Oligoastrocytoma

寡星形细胞瘤
  • 文章类型: Journal Article
    随着预期寿命的增加和人口老龄化,老年患者2级和3级胶质瘤的发病率正在增加。然而,对影响其治疗选择和总生存期(OS)的因素知之甚少.2010年至2017年,从国家癌症数据库中确定了年龄在60至89岁之间的经组织学证实为2级和3级颅内胶质瘤的老年患者。我们分析了病人的人口统计数据,肿瘤特征,治疗方式,和结果。采用Kaplan-Meier法分析OS。进行单变量和多变量分析以评估死亡率和治疗选择的预测因素。共确认6257例患者:3533例(56.3%)2063年(32.9%)七十岁老人,和679名(10.8%)八十岁老人。我们确定了患者OS较低的预测因素,包括人口因素(年龄、非零Charlson-Deyo得分,非西班牙裔种族),社会经济因素(低收入,在非学术中心治疗,政府保险),和肿瘤特异性因素(更高等级,星形细胞瘤组织学,多焦点)。接受手术和化疗与较低的死亡风险相关,而接受放疗与更好的OS无关。我们的发现为人口统计的复杂相互作用提供了有价值的见解,社会经济,以及影响老年2级和3级胶质瘤治疗选择和OS的肿瘤特异性因素。我们发现,年龄的增长与OS的降低和接受手术的可能性降低相关,化疗,或放射治疗。虽然接受手术和化疗与改善OS相关,放疗没有表现出类似的关联.
    With increasing life expectancies and population aging, the incidence of elderly patients with grade 2 and 3 gliomas is increasing. However, there is a paucity of knowledge on factors affecting their treatment selection and overall survival (OS). Geriatric patients aged between 60 and 89 years with histologically proven grade 2 and 3 intracranial gliomas were identified from the National Cancer Database between 2010 and 2017. We analyzed patients\' demographic data, tumor characteristics, treatment modality, and outcomes. The Kaplan-Meier method was used to analyze OS. Univariate and multivariate analyses were performed to assess the predictive factors of mortality and treatment selection. A total of 6257 patients were identified: 3533 (56.3 %) hexagenerians, 2063 (32.9 %) septuagenarians, and 679 (10.8 %) octogenarians. We identified predictors of lower OS in patients, including demographic factors (older age, non-zero Charlson-Deyo score, non-Hispanic ethnicity), socioeconomic factors (low income, treatment at non-academic centers, government insurance), and tumor-specific factors (higher grade, astrocytoma histology, multifocality). Receiving surgery and chemotherapy were associated with a lower risk of mortality, whereas receiving radiotherapy was not associated with better OS. Our findings provide valuable insights into the complex interplay of demographic, socioeconomic, and tumor-specific factors that influence treatment selection and OS in geriatric grade 2 and 3 gliomas. We found that advancing age correlates with a decrease in OS and a reduced likelihood of undergoing surgery, chemotherapy, or radiotherapy. While receiving surgery and chemotherapy were associated with improved OS, radiotherapy did not exhibit a similar association.
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  • 文章类型: Journal Article
    胶质瘤,一种普遍而致命的脑瘤,以显著的细胞异质性和代谢改变为标志。然而,高级细胞的起源和代谢综合景观(多形性胶质母细胞瘤,WHOIV级)和低级别(寡星形细胞瘤,WHOII级)神经胶质瘤仍然难以捉摸。
    在这项研究中,我们对这些胶质瘤级别进行了单细胞转录组测序,以阐明它们的细胞和代谢差异.在确定细胞类型之后,我们比较了高级别和低级别胶质瘤的代谢途径活性和基因表达。
    值得注意的是,星形胶质细胞和少突胶质细胞祖细胞(OPCs)在代谢途径和基因表达方面表现出最实质性的差异,表明了它们不同的起源。综合分析确定了所有细胞类型中变化最大的代谢途径(MCP)和基因,针对TCGA和CGGA数据集进一步验证了其临床相关性。
    至关重要的是,发现代谢酶磷酸二酯酶8B(PDE8B)在高级别神经胶质瘤的星形胶质细胞和OPCs中仅表达并逐渐下调.这种降低的表达将PDE8B鉴定为IDH突变型神经胶质瘤中的代谢相关癌基因,标记其作为神经胶质瘤分级和预后的保护性标记和作为神经胶质瘤进展的促进者的双重作用。
    UNASSIGNED: Glioma, a prevalent and deadly brain tumor, is marked by significant cellular heterogeneity and metabolic alterations. However, the comprehensive cell-of-origin and metabolic landscape in high-grade (Glioblastoma Multiforme, WHO grade IV) and low-grade (Oligoastrocytoma, WHO grade II) gliomas remains elusive.
    UNASSIGNED: In this study, we undertook single-cell transcriptome sequencing of these glioma grades to elucidate their cellular and metabolic distinctions. Following the identification of cell types, we compared metabolic pathway activities and gene expressions between high-grade and low-grade gliomas.
    UNASSIGNED: Notably, astrocytes and oligodendrocyte progenitor cells (OPCs) exhibited the most substantial differences in both metabolic pathways and gene expression, indicative of their distinct origins. The comprehensive analysis identified the most altered metabolic pathways (MCPs) and genes across all cell types, which were further validated against TCGA and CGGA datasets for clinical relevance.
    UNASSIGNED: Crucially, the metabolic enzyme phosphodiesterase 8B (PDE8B) was found to be exclusively expressed and progressively downregulated in astrocytes and OPCs in higher-grade gliomas. This decreased expression identifies PDE8B as a metabolism-related oncogene in IDH-mutant glioma, marking its dual role as both a protective marker for glioma grading and prognosis and as a facilitator in glioma progression.
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  • 文章类型: Case Reports
    “寡星形细胞瘤”在世界卫生组织中枢神经系统肿瘤分类第四版修订版中消失,除非附加了“未指定(NOS)”。然而,具有星形细胞瘤/少突胶质细胞瘤双重特征的病例的组织病理学和遗传背景的报道很少。我们遇到了一名54岁的右额叶神经胶质瘤,在影像学和组织病理学检查中包括两个不同的部分:4级星形细胞瘤,IDH1-R132H,ATRX损耗,p53阳性和完整的1p/19q;和具有IDH1-R132H的少突胶质细胞瘤,完整的ATRX,p53阴性和部分缺失1p/19q。在复发时,组织病理学显示低度混合星形胶质细胞和少突胶质细胞特征:前者具有IDH1-R132H,ATRX损耗,p53阳性和完整的1p/19q,后者显示IDH1-R132H,完整的ATRX,p53阴性和1p/19q共缺失。在第二次复发时,组织病理学为星形细胞瘤4级,IDH1-R132H,ATRX损耗,p53阳性和完整的1p/19q。值得注意的是,复发时获得1p/19q共缺失,第二次复发时删除CDKN2A。这些发现表明了对肿瘤发生的见解:(1)具有两个不同谱系的神经胶质瘤可能混合产生“寡星形细胞瘤”;(2)在化疗期间可能会获得1p/19q共缺失和CDKN2A缺失。最终,星形胶质细胞和少突胶质细胞克隆可能在发育中共存,或者这两个谱系可能共享一个共同的起源细胞,以IDH1-R132H为共有分子特征。
    \"Oligoastrocytoma\" disappeared as of the revised fourth edition of the World Health Organization Classification of Tumours of the Central Nervous System, except where appended with \"not otherwise specified (NOS)\". However, histopathological and genetic backgrounds of cases with dual features of astrocytoma/oligodendroglioma have been sparsely reported. We encountered a 54-year-old man with right frontal glioma comprising two distinct parts on imaging and histopathological examination: grade 4 astrocytoma with IDH1-R132H, ATRX loss, p53-positivity and intact 1p/19q; and oligodendroglioma with IDH1-R132H, intact ATRX, p53-negativity and partially deleted 1p/19q. At recurrence, histopathology showed low-grade mixed astrocytic and oligodendroglial features: the former with IDH1-R132H, ATRX loss, p53-positivity and intact 1p/19q and the latter showing IDH1-R132H, intact ATRX, p53-negativity and 1p/19q codeletion. At second recurrence, histopathology was astrocytoma grade 4 with IDH1-R132H, ATRX loss, p53-positivity and intact 1p/19q. Notably, 1p/19q codeletion was acquired at recurrence and CDKN2A was deleted at second recurrence. These findings suggest insights into tumorigenesis: (1) gliomas with two distinct lineages might mix to produce \"oligoastrocytoma\"; and (2) 1p/19q codeletion and CDKN2A deletion might be acquired during chemo-radiotherapy. Ultimately, astrocytic and oligodendroglial clones might co-exist developmentally or these two lineages might share a common cell-of-origin, with IDH1-R132H as the shared molecular feature.
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  • 文章类型: Journal Article
    一只12年零3个月的雌性混合猫出现严重的腰椎疼痛。磁共振成像和尸检显示L3水平的脊髓有肿胀病变。组织学检查发现肿瘤组织中广泛的肿瘤细胞增殖和大量坏死。识别两种类型的肿瘤细胞。一种类型的肿瘤细胞是大细胞,其特征是圆形到多边形形状和丰富的嗜酸性细胞浆(称为“大细胞”)。其他肿瘤细胞很小,密集增殖,并有圆形到不规则的形状和缺乏嗜酸性细胞的细胞质(称为“小细胞”)。两种类型的细胞对少突胶质细胞转录因子2和SRY-box转录因子10均为阳性。胶质纤维酸性蛋白在大细胞中呈阳性,但在大多数小细胞中呈阴性。对Ki-67染色的肿瘤组织进行数字分析发现,总共有21.1%±6.5%的肿瘤细胞对Ki-67呈阳性。基于这些发现,我们诊断为脊髓恶性少星形细胞瘤。
    A 12-year and 3-month spayed female mixed cat was presented with severe lumbar pain. Magnetic resonance imaging and postmortem examination revealed a swollen lesion in the spinal cord at L3 level. Histologic examination identified extensive neoplastic cell proliferation with massive necrosis in the tumor tissue. Two types of neoplastic cells were recognized. One type of neoplastic cells were large cells characterized by round to polygonal shape and abundant eosinophilic cytoplasm (referred to as \"large cells\"). The other neoplastic cells were small, densely proliferated, and had round to irregular shape and scant eosinophilic cytoplasm (referred to as \"small cells\"). Both types of cells were positive for oligodendrocyte transcription factor 2 and SRY-box transcription factor 10. Glial fibrillary acidic protein was positive in large cells but negative in most small cells. Digital analysis for Ki-67-stained tumor tissues found that total 21.1% ± 6.5% of tumor cells were positive for Ki-67. Based on these findings, we diagnosed malignant oligoastrocytoma in the spinal cord.
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  • 文章类型: Journal Article
    在2016年世界卫生组织(WHO)中枢神经系统肿瘤分类中,将染色体1p/19q共缺失作为少突胶质细胞瘤的必需诊断成分。我们试图评估指南前后少突胶质细胞瘤和少突星形细胞瘤患者中1p/19q共缺失的报告测试差异。
    国家癌症数据库(NCDB)查询了2011-2017年间经组织学证实的WHOII/III级少突胶质细胞瘤或少突星形细胞瘤患者。在指南之前(2011-2015年)和之后(2017年)计算了针对患者和医院水平因素进行1p/19q共缺失测试的调整后几率。还评估了根据报告的测试调整的接受辅助治疗(化疗和/或放疗)的可能性。
    总的来说,确定了6,404名患者。报告的1p/19q共缺失检测率从2011年的45.8%增加到2017年的59.8%。从2011-2015年,缺乏保险(OR0.77;95%CI0.62-0.97;p=0.025),较低的邮政编码教育程度(OR0.62;95%CI0.49-0.78;p<0.001),和东北部(OR0.68;95%CI0.57-0.82;p<0.001)或南部(OR0.62;95%CI0.49-0.79;p<0.001)设施地理区域与报告的检测呈负相关。2017年,黑人种族(OR0.49;95%CI0.26-0.91;p=0.024)和东北(OR0.50;95%CI0.30-0.84;p=0.009)或南部(OR0.42;95%CI0.22-0.78;p=0.007)地区与报告的测试呈负相关。已报告测试的患者更有可能接受辅助治疗(OR1.73;95%CI1.46-2.04;p<0.001)。
    尽管有2016年世卫组织指南,按地理区域报告的1p/19q共缺失测试的差异仍然存在,而种族/民族的新差异被发现,这可能会影响少突胶质细胞瘤和少突星形细胞瘤患者的管理。
    UNASSIGNED: A chromosomal 1p/19q codeletion was included as a required diagnostic component of oligodendrogliomas in the 2016 World Health Organization (WHO) classification of central nervous system tumors. We sought to evaluate disparities in reported testing for 1p/19q codeletion among oligodendroglioma and oligoastrocytoma patients before and after the guidelines.
    UNASSIGNED: The National Cancer Database (NCDB) was queried for patients with histologically-confirmed WHO grade II/III oligodendroglioma or oligoastrocytoma from 2011-2017. Adjusted odds of having a reported 1p/19q codeletion test for patient- and hospital-level factors were calculated before (2011-2015) and after (2017) the guidelines. The adjusted likelihood of receiving adjuvant treatment (chemotherapy and/or radiotherapy) based on reported testing was also evaluated.
    UNASSIGNED: Overall, 6,404 patients were identified. The reported 1p/19q codeletion testing rate increased from 45.8% in 2011 to 59.8% in 2017. From 2011-2015, lack of insurance (OR 0.77; 95% CI 0.62-0.97;p=0.025), lower zip code-level educational attainment (OR 0.62; 95% CI 0.49-0.78;p<0.001), and Northeast (OR 0.68; 95% CI 0.57-0.82;p<0.001) or Southern (OR 0.62; 95% CI 0.49-0.79;p<0.001) facility geographic region were negatively associated with reported testing. In 2017, Black race (OR 0.49; 95% CI 0.26-0.91;p=0.024) and Northeast (OR 0.50; 95% CI 0.30-0.84;p=0.009) or Southern (OR 0.42; 95% CI 0.22-0.78;p=0.007) region were negatively associated with reported testing. Patients with a reported test were more likely to receive adjuvant treatment (OR 1.73; 95% CI 1.46-2.04;p<0.001).
    UNASSIGNED: Despite the 2016 WHO guidelines, disparities in reported 1p/19q codeletion testing by geographic region persisted while new disparities in race/ethnicity were identified, which may influence oligodendroglioma and oligoastrocytoma patient management.
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  • 文章类型: Case Reports
    A case of a true dual-genotype IDH-mutant oligoastrocytoma with two different cell types within a single mass in a young woman is presented. Imaging findings of the left frontal infiltrating glioma predicted the two neoplastic components that were identified upon resection. Tissue examination demonstrated areas of tumor with contrasting histologic and molecular features, including specific IDH1, ATRX, TP53, TERT and CIC mutational profiles, consistent with oligodendroglioma and astrocytoma, respectively. The clinical and radiological course over 17 months from first diagnosis included three surgical resections with slow progression of the astrocytic component, and ultimately chemotherapy and radiation treatments were commenced. Reports of the clinical courses for these rare cases of dual-genotype oligoastrocytomas will inform therapy choices, to optimize benefit while minimizing side effects. The steadily increasing number of cases suggests that the neoplasm might be reconsidered as an official entity by the WHO.
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    文章类型: Case Reports
    Glioblastoma multiforme (GBM) is the most common and aggressive primary intracranial tumor. The tumor metastases outside the CNS are rare, so therapeutic experience with these types of tumors is limited. We present a case of a 20-year-old female with a history of left temporal anaplastic oligoastrocytoma, who was found to have biopsy proven metastases tothe lung. In April 2014, the patient presented with intermittent headache for 4 months. Physical examinations confirmed no obvious abnormalities. Computed tomography (CT) scan and magnetic resonance image (MRI) scan of the brain showed a large mass with ring-like enhancement in left temporal lobe. She underwent craniotomy to resect the intracranial tumor. Thepathological investigation showed the lesion to be a classical anaplastic oligoastrocytoma with focal necrosis and a Ki-67 labeling index of 10-30%. After operation, the patient received radiotherapy and chemotherapy. Two years later, the patient readmitted due to chest discomfort, a chest X-ray and CT showed a mass in the right lung. The lesion was confirmed to be a metastatic malignant glioma viapulmonary bronchoscopy biopsy. In conclusion, as the life expectancy is gradually increasing for GBM patients with newer therapies, the incidence of extracranial metastases may increase and this rare phenomenon may become more common and clinically relevant.
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  • 文章类型: Journal Article
    背景:超难治性癫痫持续状态(SRSE)是一种危及生命的神经系统急症,定义为“癫痫持续状态(SE),在麻醉开始后持续24小时或更长时间,包括在麻醉减少或停止时SE复发的情况,这发生在10%至15%的SE患者中,很少通过手术解决。
    方法:一名20岁的男性患者患有SRSE,有很长的左顶枕骨少叶星形细胞瘤病史,因惊厥性SE而入院,并在皮质脑电图和神经导航引导下接受了病灶切除术,以治疗局部肿瘤复发。病理诊断为少星形细胞瘤。SRSE中止,患者完全康复,无任何功能缺陷。
    结论:应考虑在皮质脑电图和神经导航引导下的病灶切除术作为SRSE患者的治疗选择。
    BACKGROUND: Super-refractory status epilepticus (SRSE) is a life-threatening neurologic emergency defined as \"status epilepticus (SE) that continues 24 hours or more after the onset of anesthesia, including those cases in which the SE recurs on the reduction or withdrawal of anesthesia,\" which occurs in 10% to 15% of patients with SE and rarely has been resolved surgically.
    METHODS: A 20-year-old man with SRSE and a long history of left parieto-occipital oligoastrocytoma was admitted for convulsive SE that became SRSE and underwent lesionectomy guided by electrocorticography and neuronavigation for local tumor recurrence. Histopathologic diagnosis was oligoastrocytoma. SRSE was aborted and the patient recovered fully without any functional deficits.
    CONCLUSIONS: The lesionectomy guided by electrocorticography and neuronavigation should be considered as a treatment option for patients with SRSE.
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  • 文章类型: Case Reports
    颅外转移是间变性少星形细胞瘤的罕见现象。当患者经过综合治疗后进展,通常没有有效的治疗方法。基因检测技术的快速发展使得胶质瘤的精准治疗成为可能。
    2014年,一名22岁女孩首次被诊断为间变性少粒星形细胞瘤,WHOIII-IV级,2016年在多个神经外病变的放化疗后进展迅速。预计她的寿命较短,并应用了下一代测序(NGS)。
    第一次NGS报告了BRAF(V600E)的突变,口服维罗非尼稳定了她的疾病6个月。Vemurafenib进展后,第二次NGS报告了PIK3CA。口服依维莫司和维罗非尼使她的疾病又稳定了6个月。然而,患者于2018年2月24日因疾病快速进展死亡.
    我们用维罗非尼和依维莫司成功治疗了BRAFV600E突变的多脑外转移瘤。对于没有明确有效治疗方案的晚期患者,NGS可以作为一种有效的选择。
    Extracranial metastasis is a rare phenomenon of anaplastic oligoastrocytoma. When patients progress after comprehensive treatment, there is often no effective treatment. Rapid development of gene detection technology makes precision treatment of glioma possible.
    A 22-year-old girl was firstly diagnosed with anaplastic oligoastrocytoma WHO grade III-IV in 2014, and progressed rapidly after chemoradiotherapy in multiple extraneural lesions in 2016. She was expected to have a short life and Next-Generation Sequencing (NGS) was applied.
    Mutation of BRAF (V600E) was reported by 1st NGS and oral vemurafenib stabilized her disease for 6 months. PIK3CA was reported by 2nd NGS after her progression of vemurafenib. The oral administration of everolimus together with vemurafenib stabilized her disease for another 6 months. However, the patient died due to the rapid progression of the disease on 24 February 2018.
    We successfully treated a BRAF V600E-mutated anaplastic oligoastrocytoma with multiple extraneural metastases with vemurafenib and everolimus. For late-staged patients who have no clear and effective treatment plan, NGS may serve as an effective option.
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  • 文章类型: Journal Article
    BACKGROUND: Low-grade gliomas (LGG) are slow-growing primary brain tumors that typically affect young adults. Advanced age is widely recognized as a poor prognostic factor in LGG. The impact of age on postoperative outcome in this patient group has not been systemically studied.
    METHODS: We performed a nationwide register-based study with data from the Swedish Brain Tumor Registry (SBTR) for all adults diagnosed with a supratentorial LGG (WHO grade II astrocytoma, oligoastrocytoma, or oligodendroglioma) during 2005-2015. Patient- and tumor-related characteristics, postoperative complications, and survival were compared between three different age groups (18-39 years, 40-59 years, and ≥60 years).
    RESULTS: We identified 548 patients; 204 patients (37.2%) aged 18-39 years, 227 patients (41.4%) aged 40-59 years, and 117 patients (21.4%) ≥60 years of age. Unfavorable preoperative prognostic factors (eg, functional status and neurological deficit) were more common with increased age (P < .001). In addition, overall survival was significantly impaired in those 60 years and above (P < .001). We observed a clear dose-response for age with separation of survival curves at 50 years. Biopsy was more common in patients ≥60 years (P < .001). Subgroup analysis of patients with resection revealed a higher amount of postoperative neurological deficits in older patients (P = .029).
    CONCLUSIONS: In general, older patients with LGG have several unfavorable prognostic factors compared with younger patients but seem to tolerate surgery in a comparable fashion. However, more neurological deficits were observed following resections in elderly. Our data further support a cutoff at 50 years rather than 40 years for selection of high-risk patients.
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