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Epidemiologic and histologic characteristics of CNS lesions: a 20-year experience of a tertiary center in Lebanon. 中枢神经系统病变的流行病学和组织学特征:黎巴嫩三级中心20年的经验。
Q1 Medicine Pub Date : 2020-06-01 Epub Date: 2020-06-30 DOI: 10.2217/cns-2020-0001
Roland Eid, Stephanie Hage, Ingrid Antonios, Rita Moussa, Makram Khoury, Fady Ghassan Haddad, Hampig Raphael Kourie, Carole Kesrouani, Claude Ghorra, Gerard Abadjian, Joseph Kattan

Aim: Report the epidemiologic and histologic characteristics of CNS lesions in the Lebanese population. Methods: We conducted a retrospective study evaluating 2025 CNS lesions diagnosed between 1998 and 2017 in the pathology laboratory of a Lebanese tertiary center. Results: 52.2% of patients were men with a median age of 50 years. The most frequent symptoms were epilepsy (22.5%), headache (20.6%) and motor impairment (19.9%). 90.7% of tumors were primary. Lung (35.6%) and breast (16.5%) were the most frequent primaries of metastases. 46.2% of primary CNS tumors were glial, predominantly astrocytic (56.4%), and (42.5%) were nonglial, predominantly meningeal tumors (58%). Conclusion: Compared with Western literature, the Lebanese population is characterized by a younger age of onset of brain tumors, a lower rate of meningiomas and a higher rate of gliomas.

目的:报道黎巴嫩人群中中枢神经系统病变的流行病学和组织学特征。方法:我们进行了一项回顾性研究,评估了1998年至2017年在黎巴嫩三级中心病理实验室诊断的2025例中枢神经系统病变。结果:52.2%的患者为男性,中位年龄50岁。最常见的症状是癫痫(22.5%)、头痛(20.6%)和运动障碍(19.9%)。90.7%为原发肿瘤。肺(35.6%)和乳腺(16.5%)是最常见的转移原发灶。46.2%的原发性中枢神经系统肿瘤为神经胶质细胞,以星形细胞为主(56.4%),42.5%为非神经胶质细胞,以脑膜肿瘤为主(58%)。结论:与西方文献相比,黎巴嫩人群的特点是脑肿瘤发病年龄更年轻,脑膜瘤发病率较低,胶质瘤发病率较高。
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引用次数: 2
Disparities in patient enrollment on glioblastoma clinical trials. 胶质母细胞瘤临床试验患者入组差异。
Q1 Medicine Pub Date : 2020-06-01 Epub Date: 2020-06-30 DOI: 10.2217/cns-2020-0008
Yang Liu, Andrea Wasilewski, Nimish A Mohile

Aim: To determine if enrollment on glioblastoma (GBM) interventional clinical trials (ICTs) in the USA is representative of the population. Materials & methods: We queried ClinicalTrials.gov for all ICTs in GBM from 1994 to 2019. Demographics were obtained from ClinicalTrials.gov or the trial publication and compared with population data from Central Brain Tumor Registry of the United States. Results: In total, 10617 GBM patients were enrolled in 118 adult ICTs: median age was 54.0 (10.05 years younger than Central Brain Tumor Registry of the United States). Age was most discrepant in recurrent tumors, nonrandomized trials and consortium studies. Median age improved from 52.0 to 59.5 over 25 years. Women represented 37.5% of subjects. Conclusion: GBM ICTs under-represent older patients but representation of women reflects the population. ICTs need to be designed to better represent the population.

目的:确定美国胶质母细胞瘤(GBM)介入性临床试验(ict)的入组是否具有代表性。材料与方法:我们在ClinicalTrials.gov网站上查询1994 - 2019年GBM患者的所有信息通信技术。从ClinicalTrials.gov或试验出版物中获得人口统计数据,并与美国中央脑肿瘤登记处的人口数据进行比较。结果:118个成人ict共纳入10617例GBM患者,中位年龄为54.0岁(比美国中央脑肿瘤登记处年轻10.05岁)。在复发肿瘤、非随机试验和联合研究中,年龄差异最大。25年间,中位年龄从52.0岁提高到59.5岁。女性占受试者的37.5%。结论:GBM患者中老年患者的比例不足,但女性的比例反映了人群的情况。信息通信技术的设计需要更好地代表人口。
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引用次数: 3
Ibrutinib in primary central nervous system diffuse large B-cell lymphoma. 依鲁替尼治疗原发性中枢神经系统弥漫性大b细胞淋巴瘤。
Q1 Medicine Pub Date : 2020-03-01 Epub Date: 2020-03-06 DOI: 10.2217/cns-2019-0022
Justin T Low, Katherine B Peters

The standard regimen for the treatment of newly diagnosed primary CNS lymphoma (PCNSL) remains regimens that contain high-dose methotrexate (MTX). While these regimens can provide control for some patients, there is a dearth of options for the treatment of patients with PCNSL who cannot tolerate MTX-containing regimens, or whose cancers are refractory to MTX. In this article, we review a promising new option; ibrutinib, a Bruton tyrosine kinase inhibitor, for patients with relapsed and refractory PCNSL.

治疗新诊断的原发性中枢神经系统淋巴瘤(PCNSL)的标准方案仍然是含有高剂量甲氨蝶呤(MTX)的方案。虽然这些方案可以为一些患者提供控制,但对于不能耐受含甲氨蝶呤方案或其癌症对甲氨蝶呤难治性的PCNSL患者,缺乏治疗选择。在本文中,我们回顾了一个有前途的新选项;ibrutinib,一种布鲁顿酪氨酸激酶抑制剂,用于复发和难治性PCNSL患者。
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引用次数: 4
Welcome to Volume 9 of CNS Oncology. 欢迎来到《中枢神经系统肿瘤学》第九卷。
Q1 Medicine Pub Date : 2020-03-01 Epub Date: 2020-03-02 DOI: 10.2217/cns-2019-0023
Jennifer Straiton
Content highlights Our most-read article of the year comes from Ugonma Chukwueke and Patrick Wen, both of the Dana-Farber Cancer Institute (MA, USA), and reviews the recommendations of the Response Assessment in Neuro-Oncology (RANO) working groups [1]. Distinct RANO groups have been set up for different tumor and treatment types, each group aiming to develop relevant and reliable criteria that can be implemented by clinicians. This review discusses how the recommendations given by the RANO groups may be applied to the care of patients with different brain tumor types and suggests what is still needed for the guidelines to fully reflect the complex nature of each tumor. For anyone interested in neuro-oncology and CNS oncology management, this is a key read. From Issue 1 of this volume, the article has been cited twice and is already the most-read article ever published in CNS Oncology. Next up, we have a preliminary communication written by David E Piccioni et al. (University of California San Diego, CA, USA), describing their study evaluating whether circulating tumor DNA could facilitate genomic interrogation in patients with primary brain tumors [2]. Contrary to previous studies in this area, the group found that 50% of patients included in the study had detectable circulating tumor DNA, making it a clinically viable option for analysis when identifying genomics-based therapy options. Another 2019 article that has hit our most-read list is an editorial that evaluates the benefits of whole-brain radiation therapy versus stereotactic radiosurgery for the treatment of brain metastases [3]. Written by a group from the City of Hope National Medical Center (CA, USA), the article discusses the shift in clinical practice from using whole-brain radiation therapy to stereotactic radiosurgery and looks to the future of radiation therapy for brain metastases. Finally, we have a set of three articles each discussing the Nativis Voyager R ©, an investigational therapeutic medical device that is being developed for the treatment of both pediatric and adult glioblastoma. With feasibility studies in both American [4] and Australian [5] populations, the researchers found no adverse events that could lead to the discontinuation of the device, and it demonstrated a benign safety profile. However, as stated in an editorial on the device by Victor Levin (MD Anderson, TX, USA), further studies are needed to determine the full impact of the therapy on overall survival of patients with glioblastoma [6].
{"title":"Welcome to Volume 9 of <i>CNS Oncology</i>.","authors":"Jennifer Straiton","doi":"10.2217/cns-2019-0023","DOIUrl":"https://doi.org/10.2217/cns-2019-0023","url":null,"abstract":"Content highlights Our most-read article of the year comes from Ugonma Chukwueke and Patrick Wen, both of the Dana-Farber Cancer Institute (MA, USA), and reviews the recommendations of the Response Assessment in Neuro-Oncology (RANO) working groups [1]. Distinct RANO groups have been set up for different tumor and treatment types, each group aiming to develop relevant and reliable criteria that can be implemented by clinicians. This review discusses how the recommendations given by the RANO groups may be applied to the care of patients with different brain tumor types and suggests what is still needed for the guidelines to fully reflect the complex nature of each tumor. For anyone interested in neuro-oncology and CNS oncology management, this is a key read. From Issue 1 of this volume, the article has been cited twice and is already the most-read article ever published in CNS Oncology. Next up, we have a preliminary communication written by David E Piccioni et al. (University of California San Diego, CA, USA), describing their study evaluating whether circulating tumor DNA could facilitate genomic interrogation in patients with primary brain tumors [2]. Contrary to previous studies in this area, the group found that 50% of patients included in the study had detectable circulating tumor DNA, making it a clinically viable option for analysis when identifying genomics-based therapy options. Another 2019 article that has hit our most-read list is an editorial that evaluates the benefits of whole-brain radiation therapy versus stereotactic radiosurgery for the treatment of brain metastases [3]. Written by a group from the City of Hope National Medical Center (CA, USA), the article discusses the shift in clinical practice from using whole-brain radiation therapy to stereotactic radiosurgery and looks to the future of radiation therapy for brain metastases. Finally, we have a set of three articles each discussing the Nativis Voyager R ©, an investigational therapeutic medical device that is being developed for the treatment of both pediatric and adult glioblastoma. With feasibility studies in both American [4] and Australian [5] populations, the researchers found no adverse events that could lead to the discontinuation of the device, and it demonstrated a benign safety profile. However, as stated in an editorial on the device by Victor Levin (MD Anderson, TX, USA), further studies are needed to determine the full impact of the therapy on overall survival of patients with glioblastoma [6].","PeriodicalId":10469,"journal":{"name":"CNS Oncology","volume":"9 1","pages":"CNS49"},"PeriodicalIF":0.0,"publicationDate":"2020-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.2217/cns-2019-0023","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"37694637","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Clinical response to pazopanib in a patient with endolymphatic sac tumor not associated with von Hippel-Lindau syndrome. 非希佩尔-林道综合征的内淋巴囊瘤患者对帕唑帕尼的临床反应
Q1 Medicine Pub Date : 2020-03-01 Epub Date: 2020-03-06 DOI: 10.2217/cns-2019-0019
Thomas Nelson, Jethro Hu, Serguei Bannykh, Xuemo Fan, Jeremy Rudnick, Eric Vail

Endolymphatic sac tumors (ELSTs) are rare, locally invasive, vascular tumors of the temporal bone. These lesions are associated with von Hippel-Lindau syndrome but may arise sporadically. Early surgical intervention is recommended to prevent permanent neurologic deficits; however, many ELSTs are unresectable or are subtotally resected due to neurovascular compromise. Chemotherapeutic salvage therapy in trials of neoplasms of associated syndromes has targeted angiogenesis with variable response. We present the case of a sporadic ELST, previously minimally responsive to bevacizumab, treated with pazopanib, a multi-kinase inhibitor and antiangiogenic, with good response. Cases such as our patient may demonstrate the utility of novel antiangiogenics in the treatment of these rare neoplasms, particularly when the tumor is unresectable or necessitates subtotal resection.

内淋巴囊肿瘤是一种罕见的局部侵袭性颞骨血管肿瘤。这些病变与希佩尔-林道综合征有关,但也可能零星发生。建议早期手术干预以防止永久性神经功能缺损;然而,许多内皮细胞瘤无法切除或因神经血管受损而被部分切除。在肿瘤相关综合征的试验中,化疗挽救治疗有针对性的血管生成和不同的反应。我们提出了一个散发性ELST病例,以前对贝伐单抗反应最低,用pazopanib治疗,一种多激酶抑制剂和抗血管生成,具有良好的反应。像我们的病人这样的病例可能证明了新型抗血管生成药物在治疗这些罕见肿瘤中的效用,特别是当肿瘤无法切除或需要次全切除时。
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引用次数: 2
Challenges of targeting BRAF V600E mutations in adult primary brain tumor patients: a report of two cases. 成人原发性脑肿瘤患者靶向BRAF V600E突变的挑战:两例报告。
Q1 Medicine Pub Date : 2019-12-01 Epub Date: 2019-12-10 DOI: 10.2217/cns-2019-0018
Matthew Smith-Cohn, Christian Davidson, Howard Colman, Adam L Cohen

Aim: Therapeutic targeting of BRAF alterations in primary brain tumor patients has demonstrated clinical activity in case reports and early trials; however, there is limited high-level evidence of the efficacy. Patients & results: Targeting BRAF V600E mutations with concurrent dabrafenib and trametinib in anaplastic pleomorphic xanthoastrocytoma resulted in a transient radiographic and clinical response and no therapeutic benefit in a patient with an epithelioid glioblastoma. Conclusion:BRAF/MEK inhibition did not produce a durable treatment effect in glioblastoma or pleomorphic xanthoastrocytoma with BRAF V600E alterations. Heterogenicity of related cases in the literature makes an evaluation of efficacy BRAF targeting therapies in gliomas difficult and requires additional investigation.

目的:针对原发性脑肿瘤患者BRAF改变的治疗靶向性研究已在病例报告和早期试验中证明具有临床活性;然而,关于疗效的高级证据有限。患者和结果:在间变性多形性黄星形细胞瘤中,靶向BRAF V600E突变,同时使用达布非尼和曲美替尼,导致短暂的放射学和临床反应,对上皮样胶质母细胞瘤患者没有治疗益处。结论:BRAF/MEK抑制对伴有BRAF V600E改变的胶质母细胞瘤或多形性黄星形细胞瘤没有持久的治疗作用。文献中相关病例的异质性使得评估BRAF靶向治疗胶质瘤的疗效变得困难,需要进一步的研究。
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引用次数: 14
Utility of flouro-deoxy-glucose positron emission tomography/computed tomography in the diagnostic and staging evaluation of patients with primary CNS lymphoma 氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描在原发性中枢神经系统淋巴瘤患者的诊断和分期评估中的应用
Q1 Medicine Pub Date : 2019-11-29 DOI: 10.2217/cns-2019-0016
Meetakshi Gupta, T. Gupta, N. Purandare, V. Rangarajan, A. Puranik, A. Moiyadi, P. Shetty, S. Epari, A. Sahay, A. Mahajan, A. Janu, B. Bagal, H. Menon, S. Kannan, R. Krishnatry, G. Sastri, R. Jalali
Aim: To prospectively assess the clinical utility of pretreatment flouro-deoxy-glucose positron emission tomography/computed tomography (18F-FDG-PET/CT) in patients with primary central nervous system (CNS) lymphoma (PCNSL). Materials & methods: Patients with suspected/proven PCNSL underwent baseline whole-body 18F-FDG-PET/CT. Maximum standardized uptake value and tumor/normal tissue ratios were compared between CNS lymphoma and other histological diagnoses. Results: The mean maximum standardized uptake value (27.5 vs 18.2; p = 0.001) and mean tumor/normal tissue ratio (2.34 vs 1.53; p < 0.001) of CNS lymphoma was significantly higher than other histologic diagnoses. Five of 50 (10%) patients with biopsy-proven CNS lymphomas had pathologically increased FDG-uptake at extraneuraxial sites uncovering systemic lymphoma. Conclusion: Pretreatment whole-body 18F-FDG-PET/CT provides valuable complementary information in the diagnostic and staging evaluation of patients with PCNSL to guide therapeutic decision-making.
目的:前瞻性评价预处理氟脱氧葡萄糖正电子发射断层扫描/计算机断层扫描(18F-FDG-PET/CT)在原发性中枢神经系统淋巴瘤(PCNSL)患者中的临床应用价值。材料与方法:疑似/确诊PCNSL患者行基线全身18F-FDG-PET/CT检查。比较中枢神经系统淋巴瘤和其他组织学诊断的最大标准化摄取值和肿瘤/正常组织比率。结果:平均最大标准化摄取值(27.5 vs 18.2;P = 0.001),平均肿瘤/正常组织比值(2.34 vs 1.53;p < 0.001)的发病率明显高于其他病理诊断。50例活检证实的中枢神经系统淋巴瘤患者中有5例(10%)在病理上在轴外部位fdg摄取增加,发现全身性淋巴瘤。结论:预处理全身18F-FDG-PET/CT为PCNSL患者的诊断和分期评估提供了有价值的补充信息,指导治疗决策。
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引用次数: 14
The role of chemotherapy in the treatment of central neurocytoma 化疗在中枢神经细胞瘤治疗中的作用
Q1 Medicine Pub Date : 2019-11-01 DOI: 10.2217/cns-2019-0012
Margaret O Johnson, J. Kirkpatrick, Mallika P. Patel, A. Desjardins, D. Randazzo, H. Friedman, D. Ashley, K. Peters
Aim: Central neurocytoma (CN) is a rare WHO grade II central nervous system (CNS) tumor. This is an update on chemotherapeutic agents used in its treatment. Patients & methods: An institutional review board-approved, chart review of patients seen at our institution resulted in a single case treated with chemotherapy and is herein included. We proceeded with a comprehensive literature review. Results: We identified 18 citations, representing 39 cases of adult and pediatric CN treated with chemotherapy. With the addition of our single case, the total number of recurrent CN patients treated with temozolomide (TMZ) is nine. Conclusion: There exists marked heterogeneity in chemotherapy used to treat CN. TMZ is incorporated into treatment regimens in the setting of tumor recurrence: its role merits further study.
目的:中枢神经细胞瘤(CN)是一种罕见的WHOⅱ级中枢神经系统肿瘤。这是一个最新的化疗药物用于其治疗。患者和方法:经机构审查委员会批准,对在我们机构就诊的患者进行图表审查,结果是接受化疗的单个病例,并在此包括在内。我们进行了全面的文献综述。结果:我们确定了18条引文,代表了39例接受化疗的成人和儿童CN病例。加上我们的单一病例,使用替莫唑胺(TMZ)治疗的CN复发患者总数为9例。结论:CN的化疗方案存在明显的异质性。TMZ在肿瘤复发的情况下被纳入治疗方案,其作用值得进一步研究。
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引用次数: 10
Biological intratumoral therapy for the high-grade glioma part II: vector- and cell-based therapies and radioimmunotherapy 高级别胶质瘤的生物瘤内治疗第二部分:载体和细胞为基础的治疗和放射免疫治疗
Q1 Medicine Pub Date : 2019-11-01 DOI: 10.2217/cns-2019-0002
J. Loya, Charlie Zhang, Emily J Cox, A. Achrol, S. Kesari
Management of high-grade gliomas (HGGs) remains a complex challenge with an overall poor prognosis despite aggressive multimodal treatment. New translational research has focused on maximizing tumor cell eradication through improved tumor cell targeting while minimizing collateral systemic side effects. In particular, biological intratumoral therapies have been the focus of novel translational research efforts due to their inherent potential to be both dynamically adaptive and target specific. This two part review will provide an overview of biological intratumoral therapies that have been evaluated in human clinical trials in HGGs, and summarize key advances and remaining challenges in the development of these therapies as a potential new paradigm in the management of HGGs. Part II discusses vector-based therapies, cell-based therapies and radioimmunotherapy.
高级别胶质瘤(HGGs)的管理仍然是一个复杂的挑战,尽管积极的多模式治疗总体预后较差。新的转化研究侧重于通过改进肿瘤细胞靶向来最大限度地根除肿瘤细胞,同时最大限度地减少附带的全身副作用。特别是,由于生物肿瘤内治疗具有动态适应性和靶向特异性的内在潜力,因此一直是新型转化研究的重点。这两部分的综述将提供已经在HGGs人体临床试验中评估的生物肿瘤内治疗的概述,并总结这些治疗作为HGGs管理的潜在新范式的关键进展和仍然存在的挑战。第二部分讨论了基于载体的治疗、基于细胞的治疗和放射免疫治疗。
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引用次数: 4
Biological intratumoral therapy for the high-grade glioma part I: intratumoral delivery and immunotoxins 高级别胶质瘤的生物瘤内治疗第一部分:瘤内输送和免疫毒素
Q1 Medicine Pub Date : 2019-11-01 DOI: 10.2217/cns-2019-0001
J. Loya, Charlie Zhang, Emily J Cox, A. Achrol, S. Kesari
Management of high-grade gliomas remains a complex challenge. Standard of care consists of microsurgical resection, chemotherapy and radiation, but despite these aggressive multimodality therapies the overall prognosis remains poor. A major focus of ongoing translational research studies is to develop novel therapeutic strategies that can maximize tumor cell eradication while minimizing collateral side effects. Particularly, biological intratumoral therapies have been the focus of new translational research efforts due to their inherent potential to be both dynamically adaptive and target specific. This two-part review will provide an overview of biological intratumoral therapies and summarize key advances and remaining challenges in intratumoral biological therapies for high-grade glioma. Part I focuses on discussion of the concepts of intratumoral delivery and immunotoxin therapies.
高级别胶质瘤的治疗仍然是一个复杂的挑战。标准治疗包括显微外科手术切除、化疗和放疗,但尽管这些积极的多模式治疗,总体预后仍然很差。正在进行的转化研究的一个主要焦点是开发新的治疗策略,可以最大限度地根除肿瘤细胞,同时最大限度地减少副作用。特别是,由于生物肿瘤内治疗具有动态适应性和靶向特异性的内在潜力,因此一直是新的转化研究的焦点。这两部分的综述将提供生物肿瘤内治疗的概述,并总结高级别胶质瘤的肿瘤内生物治疗的关键进展和仍然存在的挑战。第一部分着重讨论肿瘤内递送和免疫毒素治疗的概念。
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引用次数: 5
期刊
CNS Oncology
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