WHO中枢神经系统肿瘤分类(2007)评介

《WHO中枢神经系统肿瘤分类》第4版（以下简称第4版分类）于2007年6月由国际癌症研究机构（International Agency for Researchon Cancer,IARC）正式出版,WHO出版社发行,反映了自第3版WHO神经系统肿瘤分类（2000）出版㈨以来神经肿瘤病理学领域的重要进展。     

原发性中枢神经系统生殖细胞肿瘤3例临床病理分析

目的探讨原发性中枢神经系统生殖细胞肿瘤(central nervous system germ cell tumors,CNS GCT)的临床病理学特征。方法回顾性分析3例CNS GCT患者的临床病理特征、免疫表型、诊断、鉴别诊断、治疗及预后,并复习相关文献。结果3例患者均为男性,发病部位:例1鞍区第三脑室,例2左侧基底节区,例3松果体区。镜检:例1瘤细胞呈腺管状结构,软骨样基质呈片状分布,内见钙化;例2瘤细胞密集增生,围绕血管形成乳头状结构,胞质丰富,透明状,可见大片坏死及出血,核分裂象可见;例3于3个低倍镜视野中分别显示少数原始神经管成分。免疫表型:例1中PLAP、CD117生殖细胞均(+),PCK腺上皮(+),EMA部分腺上皮(+);例2中PCK(+),CD30、α-FP生殖细胞均(+),S-100蛋白(点+);例3中NSE和S-100蛋白原始神经上皮均(+),PCK腺上皮(+),GFAP及EMA均部分(+)。结论原发性CNS GCT有明显的临床特征,好发于儿童,且男性比例高;及时诊断、早期进行适当的干预,多数患者预后较好。     

中枢神经系统肿瘤的分类和分级探讨--4 373例病理分析

目的 探讨世界卫生组织（WHO）中枢神经系统肿瘤分类的临床应用。方法 根据4年来学习和应用WHO分类和分级的情况,结合4373例的病理分析,研究具体推行过程中存在的问题。结果 （1）2000分类 采用WHO和ICD-O（国际疾病分类肿瘤部分）的双重标准,其中的对应关系十分混乱。（2）对脑膜上皮的脑膜肿瘤细分为15种类型;存在分歧。（3）良性,非典型和间变性脑膜瘤的界定有困难。（4）间变性胶质瘤的诊断标准含混,（5）实际上,胶质母细胞瘤是一种混合性胶质瘤。（6）对特殊类型的神经上皮组织肿瘤的认识不够,结论 建议制定更适合临床应用的分类和分级标准。     

中枢神经系统孤立性纤维性肿瘤4例临床病理分析

目的探讨中枢神经系统孤立性纤维性肿瘤(solitary fibrous tumor,SFT)的临床病理特征及鉴别诊断。方法回顾性分析4例中枢神经系统SFT的临床特点,行HE和免疫组化染色,镜下观察其病理组织学特征。结果 4例均为中年男性,镜下见肿瘤细胞呈梭型或胖梭形,呈席纹状排列,免疫组化标记vimentin、BCL-2、CD34阳性率为100%。结论中枢神经系统SFT需与纤维型脑膜瘤相鉴别,免疫组化标记有助于鉴别,避免误诊造成过度治疗。     

5 109例中枢神经系统肿瘤WHO新分类统计分析

目的 探讨新的WHO分类在中枢神经系统肿瘤病理诊断及统计学分析中的意义。方法 对1999年7月～2002年6月3年间华山医院诊治的5109例中枢神经系统肿瘤采用免疫细胞化学ABC法及20余种抗体标记，依据WHO神经系统肿瘤新分类作病理诊断，并作统计分析。结果 在5109例肿瘤中，按WHO(2000)分类，属神经系统肿瘤有3981例，其中神经上皮组织肿瘤l498例(占37．64％)，颅脊神经肿瘤536例(占13．46％)，脑膜肿瘤1379例(占34．64％)，其中脑膜上皮组织肿瘤1130例(占28．38％)和脑膜间叶组织肿瘤249例(占6．25％)。未定组织来源肿瘤(血管母细胞瘤)110例(占2．76％)。淋巴瘤和造血组织肿瘤72例(占1．80％)，胚生殖细胞肿瘤52例(占1．32％)，鞍区颅咽管瘤139例(占3．49％)和转移性肿瘤195例(占4．90％)。同期的非神经系统肿瘤，即垂体腺肿瘤1042例(占20．39％)和其他类肿瘤86例(占1．68％)。结论 脑膜肿瘤和神经上皮组织肿瘤中少突胶质细胞肿瘤比例均有升高，认识先进仪器和设备并应用于肿瘤诊断，并认识少突胶质肿瘤病理形态学本质及胶质母细胞瘤新概念，以及坚持WHO分类的原则是重要的。     

儿童中枢神经系统肿瘤中INI1的表达及意义

目的研究整合酶相互作用分子1(Integrase Interactor1,INI1)在儿童中枢神经系统肿瘤的表达情况,探讨其用于中枢神经系统肿瘤诊断及鉴别诊断的意义。方法应用组织芯片和免疫组织化学方法检测INI1在55例儿童中枢神经系统肿瘤中的表达。结果 2例非典型畸胎样/横纹肌样瘤,肿瘤细胞INI1表达缺失,肿瘤内的血管内皮细胞阳性表达INI1,其余53例肿瘤包括星形细胞瘤、室管膜瘤、髓母细胞瘤、PNET等肿瘤细胞INI1均阳性表达。结论 INI1在非典型畸胎样/横纹肌样瘤中特异性表达缺失,可用于诊断及鉴别诊断非典型畸胎样/横纹肌样瘤与其他中枢神经系统肿瘤;使用免疫组织化学方法检测INI1是一种方便快速、可靠的方法;有必要对所有儿童中枢神经系统肿瘤尤其是胚胎性肿瘤应用免疫组织化学方法检测INI1表达的情况。     

100例中枢神经系统肿瘤的胶质纤维酸性蛋白免疫组化观察

对100例中枢神经系统肿瘤进行GFAP免疫组化观察。结果表明,GFAP主要分布于各种类型的星形细胞瘤,多形性胶质母细胞瘤,混合性胶质瘤及部分室管膜瘤。GFAP染色强度与瘤细胞分化程度有关。本文还探讨了室管膜瘤与少突胶质瘤存在GFAP问题。研究表明GFAP可作为诊断神经胶质瘤的一种有用标记物。     

WHO(2007年)中枢神经系统肿瘤分类的新变化

WHO(2007年版)中枢神经系统肿瘤分类出现了一些新的变化和特点,对肿瘤的认识也更加深入全而和细致,强调组织学分级对临床治疗的影响及临床预后的多因素性,并增加或明确了11种新的肿瘤种类或亚犁或变异型,其中一些对解决实际工作中的诊断问题有较大的帮助. 1 中枢神经系统肿瘤WHO(2007年)分类的特点     

中枢神经系统的胶质神经元肿瘤

中枢神经系统的胶质神经冗肿瘤是含有肿瘤性神经元和胶质两种成分的神经上皮肿瘤,但不包括可以向神经细胞和胶质双向分化的胚胎性肿瘤。胶质神经元肿瘤较少见,其中相对常见的是节细胞胶质瘤,而胚胎发育不良性神经上皮肿瘤、促纤维增生性婴儿节细胞胶质瘤以及近年来陆续报道的一些具有形态特点的新的胶质神经元肿瘤,如乳头状胶质神经元肿瘤和伴有神经毡样岛或菊形团的胶质神经元肿瘤,都是少见的类型。     

原发性中枢神经系统弥漫大B细胞性淋巴瘤16例临床病理分析

目的 探讨原发性中枢神经系统弥漫大B细胞淋巴瘤(diffuse large B-cell lymphomas,DLBCL)的临床病理特点和免疫分子亚型及与预后的相关性.方法 回顾性分析16例原发性中枢神经系统DLBCL的临床表现、影像特点,采用HE染色及EliVision法观察其病理组织学特点和免疫表型特征.结果 临床上主要表现为颅内压增高、肢体乏力、视力障碍和神经精神症状,可为单发或多发性病灶;影像学上,CT示肿块多呈稍低密度阴影,MRI示T1WI呈低或等信号,T2WI呈高或等信号,强化明显,病灶周围常见明显水肿带.组织学特点为瘤细胞细胞形态较单一,弥漫浸润,瘤细胞常围绕血管形成袖套状,伴片状坏死及出血.免疫分子分型示9例DLBCL为生发中心型,7例为非生发中心型.结论 原发性中枢神经系统DLBCL是高度侵袭性淋巴瘤,为2008年WHO淋巴造血系统肿瘤分类已新增加的DLBCL亚型,具有较独特的临床病理学特点及生物学行为.好发中老年人,临床表现和影像学缺乏特异性.病理组织形态特点为瘤细胞弥漫或结节分布,以瘤细胞常围绕血管袖套状浸润为其特征性结构,可有坏死、出血;分子亚型主要为生发中心型.     

Central nervous system involvement in patients with acute myeloid leukemia

Background/aim To evaluate the incidence, clinical features, risk factors, and prognosis of central nervous system (CNS) involvement in patients with acute myeloid leukemia (AML).Materials and methodsAll AML patients who were admitted to Hacettepe University hospital between 2000 and 2021 were evaluated. The medical records of 548 AML cases were retrospectively analyzed. Results The frequency of CNS involvement was 2.4% (n = 13) at diagnosis and 4.6% (n = 25) at diagnosis or during follow-up. Parenchymal involvement was seen in 5 patients, leptomeningeal involvement was seen in 11 patients. Three patients had both leptomeningeal and parenchymal involvements, and 6 patients had optic nerve or ocular involvement. In univariate analysis, younger age and extramedullary involvement at diagnosis were associated with CNS disease at diagnosis, and extramedullary involvement at diagnosis was associated with CNS disease during follow-up. In multivariate analysis; younger age and extramedullary involvement at diagnosis were associated with CNS disease at diagnosis and during follow-up respectively. Median overall survival was 5.4 months in patients with CNS disease at diagnosis and 16.9 months in patients with CNS disease during follow-up and 16.2 months in patients with no CNS disease.Conclusion CNS disease is a rare complication of AML. Younger age and extramedullary involvement at diagnosis are risk factors for CNS involvement.     

原发性中枢神经系统淋巴瘤9例浸润播散方式的临床病理观察

目的 探讨原发性中枢神经系统淋巴瘤(primary central nervous system lymphoma,PCNSL)在脑组织内浸润播散的方式.方法 复习9例PCNSL的临床病理资料,重点观察其在脑组织内浸润播散方式.结果 9例PCNSL中,8例为手术标本,1例为尸解标本,病理类型均为非霍奇金淋巴瘤,8例为弥漫大B细胞淋巴瘤,1例为非特异性外周T细胞淋巴瘤,中位年龄57岁,观察到3种基本浸润和播散方式:(1)沿血管周隙浸润,破坏血管周隙在血管周围浸润,侵入血管壁呈血管炎状改变,使血管腔闭塞.(2)沿神经束间浸润,在大脑灰质层和小脑分子层形成单个或数个细胞与脑表面垂直的条索向脑表面软脑膜下浸润.(3)上述两种方式浸润的瘤细胞最终进入脑皮质浅层和蛛网膜下腔.结论 PCNSL沿血管周隙、血管周围、神经纤维间隙等结构浸润至脑皮层浅层和蛛网膜下隙,并进一步播散.     

Congenital tumors of the central nervous system: an institutional review of 64 cases with emphasis on tumors with unique histologic and molecular characteristics

Congenital brain tumors are rare accounting for 0.5%–1.9% of all pediatric brain tumors. While different criteria have been used to classify a tumor as congenital, those diagnosed prior to 6 months of age are considered to be “probably” congenital in origin. We performed an institutional review of all central nervous system (CNS) tumors (surgical and autopsy specimens from 1990 to 2019) in patients less than 6 months old. Sixty‐four unique cases were identified, and these accounted for 2.0% of all CNS tumor specimens at our institution. The most common tumor types were high‐grade gliomas, low‐grade gliomas and medulloblastomas. Atypical teratoid rhabdoid tumors, choroid plexus tumors and germ cell tumors also accounted for a significant portion of the cohort. Seven tumors were diagnosed prenatally. The most common clinical presentation at diagnosis was increased head circumference. At the conclusion of the study, over half of the patients were alive including all patients with WHO grade I and II tumors. Ninety‐two percent of cases were classifiable using the 2016 WHO system, and when available, molecular findings supported the histologic diagnoses. However, several gliomas had unusual histologic features and did not correspond to a well‐defined entity. Molecular testing was essential for accurate classification of a subset of these tumors, and several high‐grade gliomas exhibited fusions considered unique to infantile gliomas, including those involving the MET, ALK and NTRK genes. To our knowledge, this cohort represents the largest single‐institution study of congenital CNS tumors and highlights many ways in which congenital CNS tumors are distinct from CNS tumors of older pediatric patients and adults.     

Selective central nervous system tropism of primary central nervous system lymphoma

Primary Central nervous system lymphoma (PCNSL) is most frequently a diffuse large B cell lymphoma (DLBCL), which is confined to the Central nervous system (CNS). We performed an experiment in which lymphoma cells from a PCNSL patient were implanted subcutaneously in an athymic mouse. The lymphoma cells were shown to home to the CNS with histologic evaluations of the brain showing multiple large B cells in blood vessels consistent with intravascular large B cell lymphoma (IVL). We did not find any evidence of lymphoma at the site of implantation or other locations. The findings are consistent with highly selective tropism of PCNSLforthe CNS and its vasculature.     

Intraoperative squash cytology of central nervous system lesions: a single center study of 326 cases

Cytology has been shown to be of great value in intraoperative consultations of central nervous system (CNS) pathology. Intraoperative smear cytology provides a rapid and reliable intraoperative diagnosis and guidance to the neurosurgeon during surgical resection and lesion targeting. It also helps the surgeon to monitor and modify the approach at surgery. The current study was undertaken to assess the accuracy and utility of intraoperative consultations for cytomorphological diagnosis by smear technique and correlate with histopathological diagnosis. A retrospective study of 326 cases of CNS intraoperative consultations was performed. Smears were prepared from the biopsy samples sent in isotonic saline for immediate processing and stained by the Haematoxylin and Eosin method. The cytomorphological features were noted and correlated with final histopathological diagnosis. Concordance between the intraoperative diagnosis and the final diagnosis was seen in 83.7% of cases. We demonstrated >95% accuracy for glioblastomas and >89% accuracy for meningiomas and schwannomas. A reduction of diagnostic accuracy was seen in oligodendrogliomas (60%) and anaplastic oligodendrogliomas (57.2%). Smear technique is a fairly accurate, relatively safe, rapid, simple, easily reproducible, and cost effective tool to diagnose brain tumors. Smear cytology is of great value in intraoperative consultation of CNS pathology.     

Pathology of fungal infections of the central nervous system: 17 years' experience from Southern India

Aims:  To describe the pathology of central nervous system (CNS) fungal infections with particular reference to India.
Methods and results:  This was a retrospective study from 1988 to 2004 constituting 130 cases. The diagnosis was based on morphology of biopsy/autopsy material. These included aspergillosis ( n  = 73), zygomycosis ( n  = 40), cryptococcosis ( n  = 2), rhodotorulosis ( n  = 1), candidiasis ( n  = 5), maduramycosis ( n  = 1), pheohyphomycosis ( n  = 3) and mixed infections ( n  = 5). Predisposing risk factors were present in 49 (38%) patients only. The majority of the patients were immunocompetent. The commonest risk factor was diabetes mellitus, the commonest route of infection was from a contiguous site and the commonest pathology was granuloma. Culture positivity was seen in only 31%.
Conclusion:  Environmental factors in tropical countries such as India play a significant role in the pathogenesis of CNS fungal infections.