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diffuse intrinsic pontine glioma:current insights and future directions

Chinese Neurosurgical Journal 2021年第4期7卷 289-299页

作者： Dilakshan Srikanthan Michael S.Taccone Randy Van Ommeren Joji Ishida Stacey L.Krumholtz James T.Rutka Cell Biology Program The Hospital for Sick Children686 Bay StTorontoON M5G 0A4Canada The Arthur and Sonia Labatt Brain Tumour Research Centre The Hospital for Sick Children686 Bay StTorontoON M5G 0A4Canada Department of Laboratory Medicine and Pathobiology University of TorontoTorontoONCanada Division of Neurosurgery Department of SurgeryThe Ottawa HospitalOttawaONCanada Developmental and Stem Cell Biology Program The Hospital for Sick Children686 Bay StTorontoON M5G 0A4Canada Institute of Medical Sciences University of TorontoTorontoONCanada Division of Neurosurgery Department of SurgeryThe Hospital for Sick ChildrenSuite 1503555University AvenueTorontoON M5G 1X8Canada

diffuse intrinsic pontine glioma(DIPG)is a lethal pediatric brain tumor and the leading cause of brain tumor–related death in *** several clinical trials over the past few decades have led to no significant improvements in outcome,the current standard of care remains fractionated focal *** to the recent increase in stereotactic biopsies,tumor tissue availabilities have enabled our advancement of the genomic and molecular characterization of this lethal *** groups have identified key histone gene mutations,genetic drivers,and methylation changes in DIPG,providing us with new insights into DIPG ***,there has been increased development of in vitro and in vivo models of DIPG which have the capacity to unveil novel therapies and strategies for drug *** review outlines the clinical characteristics,genetic landscape,models,and current treatments and hopes to shed light on novel therapeutic avenues and challenges that remain.

关键词： diffuse intrinsic pontine glioma Molecular genetics Neuro-oncology Therapeutics Disease models Pediatrics Neurosurgery

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Adult diffuse intrinsic pontine glioma:Clinical,Radiological,Pathological,Molecular Features,and Treatments of 96 patients

Adult Diffuse Intrinsic Pontine Glioma:Clinical,Radiological...

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第十六届中国医师协会神经外科医师年会

作者： Yi Wang Changcun Pan Mingguo Xie Pengcheng Zuo Xiaoou Li Guocan Gu Tian Li Zhuang Jiang Zhen Wu Junting Zhang Liwei Zhang Department of Neurosurgery Beijing Tiantan HospitalCapital Medical University Department of Neurosurgery Sanbo Brain Hospital China National Clinical Research Center for Neurological Diseases

*** its pediatric counterpart,adult diffuse intrinsic pontine glioma(DIPG) remains largely *** study examined the clinical,radiological,pathological,molecular,and clinical aspects of 96 adult *** National Brain Tumor Registry of China(2013/04-2019/12) was used to collect data on radiologically diagnosed adult DIPG *** analysis was conducted using the Kaplan–Meier Curve,univariate and multivariate Cox *** Chi-square test/Wilcoxon rank-sum test and multivariable logistic regression were used to examine the characteristics of long-term survival.P<0.05 was deemed *** median age at onset was 33.5 years,and the median duration of symptoms was 4.5 months.H3K27M and IDH1 mutations were 37.2% and 26.5%,*** patients had a median overall survival(OS) of 19.5 months,with 1-,2-and 3-year survival rates of 67.0%,42.8%,and 36.0%,*** patients with H3K27M showed a poorer prognosis than IDH-mutant patients(P<0.001) and H3K27M(-)/IDH-WT patients(P=0.002).The median OS of patients treated with RT+CT,RT/CT,and NO was 23.0 months,17.0 months,13.4 months respectively(P=0.097).The median OS of H3K27M patients who received the treatments and those who did not get treatments was 13.8 months and 7.5 months respectively(P=0.016).Short-term survival was related with cranial nerve palsy at diagnosis(OR,9.788;95%CI,2.242-42.731;P=0.002) and contrast enhancement in MRI(OR,7.741;95%CI,1.335-44.874;P=0.022).In patients with and without a pathological diagnosis,H3K27M(HR 8.084;95% CI 2.493-26.216;P<0.001) and contrast enhancement on MRI(HR 12.246;95% CI 2.356-63.650;P=0.003) both imparted a worse prognosis,*** were the predictive factor for H3K27M patients(P=0.038),whereas contrast enhancement in MRI was the prognostic factor for H3K27M(-) group(P=0.038).*** is recommended that all adult DIPG patients get genomic testing for H3K27M and IDH *** the

关键词： adult diffuse intrinsic pontine glioma prognosis H3K27M IDH mutation

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Exploratory therapy for brainstem gliomas

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Cancer Advances 2023年第19期6卷 1-10页

作者： Michelot Michel Miguel Tusa Lavieri Maria Paula Aguilera-Pen Brandon Lucke-Wold Department of Neurosurgery University of FloridaGainesville 32608USA Department of Neurosurgery Weill Cornell Medical CollegeNew York 10065USA Department of Neurosurgery Cedars-Sinai Medical CenterLos Angeles 90048USA

Brainstem gliomas comprise both slow-growing and highly aggressive tumors,the latter carrying a dismal prognosis of approximately 10 months in *** their common locations along the brainstem,they are often not amenable to surgical *** are currently a host of exploratory therapies under investigation ranging from immunotherapy,small molecular inhibitors,epigenetic-modifying agents,and radiation protocols to combat these difficult-to-treat *** discoveries highlighting the role of H3 histone mutations in diffuse midline glioma oncogenesis have yielded a variety of new targetable antigens and aberrant signaling *** many of these studies have shown promise in terms of inhibiting tumor growth and disease progression,results to date have been modest in their ability to translate into meaningful clinical *** review will serve as an updated report on the current state of literature concerning pre-clinical and clinical therapies being investigated for brainstem *** addition,this review will serve as a guide for clinicians as we review the evolving nomenclature of brainstem gliomas,commonly presenting symptoms,diagnostic tools,and standard therapies.

关键词： brainstem glioma diffuse midline glioma diffuse intrinsic pontine glioma H3K27M

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