变,且在复发病例中出现了 10 号染色体缺 失及CDK4、MDM2扩增。结论 PLNTY 好发于青少年,为一种长期癫痫相关肿瘤,多见于颞叶及顶枕 叶;肿瘤呈浸润性生长,可见多形性细胞和少突样细胞成分,CD34 弥漫阳性,分子改变以BRAF或FGFR 变异为主。大部分预后良好,术后癫痫症状改善;个别病例复发后可发生恶性转化,分子病理结果显示 FGFR3：：TACC3融合并 TERT 启动子突变;临床医生需要对 PLNTY 临床病理及分子病理特征进行综合 评估并提高警惕。     

中枢神经系统肿瘤的病理

简明地介绍脑肿瘤的病理检查以及诊断原则 ,并根据 2 0 0 0年世界卫生组织 (WHO)关于神经系统肿瘤的分类 ,扼要介绍主要颅脑肿瘤的病理特点 ,着重介绍 1 0种特殊类型的神经上皮肿瘤的临床和病理特点以及治疗上的考虑     

神经副肿瘤综合征

神经副肿瘤综合征(paraneoplastic neurologic syndromes,PNS)是一组与恶性肿瘤相关,非代谢感染、退行性、转移性或医源性的神经系统疾病受累的疾病。经典的PNS包括了脑脊髓炎、边缘性脑炎(limbic encephalitis,LE)、亚急性小脑变性、感觉神经病、Lambert-Eaton肌无力综合征(Lambert-Eaton myasthenic syndrome,LEMS)、皮肌炎等[1]。目前认为,部分PNS发生与肿瘤神经元抗体相关,其病程特征通常是亚急性起病、进行性进展之后病情趋于稳定。少数PNS病例可在原发性PNS后出现第二个PNS。PNS最常见的原发性肿瘤包括了小细胞肺癌(small-cell lung cancer,SCLC)、卵巢癌、乳腺癌、神经内分泌肿瘤、胸腺瘤和淋巴瘤。PNS的症状通常出现在发现恶性肿瘤之前,因而易导致误诊。诊断PNS对于早期干预原发性疾病,稳定和改善症状尤为关键。     

第二届亚洲神经病理学术会议暨第11届全国神经病理学术会议通知

受业洲神经病理学会(Asian Society of Neuropathology)的委托,由亚洲神经病理学会、中华医学会神经病学分会神经病理学组和病理学分会脑神经病理学组联合主办的第二届亚洲神经病理学术会议(Secnd Congress of Asian Society ofNeuropthology),拟定于2011年11月3-7日在北京隆重召开,并同期举办第11届全国神经病理学术会议。本次盛会旨在介绍国内外神经病理学基础与神经病学临床研究的最新进展,加强国内外同行专家的交流与合作,为亚洲神经病理学和神经病学专家、学者提供一个良好的学术和经验交流平台。会议特别邀请亚洲及欧美等国家著名神经病理学和神经病学专家作报告和讲座,并进行大会论文发言、壁报交流等形式多样、内容丰富的学术活动。参会者将授予国家级继续医学教育Ⅰ类学分6分。     

第二届亚洲神经病理学术会议暨第11届全国神经病理学术会议通知

受亚洲神经病理学会（Asian Society of Neuropathology）的委托,由亚洲神经病理学会、中华医学会神经病学分会神经病理学组和病理学分会脑神经病理学组联合主办的第二届亚洲神经病理学术会议（Second Congress of Asian Society of Neuropathology）,拟定于2011年11月3—7日在北京市隆重召开,并同期举办第11届全国神经病理学术会议。     

难治性癫痫杏仁核神经病理研究

关于癫痫的杏仁核神经病理研究逐渐增多。我院对2例难治性癫痫病人行左侧杏仁核毁损前,作局部活检,1例为正常组织,1例为轻度胶质增生。这样在作     

第二届亚洲神经病理学术会议暨第11届全国神经病理学术会议通知

受亚洲神经病理学会(Asian Society of Neuropathology)的委托,由亚洲神经病理学会、中华医学会神经病学分会神经病理学组和病理学分会脑神经病理学组联合主办的第二届亚洲神经病理学术会议(Second Congress of Asian Society ofNeuropathology),拟定于2011年11月3-7日在北京市隆重召开,并同期举办第11届全国神经病理学术会议。本次盛会旨在介绍国内外神经病理学基础与神经病学临床研究的最新进展,加强国内外同行专家的交流与合作,为亚洲神经病理学和神经病     

阿尔茨海默病神经病理标志物的研究进展

阿尔茨海默病(Alzheimer's disease,AD)是一种缓慢进展的原发性退行性脑变性疾病,多起病于老年期,潜隐起病,缓慢不可逆进展,临床上以认知损害为主.     

中枢神经系统肿瘤病理学的十年进展

近10年来,中枢神经系统肿瘤病理学的新进展主要包括组织病理学和分子病理学两大方面.组织病理学进展主要为中枢神经系统肿瘤WHO分类和分级标准的不断完善,相继发现了一些新的肿瘤类型,对癫痫相关肿瘤的认识更加深化.随着分子生物学与分子遗传学新技术在该类肿瘤研究中的应用,已发现一系列对诊断、鉴别诊断及指导分子靶向治疗有实用价值的生物学标志,并根据生物学标志的不同,初步提出了胶质瘤的分子遗传学分型.     

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.     

中枢神经系统常见肿瘤分子病理学及靶向治疗

中枢神经系统肿瘤采用传统治疗方法难以治愈,由于传统化疗药物缺乏特异性,在抗肿瘤的同时,对正常组织和器官也产生毒性作用。因此,针对中枢神经系统肿瘤细胞内分子通路特异性靶向治疗成为近年研究的热点。本文拟从细胞内关键责任信号转导通路及基因突变位点的角度分别介绍髓母细胞瘤、少突胶质细胞肿瘤和胶质母细胞瘤分子生物学特征及其与肿瘤预后的关系,以及肿瘤靶向治疗现状及进展,为中枢神经系统肿瘤的个体化治疗提供一些新的信息。     

Chemokines in central nervous system pathology

Chemokines are a family of proinflammatory cytokines which are able to stimulate directional migration of leukocytes in vitro and in vivo. Because of this feature chemokines may be potent mediators of inflammatory processes. Numerous observations indicate that chemokines may be involved in the pathogenesis of autoimmune and infectious inflammation within the central nervous system (CNS). Moreover, there are many reports showing increased expression of some chemokines in experimental models of brain mechanical injury and ischaemia. Several lines of cultured CNS cells are able to produce chemokines in vitro. All those data suggest that chemokines are important mediators of CNS pathology and that they can be a promising target for future therapy of neurological diseases.     

Ultrastructural pathology of the peripheral nervous system

Summary The present paper gives an introduction to fundamental pathologic features of various diseases of the peripheral nervous system, such as Wallerian degeneration, axonal degeneration, segmental degeneration, primary impairment of the endoneural interstitium. It is based on electron-microscope analysis. The range of reactions available to the peripheral nervous system is limited; thus noxious influences of various etiologies will act via largely common pathogenic mechanisms to produce similar morphologic changes.Supported by the Swiss National Fund (3. 747. 72).     

Human nervous system tumors

Thick sections (0.5--2 mu) of biopsies from human nervous system tumors (Schwannoma, ependymoma, medulloblastoma), fixed in aldehydes followed by osmium, and stained with uranyl acetate and lead, have been studied at 2.5 MV, and compared to thin sections of the same material observed by ordinary low voltage electron microscopy. High voltage electron microscopy permits direct observation of cell fine structure in three dimensions, including the spatial relations of organelles. Details of contours of nuclear envelopes, shapes of mitochondria, fine aspects of the structure of cell surfaces and processes, such as the flat sheet-like and irregular cylindrical processes of Schwannoma cells, the small projections and ridges on their surfaces, and microvilli and cilia of ependymoma cells, and other features have been observed. These initial observations demonstrate the applicability of high voltage electron microscopy to further study of neural neoplasms.