2021 WHO classification of central nervous system tumors

2021 年 WHO 中枢神经系统肿瘤分类
  • 文章类型: Journal Article
    2021年世界卫生组织中枢神经系统肿瘤分类更新了神经胶质瘤亚型和分级系统,并将EGFR扩增(Amp)作为胶质母细胞瘤(GBM)的诊断标志物之一。
    本研究旨在描述频率,基于最新分类的EGFRAmp在弥漫性胶质瘤中的临床价值和分子相关性。
    我们回顾了2011年至2022年在我们医院的神经胶质瘤患者,其中包括187名成人神经胶质瘤患者,有可用的肿瘤组织用于检测EGFRAmp和其他59个感兴趣的分子标志物。临床,根据EGFRAmp在不同胶质瘤亚型中的状态分析影像学和病理学资料。
    163个胶质瘤被分类为成人型弥漫性胶质瘤,星形细胞瘤的数量,少突胶质细胞瘤和GBM分离为41、46和76。EGFRAmp在IDH野生型弥漫性神经胶质瘤(66.0%)和GBM(85.5%)中比IDH突变型弥漫性神经胶质瘤(32.2%)及其亚型(星形细胞瘤,29.3%;少突胶质细胞瘤,34.8%)。EGFRAmp未对IDH突变型弥漫性神经胶质瘤和星形细胞瘤的总生存期(OS)进行分层,虽然与IDH野生型弥漫性神经胶质瘤的OS较差显著相关,组织学2级和3级IDH-野生型弥漫性星形胶质细胞瘤和GBM。
    我们的研究验证了EGFRAmp是GBM的诊断标志物,并且仍然是该组中OS缩短的有用预测因子。
    UNASSIGNED: The 2021 World Health Organization Classification of Central Nervous System Tumors updates glioma subtyping and grading system, and incorporates EGFR amplification (Amp) as one of diagnostic markers for glioblastoma (GBM).
    UNASSIGNED: This study aimed to describe the frequency, clinical value and molecular correlation of EGFR Amp in diffuse gliomas based on the latest classification.
    UNASSIGNED: We reviewed glioma patients between 2011 and 2022 at our hospital, and included 187 adult glioma patients with available tumor tissue for detection of EGFR Amp and other 59 molecular markers of interest. Clinical, radiological and pathological data was analyzed based on the status of EGFR Amp in different glioma subtypes.
    UNASSIGNED: 163 gliomas were classified as adult-type diffuse gliomas, and the number of astrocytoma, oligodendroglioma and GBM was 41, 46, and 76. EGFR Amp was more common in IDH-wildtype diffuse gliomas (66.0%) and GBM (85.5%) than IDH-mutant diffuse gliomas (32.2%) and its subtypes (astrocytoma, 29.3%; oligodendroglioma, 34.8%). EGFR Amp did not stratify overall survival (OS) in IDH-mutant diffuse gliomas and astrocytoma, while was significantly associated with poorer OS in IDH-wildtype diffuse gliomas, histologic grade 2 and 3 IDH-wildtype diffuse astrocytic gliomas and GBM.
    UNASSIGNED: Our study validated EGFR Amp as a diagnostic marker for GBM and still a useful predictor for shortened OS in this group.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    背景:2021年发布的世界卫生组织(WHO)中枢神经系统(CNS)肿瘤分类的最新第五版(WHOCNS5分类)定义了星形细胞瘤,IDH-突变体,4年级。然而,对这种亚型的理解仍然有限。我们进行了这项研究来描述星形细胞瘤的特征,IDH-突变体,4级,并探讨了组织学和分子亚型之间的异同。
    方法:2011年1月至2022年1月接受手术的患者,分类为星形细胞瘤,IDH-突变体,该研究包括4级。临床,放射学,组织病理学,分子病理学,收集生存数据进行分析.
    结果:总共33例星形细胞瘤患者,IDH-突变体,4年级被选中,包括20例组织学和13例分子WHO4级星形细胞瘤。肿瘤增强,肿瘤内坏死样表现,肿瘤周围水肿较大,在组织学WHO4级星形细胞瘤中经常观察到更明确的肿瘤边缘。此外,分子WHO4级星形细胞瘤显示出相对较长的总体生存期的趋势,而没有达到统计学意义(47vs.25个月,p=0.22)。通常观察到TP53、CDK6和PIK3CA改变,而PIK3R1(p=0.033),Notch1(p=0.027),和Mycn(p=0.027)改变可能会影响WHO4级星形细胞瘤的总体生存率。
    结论:我们的研究仔细检查了IDH突变体,4级星形细胞瘤。因此,应考虑进一步分类,因为组织学和分子WHO4级星形细胞瘤的预后不同.值得注意的是,针对PIK3R1,Notch1和Mycn的治疗可能是有益的.
    The latest fifth edition of the World Health Organization (WHO) classification of the central nervous system (CNS) tumors (WHO CNS 5 classification) released in 2021 defined astrocytoma, IDH-mutant, Grade 4. However, the understanding of this subtype is still limited. We conducted this study to describe the features of astrocytoma, IDH-mutant, Grade 4 and explored the similarities and differences between histological and molecular subtypes.
    Patients who underwent surgery from January 2011 to January 2022, classified as astrocytoma, IDH-mutant, Grade 4 were included in this study. Clinical, radiological, histopathological, molecular pathological, and survival data were collected for analysis.
    Altogether 33 patients with astrocytoma, IDH-mutant, Grade 4 were selected, including 20 with histological and 13 with molecular WHO Grade 4 astrocytoma. Tumor enhancement, intratumoral-necrosis like presentation, larger peritumoral edema, and more explicit tumor margins were frequently observed in histological WHO Grade 4 astrocytoma. Additionally, molecular WHO Grade 4 astrocytoma showed a tendency for relatively longer overall survival, while a statistical significance was not reached (47 vs. 25 months, p = 0.22). TP53, CDK6, and PIK3CA alteration was commonly observed, while PIK3R1 (p = 0.033), Notch1 (p = 0.027), and Mycn (p = 0.027) alterations may affect the overall survival of molecular WHO Grade 4 astrocytomas.
    Our study scrutinized IDH-mutant, Grade 4 astrocytoma. Therefore, further classification should be considered as the prognosis varied between histological and molecular WHO Grade 4 astrocytomas. Notably, therapies aiming at PIK3R1, Notch 1, and Mycn may be beneficial.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    老年胶质母细胞瘤(GBM)患者的特点是发病率高,预后差。目前,然而,老年GBM患者仍然缺乏足够的分子表征。WHO第5版中枢神经系统肿瘤分类(WHO5)为GBM提供了一种新的分类方法,老年GBM患者的分子特征需要在这个新框架下进行研究。
    比较了不同分类和不同年龄患者的临床和影像学特征。使用单变量Cox回归和Kaplan-Meier生存分析发现WHO5分类下老年GBM患者的潜在预后分子标志物。
    总共226名患者被纳入研究。在WHO5分类下,年轻和老年GBM患者的预后差异更为明显。神经功能缺损在老年患者中更为常见(p=0.001),而颅内高压(p=0.034)和癫痫(p=0.038)在年轻患者中更为常见。老年患者更可能有更高的Ki-67(p=0.013),在老年WHO5GBM患者中,KMT5B(p=0.082),KRAS(p=0.1)和PPM1D(p=0.055)均与总生存期(OS)相关。其中,发现KRAS和PPM1D是WHO5老年GBM患者特有的预后特征。
    我们的研究表明,WHO5分类可以更好地区分老年和年轻GBM的预后。此外,KRAS和PPM1D可能是WHO5老年GBM患者的潜在预后预测因子。这两个基因在老年GBM中的具体机制还有待进一步研讨。
    UNASSIGNED: Elderly glioblastoma (GBM) patients is characterized by high incidence and poor prognosis. Currently, however, there is still a lack of adequate molecular characterization of elderly GBM patients. The fifth edition of the WHO Classification of Central Nervous System Tumors (WHO5) gives a new classification approach for GBM, and the molecular characteristics of elderly GBM patients need to be investigated under this new framework.
    UNASSIGNED: The clinical and radiological features of patients with different classifications and different ages were compared. Potential prognostic molecular markers in elderly GBM patients under the WHO5 classification were found using Univariate Cox regression and Kaplan-Meier survival analysis.
    UNASSIGNED: A total of 226 patients were included in the study. The prognostic differences between younger and elderly GBM patients were more pronounced under the WHO5 classification. Neurological impairment was more common in elderly patients (p = 0.001), while intracranial hypertension (p = 0.034) and epilepsy (p = 0.038) were more common in younger patients. Elderly patients were more likely to have higher Ki-67(p = 0.013), and in elderly WHO5 GBM patients, KMT5B (p = 0.082), KRAS (p = 0.1) and PPM1D (p = 0.055) were each associated with overall survival (OS). Among them, KRAS and PPM1D were found to be prognostic features unique to WHO5 elderly GBM patients.
    UNASSIGNED: Our study demonstrates that WHO5 classification can better distinguish the prognosis of elderly and younger GBM. Furthermore, KRAS and PPM1D may be potential prognostic predictors in WHO5 elderly GBM patients. The specific mechanism of these two genes in elderly GBM remains to be further studied.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

  • 文章类型: Journal Article
    2021年发布的世界卫生组织(WHO)中枢神经系统(CNS)肿瘤分类第五版正式定义了小儿型弥漫性神经胶质瘤。然而,对小儿型弥漫性神经胶质瘤的了解仍然很少,对成年患者的关注甚至更少。因此,这项研究描述了临床放射学,生存,和成人儿童型胶质瘤患者的分子特征。
    从2011年1月至2022年1月接受手术的成年患者,分类为小儿型神经胶质瘤,包括在这项研究中。临床,放射学,组织病理学,分子病理学,收集生存数据进行分析.
    在596名成年患者中,筛查了20例小儿型胶质瘤患者,包括6个弥漫性星形细胞瘤,MYB-或MYBL1-改变,2伴有弥漫性中线胶质瘤,H3K27-改变,和12患有弥漫性小儿型高级别神经胶质瘤,H3-野生型和IDH-野生型。小儿高级别胶质瘤(pHGG)经常显示肿瘤增强,瘤周水肿,和肿瘤内坏死.成人pHGG患者比成人胶质母细胞瘤患者的预期寿命更长。常见的分子改变包括染色体改变和CDKN2A/B,PIK3CA,和PTEN,而改变的KMT5B和MET被发现影响总生存期。
    我们的研究证明了患有儿童型神经胶质瘤的成年患者。值得注意的是,我们的研究旨在扩大目前对成人儿童型弥漫性胶质瘤患者的认识.此外,由MET和VEGFA的靶向分子抑制剂组成的个性化治疗可能在相应人群中表现出有益效果.
    UNASSIGNED: The fifth edition of the World Health Organization (WHO) classification of central nervous system (CNS) tumors released in 2021 formally defines pediatric-type diffuse gliomas. However, there is still little understanding of pediatric-type diffuse gliomas, and even less attention has been paid to adult patients. Therefore, this study describes the clinical radiological, survival, and molecular features of adult patients with pediatric-type glioma.
    UNASSIGNED: Adult patients who underwent surgery from January 2011 to January 2022, classified as pediatric-type glioma, were included in this study. Clinical, radiological, histopathological, molecular pathological, and survival data were collected for analysis.
    UNASSIGNED: Among 596 adult patients, 20 patients with pediatric-type glioma were screened, including 6 with diffuse astrocytoma, MYB- or MYBL1-altered, 2 with diffuse midline glioma, H3 K27-altered, and 12 with diffuse pediatric-type high-grade glioma, H3-wildtype and IDH-wildtype. Pediatric high-grade glioma (pHGG) frequently showed tumor enhancement, peritumoral edema, and intratumoral necrosis. Adult patients with pHGG showed a longer life expectancy than adult patients with glioblastoma. Common molecular alterations included chromosome alterations and CDKN2A/B, PIK3CA, and PTEN, while altered KMT5B and MET were found to affect the overall survival.
    UNASSIGNED: Our study demonstrated adult patients with pediatric-type glioma. Notably, our research aims to expand the current understanding of adult patients with pediatric-type diffuse gliomas. Furthermore, personalized therapies consisting of targeted molecular inhibitors for MET and VEGFA may exhibit beneficial effects in the corresponding population.
    导出

    更多引用

    收藏

    翻译标题摘要

    我要上传

       PDF(Pubmed)

公众号