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Brainstem glioma

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https://www.readbyqxmd.com/read/28792659/molecular-alterations-in-pediatric-brainstem-gliomas
#1
Mikaela Porkholm, Anna Raunio, Reetta Vainionpää, Tarja Salonen, Juha Hernesniemi, Leena Valanne, Jarno Satopää, Atte Karppinen, Minna Oinas, Olli Tynninen, Virve Pentikäinen, Sanna-Maria Kivivuori
BACKGROUND: Diffuse intrinsic pontine gliomas (DIPGs) have a dismal prognosis. Previously, diagnosis was based on a typical clinical presentation and magnetic resonance imaging findings. After the start of the era of biopsies, DIPGs bearing H3 K27 mutations have been reclassified into a novel entity, diffuse midline glioma, based on the presence of this molecular alteration. However, it is not well established how clinically diagnosed DIPG overlap with H3 K27-mutated diffuse midline gliomas, and whether rare long-term survivors also belong to this group...
August 9, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28790919/potential-new-therapies-for-pediatric-diffuse-intrinsic-pontine-glioma
#2
REVIEW
Wenyong Long, Yang Yi, Shen Chen, Qi Cao, Wei Zhao, Qing Liu
Diffuse intrinsic pontine glioma (DIPG) is an extensively invasive malignancy with infiltration into other regions of the brainstem. Although large numbers of specific targeted therapies have been tested, no significant progress has been made in treating these high-grade gliomas. Therefore, the identification of new therapeutic approaches is of great importance for the development of more effective treatments. This article reviews the conventional therapies and new potential therapeutic approaches for DIPG, including epigenetic therapy, immunotherapy, and the combination of stem cells with nanoparticle delivery systems...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28775236/patient-derived-dipg-cells-preserve-stem-like-characteristics-and-generate-orthotopic-tumors
#3
Cheng Xu, Xiaoqing Liu, Yibo Geng, Qingran Bai, Changcun Pan, Yu Sun, Xin Chen, Hai Yu, Yuliang Wu, Peng Zhang, Wenhao Wu, Yu Wang, Zhen Wu, Juntin Zhang, Zhaohui Wang, Rui Yang, Jenna Lewis, Darell Bigner, Fangping Zhao, Yiping He, Hai Yan, Qin Shen, Liwei Zhang
Diffuse intrinsic pontine glioma (DIPG) is a devastating brain tumor, with a median survival of less than one year. Due to enormous difficulties in the acquisition of DIPG specimens and the sophisticated technique required to perform brainstem orthotopic injection, only a handful of DIPG pre-clinical models are available. In this study, we successfully established eight patient-derived DIPG cell lines, mostly derived from treatment-naïve surgery or biopsy specimens. These patient-derived cell lines can be stably passaged in serum-free neural stem cell media and displayed distinct morphologies, growth rates and chromosome abnormalities...
July 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28775233/genetic-and-immune-features-of-resectable-malignant-brainstem-gliomas
#4
Yang Zhang, Changcun Pan, Junmei Wang, Jingli Cao, Yuhan Liu, Yajie Wang, Liwei Zhang
We surveyed common genetic mutations (IDH1, H3F3A, PPM1D, and TP53) and immune features (PD-L1 expression and CD8+ T cell tumor infiltration) in a series of 62 malignant brainstem gliomas that were resected via microsurgery. IDH1 mutations were mutually exclusive with H3F3A mutations. IDH1 mutations appeared only in adults and occurred more frequently in tumors larger than 10cm3 (8/29 vs 1/32, Fisher's exact test, p=0.010). H3F3A mutations occurred more frequently in children and adolescent patients (19/24 vs 18/38, chi-square test, p=0...
July 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28766000/-pediatric-brain-tumors
#5
REVIEW
W Reith, S Bodea, R Mühl-Benninghaus
Brain tumors differ between children and adults both in histology and localization. Malignant gliomas and meningiomas predominate in adults while medulloblastomas and low-grade astrocytomas are the most frequent brain tumors in children. More than one half (50-70%) of pediatric brain tumors have an infratentorial location but only approximately 30% in adults. Brain tumors can be recognized in sonography, cranial computed tomography (CCT) and magnetic resonance imaging (MRI) by their space-consuming character and by their divergent density and intensity in comparison to normal brain parenchyma...
August 1, 2017: Der Radiologe
https://www.readbyqxmd.com/read/28753090/brainstem-angiocentric-glioma-report-of-2-cases
#6
Kristin J Weaver, Lexi M Crawford, Jeffrey A Bennett, Marie L Rivera-Zengotita, David W Pincus
Angiocentric glioma is a rare tumor that was recognized by the WHO Classification of Tumours of the Central Nervous System as a distinct clinicopathological entity in 2007. Since this initial description, the vast majority of cases of angiocentric glioma reported in the literature have involved tumors of the cerebral hemispheres. To date, only 1 case of angiocentric glioma arising from the posterior midbrain has been reported. The authors present the cases of 2 pediatric patients who were found to have brainstem angiocentric gliomas...
July 28, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28711290/biopsies-of-pediatric-brainstem-lesions-display-low-morbidity-but-strong-impact-on-further-treatment-decisions
#7
Johanna Quick-Weller, Stephanie Tritt, Bedjan Behmanesh, Michel Mittelbronn, Andrea Spyrantis, Nazife Dinc, Lutz Weise, Volker Seifert, Gerhard Marquardt, Thomas M Freiman
OBJECTIVE: The course of malignant brain stem gliomas in childhood is rarely positive. Because of limited therapeutic options and potentially hazardous biopsies oncologist often relay on MRI diagnoses only for further therapy decisions. In this study we show that brain stem biopsies display a low morbidity rate and neuropathological assessment has a considerable impact on further treatment decision. METHODS: Within 18-months five children with brainstem symptoms and the radiological diagnosis of a malignant brainstem glioma, were identified...
July 12, 2017: Journal of Clinical Neuroscience: Official Journal of the Neurosurgical Society of Australasia
https://www.readbyqxmd.com/read/28686124/influence-of-an-intratumoral-cyst-on-drug-distribution-by-convection-enhanced-delivery-case-report
#8
Iryna Ivasyk, Peter F Morgenstern, Eva Wembacher-Schroeder, Mark M Souweidane
Convection-enhanced delivery (CED) uses positive pressure to induce convective flow of molecules and maximize drug distribution. Concerns have been raised about the effect of cystic structures on uniform drug distribution with CED. The authors describe the case of a patient with a diffuse intrinsic pontine glioma (DIPG) with a large cyst and examine its effect on drug distribution after CED with a radiolabeled antibody. The patient was treated according to protocol with CED of (124)I-8H9 to the pons for nonprogressive DIPG after radiation therapy as part of a Phase I trial (clinical trial registration no...
July 7, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28686121/surgical-and-molecular-considerations-in-the-treatment-of-pediatric-thalamopeduncular-tumors
#9
Ryan P Lee, Kimberly A Foster, Jock C Lillard, Paul Klimo, David W Ellison, Brent Orr, Frederick A Boop
OBJECTIVE Thalamopeduncular tumors are a group of pediatric low-grade gliomas that arise at the interface of the thalamus and brainstem peduncle. They typically occur within the first 2 decades of life, presenting with progressive spastic hemiparesis. Treatment strategies, including surgical intervention, have varied significantly. The authors present their experience in the treatment of 13 children, ages 2-15 years, with non-neurofibromatosis-related pilocytic astrocytomas located in the thalamopeduncular region...
July 7, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28640700/cns-tumors-in-neurofibromatosis
#10
Jian Campian, David H Gutmann
Neurofibromatosis (NF) encompasses a group of distinct genetic disorders in which affected children and adults are prone to the development of benign and malignant tumors of the nervous system. The purpose of this review is to discuss the spectrum of CNS tumors arising in individuals with NF type 1 (NF1) and NF type 2 (NF2), their pathogenic etiologies, and the rational treatment options for people with these neoplasms. This article is a review of preclinical and clinical data focused on the treatment of the most common CNS tumors encountered in children and adults with NF1 and NF2...
July 20, 2017: Journal of Clinical Oncology: Official Journal of the American Society of Clinical Oncology
https://www.readbyqxmd.com/read/28621573/update-on-the-diagnostic-value-and-safety-of-stereotactic-biopsy-for-pediatric-brainstem-tumors-a-systematic-review-and-meta-analysis-of-735-cases
#11
Christina Hamisch, Philipp Kickingereder, Matthias Fischer, Thorsten Simon, Maximilian I Ruge
OBJECTIVE Recent studies have shed light on the molecular makeup of diffuse intrinsic pontine gliomas and led to the identification of potential treatment targets for these lesions, which account for the majority of pediatric brainstem tumors (pedBSTs). Therefore, stereotactic biopsy-driven molecular characterization of pedBSTs may become an important prerequisite for the management of these fatal brain tumors. The authors conducted a systemic review and meta-analysis to precisely determine the safety and diagnostic success of stereotactic biopsy of pedBSTs...
June 16, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28620564/moyamoya-syndrome-in-a-child-with-neurofibromatosis-type-1-magnetic-resonance-imaging-as-a-tool-for-clinical-decision-making
#12
Jonathan Mayl, Hanisha Patel, Tushar Chandra
Moyamoya syndrome is a rare cerebrovasculopathy of unknown etiology which is associated with multiple risk factors. Moyamoya was first discovered in Japan and is reported to have an increased prevalence in the Japanese population. The term "Moyamoya" translates into "puff of smoke" and is named after the finding of the collateral cerebral vasculature that develops secondary to occlusion of an internal carotid artery at the entrance into the circle of Willis. This collateral vasculature characterizes the disease...
May 9, 2017: Curēus
https://www.readbyqxmd.com/read/28592748/epigenetic-targeted-therapy-for-diffuse-intrinsic-pontine-glioma
#13
Rintaro Hashizume
Diffuse intrinsic pontine glioma (DIPG) is a rare but uniformly fatal cancer of the brain, with peak incidence in children of 5-7 years of age. In contrast to most types of human cancer, there has been no significant improvement in treatment outcomes for patients with DIPG. Since DIPG occurs in the brainstem, a vital region of the brain, there are no surgical options for providing relief to patients, and chemotherapy as well as radiation therapy provide palliative relief at best. To date, more than 250 clinical trials evaluating radiotherapy along with conventional cytotoxic chemotherapy, as well as newer biologic agents, have failed to improve the dismal outcome when compared with palliative radiation alone...
June 7, 2017: Neurologia Medico-chirurgica
https://www.readbyqxmd.com/read/28567590/evaluation-of-outcomes-after-stereotactic-radiosurgery-for-pilocytic-astrocytoma
#14
Daniel M Trifiletti, M Sean Peach, Zhiyuan Xu, Ronald Kersh, Timothy N Showalter, Jason P Sheehan
Pilocytic astrocytomas are rare intracranial gliomas that are typically treated with surgical extirpation. Our aim was to report the radiologic and clinical outcomes of patients treated with stereotactic radiosurgery (SRS) for pilocytic astrocytoma in the primary and salvage setting. Patients with pilocytic astrocytoma treated at a single institution with SRS from 1990 to 2015 were reviewed. Patient, disease, and treatment characteristics were collected and overall survival, local control, and toxicity were evaluated...
May 31, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28544746/clinical-outcomes-following-proton-therapy-for-children-with-central-nervous-system-tumors-referred-overseas
#15
Daniel J Indelicato, Julie A Bradley, Eric S Sandler, Philipp R Aldana, Amy Sapp, Jennifer E Gains, Adrian Crellin, Ronny L Rotondo
BACKGROUND: International, multidisciplinary care of children with central nervous system (CNS) tumors presents unique challenges. The aim of this study is to report patient outcomes of U.K. children referred for proton therapy to a North American facility. METHODS: From 2008 to 2016, 166 U.K. children with approved CNS tumors were treated with proton therapy at a single academic medical center in the United States. Median age was 7 years (range, 1-19). Median follow-up was 2...
May 24, 2017: Pediatric Blood & Cancer
https://www.readbyqxmd.com/read/28522693/histone-h3-3k27m-represses-p16-to-accelerate-gliomagenesis-in-a-murine-model-of-dipg
#16
Francisco J Cordero, Zhiqing Huang, Carole Grenier, Xingyao He, Guo Hu, Roger E McLendon, Susan K Murphy, Rintaro Hashizume, Oren J Becher
Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive pediatric brainstem tumor genetically distinguished from adult GBM by the high prevalence of the K27M mutation in the histone H3 variant H3.3 (H3F3A). This mutation reprograms the H3K27me3 epigenetic landscape of DIPG by inhibiting the H3K27-specific histone methyltransferase EZH2. This globally reduces H3K27me2/3, critical repressive marks responsible for cell fate decisions, and also causes focal gain of H3K27me3 throughout the epigenome. To date, the tumor-driving effects of H3...
May 18, 2017: Molecular Cancer Research: MCR
https://www.readbyqxmd.com/read/28521451/dosimetric-comparison-of-intensity-modulated-radiation-therapy-and-volumetric-modulated-arc-therapy-plans-for-the-treatment-of-glioma-using-flattening-filter-free-and-flattening-filter-modes
#17
Dandan Xu, Fei Jia, Guowen Li, Hongfei Li
In the present study, the dose verification between 6X and 6X flattening filter-free (FFF) in intensity-modulated radiation therapy (IMRT) and volumetric modulated arc therapy (VMAT) was compared, and the advantages and disadvantages of different radiotherapy plans were evaluated. All four plans achieved comparable heterogeneity and conformity indices. For frontal tumor, VMAT demonstrated more improved sparing of the brainstem compared with the IMRT (P=0.045); while in the model of FFF, the Dmax of eye lens was significantly reduced by 16-21% (P<0...
May 2017: Oncology Letters
https://www.readbyqxmd.com/read/28484537/dorsally-exophytic-glioblastoma-arising-from-the-medulla-oblongata-in-an-adult-presenting-as-4-th-ventricular-mass
#18
Kuntal Kanti Das, Guru Prasad Bettaswamy, Anant Mehrotra, Sushila Jaiswal, Awadhesh Kumar Jaiswal, Sanjay Behari
Brainstem gliomas are relatively rare in adults (<2% of all gliomas). Exophytic gliomas are focal brainstem lesions, which project into the 4(th) ventricle or cerebellopontine angles. These exophytic lesions are usually of low-grade histology (pilocytic astrocytoma or ganglioglioma) and have a relatively better outcome compared with brainstem gliomas as a whole. Glioblastoma is the commonest primary glial cell neoplasm and mostly occurs in the supratentorial compartment. It is rather uncommon in the brainstem and seldom has been described as having an exophytic growth pattern...
April 2017: Asian Journal of Neurosurgery
https://www.readbyqxmd.com/read/28440867/the-epileptology-of-koolen-de-vries-syndrome-electro-clinico-radiologic-findings-in-31-patients
#19
Kenneth A Myers, Simone A Mandelstam, Georgia Ramantani, Elisabeth J Rushing, Bert B de Vries, David A Koolen, Ingrid E Scheffer
OBJECTIVE: This study was designed to describe the spectrum of epilepsy phenotypes in Koolen-de Vries syndrome (KdVS), a genetic syndrome involving dysmorphic features, intellectual disability, hypotonia, and congenital malformations, that occurs secondary to 17q21.31 microdeletions and heterozygous mutations in KANSL1. METHODS: We were invited to attend a large gathering of individuals with KdVS and their families. While there, we recruited individuals with KdVS and seizures, and performed thorough phenotyping...
June 2017: Epilepsia
https://www.readbyqxmd.com/read/28439659/reported-outcomes-of-children-with-newly-diagnosed-high-grade-gliomas-treated-with-nimotuzumab-and-irinotecan
#20
Nongnuch Sirachainan, Atthaporn Boongird, Thiti Swangsilpa, Wipawi Klaisuban, Apasri Lusawat, Suradej Hongeng
PURPOSE: The outcome of children with high-grade gliomas (HGGs) treated with radiation and adjuvant chemotherapy remains poor. The expression of epidermal growth factor receptor (EGFR) has been established in children with HGGs. This report demonstrated the outcomes of adjuvant nimotuzumab, an EGFR inhibitor, with irinotecan in pediatric HGGs. METHODS: Children with newly diagnosed HGGs were enrolled. Two weeks after surgery, nimotuzumab with a dose of 150 mg/m(2) was given every week during radiation...
April 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
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