keyword

Brainstem glioma

keyword
https://read.qxmd.com/read/32228694/the-molecular-characteristics-of-spinal-cord-gliomas-with-or-without-h3-k27m-mutation
#1
Rui-Chao Chai, Yao-Wu Zhang, Yu-Qing Liu, Yu-Zhou Chang, Bo Pang, Tao Jiang, Wen-Qing Jia, Yong-Zhi Wang
Due to the rare incidence of spinal cord astrocytomas, their molecular features remain unclear. Here, we characterized the landscapes of mutations in H3 K27M, isocitrate dehydrogenase 1 (IDH1) R132H, BRAF V600E, and the TERT promoter in 83 diffuse spinal cord astrocytic tumors. Among these samples, thirty-five patients had the H3 K27M mutation; this mutant could be observed in histological grade II (40%), III (40%), and IV (20%) astrocytomas. IDH1 mutations were absent in 58 of 58 cases tested. The BRAF V600E mutation (7/57) was only observed in H3-wildtype astrocytomas, and was associated with a better prognosis in all histological grade II/III astrocytomas...
March 30, 2020: Acta Neuropathologica Communications
https://read.qxmd.com/read/32196609/h3k27m-mutant-diffuse-midline-glioma-a-case-report
#2
Y Luo, L Zeng, X-Q Xie, F Wang, Y-Z Liu, J-B Kang, X-F Li, D-B Wu, B-L Qu
OBJECTIVE: Diffuse midline glioma with H3K27M mutation is a new tumor type of WHO central nervous system tumor classification. It often occurs in the midline structure and usually has a poor prognosis. CASE REPORT: A 38-year-old male patient presented with 2 years history of right limb with facial numbness, tumors in the left thalamic region and lateral ventricle was detected by imaging. The patient underwent the first surgery. RESULTS: The pathological examination results: Glioblastoma...
March 2020: European Review for Medical and Pharmacological Sciences
https://read.qxmd.com/read/32192255/en-mann-i-60-%C3%A3-rene-med-gangvansker-dysartri-og-lett-kognitiv-svikt
#3
Ludmila Nordaa, Elin Bjelland Forsaa, Audun Odland, Soffien Ajmi
BACKGROUND: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is an immune-mediated, treatable and inflammatory CNS disease first reported by Pittock et al. (2010). CASE PRESENTATION: We describe a 66-year-old man with previous history of diabetes, atrial fibrillation and hypertension, who was admitted to hospital with reduced general condition. He had experienced dizziness and unstable gait for a year, and had been periodically confused, especially in the previous month...
March 17, 2020: Tidsskrift for Den Norske Lægeforening: Tidsskrift for Praktisk Medicin, Ny Række
https://read.qxmd.com/read/32166329/pharmacologic-inhibition-of-lysine-specific-demethylase-1-lsd1-as-a-therapeutic-and-immune-sensitization-strategy-in-pediatric-high-grade-glioma-phgg
#4
Cavan P Bailey, Mary Figueroa, Achintyan Gangadharan, Yanwen Yang, Megan M Romero, Bridget A Kennis, Sridevi Yadavilli, Verlene Henry, Tiara Collier, Michelle Monje, Dean A Lee, Linghua Wang, Javad Nazarian, Vidya Gopalakrishnan, Wafik Zaky, Oren J Becher, Joya Chandra
BACKGROUND: Diffuse midline gliomas (DMG), including brainstem DIPG, are incurable pediatric high grade gliomas (pHGG). Mutations in the H3 histone tail (H3.1/3.3-K27M) are a feature of DIPG, rendering them therapeutically sensitive to small-molecule inhibition of chromatin modifiers. Pharmacological inhibition of lysine specific demethylase-1 (LSD1) is clinically relevant but has not been carefully investigated in pHGG or DIPG. METHODS: Patient-derived DIPG cell lines, orthotopic mouse models, and pHGG datasets were used to evaluate effects of LSD1 inhibitors on cytotoxicity and immune gene expression...
March 13, 2020: Neuro-oncology
https://read.qxmd.com/read/32118205/noninvasive-quantification-of-sirt1-expression-activity-and-pharmacologic-inhibition-in-a-rat-model-of-intracerebral-glioma-using-2-18-f-bzaha-pet-ct-mri
#5
Maxwell T Laws, Robin E Bonomi, David J Gelovani, Jeremy Llaniguez, Xin Lu, Thomas Mangner, Juri G Gelovani
Background: Several studies demonstrated that glioblastoma multiforme progression and recurrence is linked to epigenetic regulatory mechanisms. Sirtuin 1 (SIRT1) plays an important role in glioma progression, invasion, and treatment response and is a potential therapeutic target. The aim of this study is to test the feasibility of 2-[18 F]BzAHA for quantitative imaging of SIRT1 expression-activity and monitoring pharmacologic inhibition in a rat model of intracerebral glioma. Methods: Sprague Dawley rats bearing 9L ( N = 12) intracerebral gliomas were injected with 2-[18 F]BzAHA (300-500 µCi/animal i...
January 2020: Neuro-oncology advances
https://read.qxmd.com/read/32078691/epigenetic-reprogramming-and-chromatin-accessibility-in-pediatric-diffuse-intrinsic-pontine-gliomas-a-neural-developmental-disease
#6
Flor M Mendez, Felipe J Núñez, Maria B Garcia-Fabiani, Santiago Haase, Stephen Carney, Jessica C Gauss, Oren J Becher, Pedro R Lowenstein, Maria G Castro
Diffuse intrinsic pontine glioma (DIPG) is a rare but deadly pediatric brainstem tumor. To date, there is no effective therapy for DIPG. Transcriptomic analyses have revealed DIPGs have a distinct profile from other pediatric high-grade gliomas occurring in the cerebral hemispheres. These unique genomic characteristics coupled with the younger median age group suggest that DIPG has a developmental origin. The most frequent mutation in DIPG is a lysine to methionine (K27M) mutation that occurs on H3F3A and HIST1H3B/C, genes encoding histone variants...
February 20, 2020: Neuro-oncology
https://read.qxmd.com/read/32050461/retrospective-and-randomized-analysis-of-influence-and-correlation-of-clinical-and-molecular-prognostic-factors-in-a-mono-operative-series-of-122-patients-with-glioblastoma-treated-with-str-or-gtr
#7
Maurizio Salvati, Placido Bruzzaniti, Michela Relucenti, Mariagrazia Nizzola, Pietro Familiari, Marco Giugliano, Anthony Kevin Scafa, Santi Galletta, Xiaobo Li, Rui Chen, Claudio Barbaranelli, Alessandro Frati, Antonio Santoro
Glioblastoma is a solid, infiltrating, and the most frequent highly malignant primary brain tumor. Our aim was to find the correlation between sex, age, preoperative Karnofsky performance status (KPS), presenting with seizures, and extent of resection (EOR) with overall survival (OS), progression-free survival (PFS), and postoperative KPS, along with the prognostic value of IDH1, MGMT, ATRX, EGFR, and TP53 genes mutations and of Ki67 through the analysis of a single-operator series in order to avoid the biases of a multi-operator series, such as the lack of homogeneity in surgical and adjuvant nonsurgical treatments...
February 9, 2020: Brain Sciences
https://read.qxmd.com/read/32028001/longitudinal-monitoring-of-gd-dtpa-following-convection-enhanced-delivery-in-the-brainstem
#8
Umberto Tosi, Mark M Souweidane
BACKGROUND: Convection-enhanced delivery (CED) has been introduced into contemporary therapeutic strategies for incurable brain neoplasms as diffuse intrinsic pontine glioma (DIPG). Therapeutic benefit in part is predictably dependent upon drug distribution within tumor. However, therapeutics can rarely be detected through conventional imaging techniques. Co-infusion of the tracer Gadolinium-diethylenetriaminepentacetate (Gd-DTPA) has been advocated to monitor drug distributive features including volume (Vd), tumor coverage, and efflux during and after administration...
February 3, 2020: World Neurosurgery
https://read.qxmd.com/read/32025728/high-grade-gliomas-in-adolescents-and-young-adults-highlight-histomolecular-differences-with-their-adult-and-paediatric-counterparts
#9
Alexandre Roux, Johan Pallud, Raphaël Saffroy, Myriam Edjlali-Goujon, Marie-Anne Debily, Nathalie Boddaert, Marc Sanson, Stéphanie Puget, Steven Knafo, Clovis Adam, Thierry Faillot, Dominique Cazals-Hatem, Emmanuel Mandonnet, Marc Polivka, Georges Dorfmüller, Aurélie Dauta, Mathilde Desplanques, Albane Gareton, Mélanie Pages, Arnault Tauziede-Espariat, Jacques Grill, Franck Bourdeaut, François Doz, Frédéric Dhermain, Karima Mokhtari, Fabrice Chretien, Dominique Figarella-Branger, Pascale Varlet
BACKGROUND: Considering that paediatric high-grade gliomas (HGGs) are biologically distinct from their adult counterparts, the objective of this study was to define the landscape of HGGs in adolescents and young adults (AYAs). METHODS: We performed a multicentric retrospective study of 112 AYAs from adult and paediatric Ile-de-France neurosurgical units, treated between 1998 and 2013 to analyse their clinicoradiological and histomolecular profiles. The inclusion criteria were age between 15 and 25-years, histopathological HGG diagnosis, available clinical data, pre-operative and follow-up MRI...
February 6, 2020: Neuro-oncology
https://read.qxmd.com/read/32016716/radiation-dose-response-of-neurologic-symptoms-during-conformal-radiotherapy-for-diffuse-intrinsic-pontine-glioma
#10
Christopher L Tinkle, Kristen Campbell, Yuanyuan Han, Yimei Li, Brandon Bianski, Alberto Broniscer, Raja B Khan, Thomas E Merchant
PURPOSE: To estimate the rate and magnitude of neurologic symptom change during radiation therapy (RT) and impact of symptom change on survival outcomes in patients with diffuse intrinsic pontine glioma (DIPG). METHODS: From 2006 to 2014, 108 patients with newly diagnosed DIPG were treated with conventionally fractionated radiation therapy (RT) to 54 Gy (median) at our institution. The presence and severity of neurologic symptoms related to cranial neuropathy (CN) and cerebellar (CB) and long-tract (LT) signs was reviewed before and weekly during RT for each patient...
February 3, 2020: Journal of Neuro-oncology
https://read.qxmd.com/read/32002312/impact-of-intravenous-vitamin-c-and-endolaser-therapies-on-a-pediatric-brainstem-glioma-case
#11
Ivette M Solís-Nolasco, Gilberto Caraballo, Michael J González, José Olalde, Raul H Morales-Borges
Pediatric cases of a brainstem glioma are one of the most difficult tumors to treat. In children, this type of cancer is perhaps the most dreaded of cancers due to its poor prognosis, yet it remains an area of intense research. In the case presented herein, conventional chemotherapy and radiotherapy treatments were not showing any improvement. The patient was then treated with high-dose intravenous vitamin C (IVC) and endolaser therapy. A significant reduction in glioma's size was achieved in 2 months with this adjunct therapy...
2020: Global Advances in Health and Medicine: Improving Healthcare Outcomes Worldwide
https://read.qxmd.com/read/31940975/combined-targeting-of-mutant-p53-and-jumonji-family-histone-demethylase-augments-therapeutic-efficacy-of-radiation-in-h3k27m-dipg
#12
Anatoly Nikolaev, John B Fiveash, Eddy S Yang
Diffuse intrinsic pontine glioma (DIPG) is an aggressive pediatric brainstem tumor with a 5-year survival of <1%. Up to 80% of the DIPG tumors contain a specific K27M mutation in one of the two genes encoding histone H3 (H3K27M). Furthermore, p53 mutations found in >70-80% of H3K27M DIPG, and mutant p53 status is associated with a decreased response to radiation treatment and worse overall prognosis. Recent evidence indicates that H3K27M mutation disrupts tri-methylation at H3K27 leading to aberrant gene expression...
January 13, 2020: International Journal of Molecular Sciences
https://read.qxmd.com/read/31925004/alk2-inhibitors-display-beneficial-effects-in-preclinical-models-of-acvr1-mutant-diffuse-intrinsic-pontine-glioma
#13
Diana Carvalho, Kathryn R Taylor, Nagore Gene Olaciregui, Valeria Molinari, Matthew Clarke, Alan Mackay, Ruth Ruddle, Alan Henley, Melanie Valenti, Angela Hayes, Alexis De Haven Brandon, Suzanne A Eccles, Florence Raynaud, Aicha Boudhar, Michelle Monje, Sergey Popov, Andrew S Moore, Jaume Mora, Ofelia Cruz, Mara Vinci, Paul E Brennan, Alex N Bullock, Angel Montero Carcaboso, Chris Jones
Diffuse intrinsic pontine glioma (DIPG) is a lethal childhood brainstem tumour, with a quarter of patients harbouring somatic mutations in ACVR1, encoding the serine/threonine kinase ALK2. Despite being an amenable drug target, little has been done to-date to systematically evaluate the role of ACVR1 in DIPG, nor to screen currently available inhibitors in patient-derived tumour models. Here we show the dependence of DIPG cells on the mutant receptor, and the preclinical efficacy of two distinct chemotypes of ALK2 inhibitor in vitro and in vivo...
May 9, 2019: Communications Biology
https://read.qxmd.com/read/31924736/radiological-evaluation-of-newly-diagnosed-non-brainstem-pediatric-high-grade-glioma-in-the-herby-phase-ii-trial
#14
Daniel Rodriguez Gutierrez, Chris Jones, Pascale Varlet, Alan Mackay, Daniel Warren, Monika Warmuth-Metz, Esther Sánchez Aliaga, Raphael Calmon, Darren R Hargrave, Adela Cañete, Maura Massimino, Amedeo A Azizi, Marie-Cécile Le Deley, Frank Saran, Raphael F Rousseau, Gudrun Zahlmann, Josep Garcia, Gilles Vassal, Jacques Grill, Paul S Morgan, Tim Jaspan
BACKGROUND: The HERBY trial evaluated the benefit of the addition of the antiangiogenic agent Bevacizumab (BEV) to radiotherapy/Temozolomide (RT/TMZ) in pediatric patients with newly diagnosed non-brainstem high-grade glioma (HGG). The work presented here aims to correlate imaging characteristics and outcome measures with pathological and molecular data. METHODS: Radiological, pathological and molecular data were correlated with trial clinical information to retrospectively re-evaluate event free and overall survival...
January 10, 2020: Clinical Cancer Research
https://read.qxmd.com/read/31912225/phrenic-nerve-neurotization-utilizing-half-of-the-spinal-accessory-nerve-to-the-functional-restoration-of-the-paralyzed-diaphragm-in-high-spinal-cord-injury-secondary-to-brain-tumor-resection
#15
Antonio Heredia Gutiérrez, Gabriel Emmanuel Cachón Cámara, Vicente González Carranza, Samuel Torres García, Fernando Chico Ponce de León
The authors present a case of functional improvement of diaphragmatic paralysis in high spinal cord injury, performing a neurotization of the phrenic nerve with accessory spinal nerve hemisection. A C1-C2 injury of the spinal cord was diagnosed in a 12-year-old male, secondary to resection of a brainstem glioma. The patient did not have diaphragmatic motility at the moment that the mechanical ventilation was removed; however, he presented apnea. The patient underwent neurotization of the right phrenic nerve with the right spinal accessory nerve, 5 months after the injury and 6 months after nerve transfer; he had complete mobilization of the right hemidiaphragm, nevertheless persisted with paralysis of the left hemidiaphragm...
January 8, 2020: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://read.qxmd.com/read/31903354/pediatric-brainstem-gliomas-an-institutional-experience
#16
Altaf Ali Laghari, Mirza Zain Baig, Ehsan Bari, Aneela Darbar, Naureen Mushtaq, Umm E Hani Abdullah, Daniyal Aziz Khan
Objective: The aim of this study was to analyze the clinical profiles and outcomes of pediatric brainstem gliomas treated at our institute. Methodology: We reviewed the files of 18 pediatric age group patients diagnosed with brainstem glioma at our institution. The following variables were recorded: age, sex, duration of symptoms, date of diagnosis, main clinical symptoms, Karnofsky performance status score, magnetic resonance imaging findings, histopathology findings, details of the treatment given, disease progression, and date of mortality/last follow-up...
October 2019: Asian Journal of Neurosurgery
https://read.qxmd.com/read/31896090/evaluating-infusate-parameters-for-direct-drug-delivery-to-the-brainstem-a-comparative-study-of-convection-enhanced-delivery-versus-osmotic-pump-delivery
#17
Julian S Rechberger, Erica A Power, Victor M Lu, Liang Zhang, Jann N Sarkaria, David J Daniels
OBJECTIVE: Convection-enhanced delivery (CED) and osmotic pump delivery both have been promoted as promising techniques to deliver drugs to pediatric diffuse intrinsic pontine gliomas (DIPGs). Correspondingly, the aim of this study was to understand how infusate molecular weight (MW), duration of delivery, and mechanism of delivery (CED or osmotic pump) affect volume of distribution (Vd) in the brainstem, to better inform drug selection and delivery in future DIPG investigations. METHODS: A series of in vivo experiments were conducted using rat models...
January 1, 2020: Neurosurgical Focus
https://read.qxmd.com/read/31896089/deformational-changes-after-convection-enhanced-delivery-in-the-pediatric-brainstem
#18
Evan D Bander, Karima Tizi, Eva Wembacher-Schroeder, Rowena Thomson, Maria Donzelli, Elizabeth Vasconcellos, Mark M Souweidane
OBJECTIVE: In the brainstem, there are concerns regarding volumetric alterations following convection-enhanced delivery (CED). The relationship between distribution volume and infusion volume is predictably greater than one. Whether this translates into deformational changes and influences clinical management is unknown. As part of a trial using CED for diffuse intrinsic pontine glioma (DIPG), the authors measured treatment-related volumetric alterations in the brainstem and ventricles...
January 1, 2020: Neurosurgical Focus
https://read.qxmd.com/read/31896081/progress-in-diffuse-intrinsic-pontine-glioma-advocating-for-stereotactic-biopsy-in-the-standard-of-care
#19
John R Williams, Christopher C Young, Nicholas A Vitanza, Margaret McGrath, Abdullah H Feroze, Samuel R Browd, Jason S Hauptman
Diffuse intrinsic pontine glioma (DIPG) is a universally fatal pediatric brainstem tumor affecting approximately 300 children in the US annually. Median survival is less than 1 year, and radiation therapy has been the mainstay of treatment for decades. Recent advances in the biological understanding of the disease have identified the H3K27M mutation in nearly 80% of DIPGs, leading to the 2016 WHO classification of diffuse midline glioma H3K27M-mutant, a grade IV brainstem tumor. Developments in epigenetic targeting of transcriptional tendencies have yielded potential molecular targets for clinical trials...
January 1, 2020: Neurosurgical Focus
https://read.qxmd.com/read/31873874/the-role-of-radiation-and-chemotherapy-in-adult-patients-with-high-grade-brainstem-gliomas-results-from-the-national-cancer-database
#20
Panagiotis Kerezoudis, Anshit Goyal, Victor M Lu, Mohammed Ali Alvi, Mohamad Bydon, Sani H Kizilbash, Terry C Burns
INTRODUCTION: Surgical resection of high-grade brainstem gliomas is challenging and treatment mostly involves radiation and chemotherapy. In this study, we utilized registry data to determine prognostic features and impact of chemotherapy and radiation on overall survival. METHODS: The National Cancer Database was queried from 2006 to 2015 for adult cases with histologically confirmed high-grade brainstem glioma. Covariates including patient demographics, comorbidities, tumor characteristics and treatment parameters were captured...
December 24, 2019: Journal of Neuro-oncology
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