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Diffuse Intrinsic Pontine Glioma

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https://www.readbyqxmd.com/read/28339768/the-transcription-factor-olig2-is-important-for-the-biology-of-diffuse-intrinsic-pontine-gliomas
#1
Jane L Anderson, Ranjithmenon Muraleedharan, Nicole Oatman, Amanda Klotter, Satarupa Sengupta, Ronald R Waclaw, Jianqiang Wu, Rachid Drissi, Lili Miles, Eric H Raabe, Matthew L Weirauch, Maryam Fouladi, Lionel M Chow, Lindsey Hoffman, Mariko DeWire, Biplab Dasgupta
Background.: Diffuse intrinsic pontine glioma (DIPG) is a high-grade brainstem glioma of children with dismal prognosis. There is no single unifying model about the cell of origin of DIPGs. Proliferating cells in the developing human and mouse pons, the site of DIPGs, express neural stem/progenitor cell (NPC) markers, including Sox2, nestin, vimentin, Olig2, and glial fibrillary acidic protein, in an overlapping and non-overlapping manner, suggesting progenitor cell heterogeneity in the pons...
February 23, 2017: Neuro-oncology
https://www.readbyqxmd.com/read/28303929/neuro-oncology-new-therapeutic-targets-for-diffuse-intrinsic-pontine-glioma
#2
Charlotte Ridler
No abstract text is available yet for this article.
March 17, 2017: Nature Reviews. Neurology
https://www.readbyqxmd.com/read/28298418/disrupting-the-cd47-sirp%C3%AE-anti-phagocytic-axis-by-a-humanized-anti-cd47-antibody-is-an-efficacious-treatment-for-malignant-pediatric-brain-tumors
#3
Sharareh Gholamin, Siddhartha S Mitra, Abdullah H Feroze, Jie Liu, Suzana A Kahn, Michael Zhang, Rogelio Esparza, Chase Richard, Vijay Ramaswamy, Marc Remke, Anne K Volkmer, Stephen Willingham, Anitha Ponnuswami, Aaron McCarty, Patricia Lovelace, Theresa A Storm, Simone Schubert, Gregor Hutter, Cyndhavi Narayanan, Pauline Chu, Eric H Raabe, Griffith Harsh, Michael D Taylor, Michelle Monje, Yoon-Jae Cho, Ravi Majeti, Jens P Volkmer, Paul G Fisher, Gerald Grant, Gary K Steinberg, Hannes Vogel, Michael Edwards, Irving L Weissman, Samuel H Cheshier
Morbidity and mortality associated with pediatric malignant primary brain tumors remain high in the absence of effective therapies. Macrophage-mediated phagocytosis of tumor cells via blockade of the anti-phagocytic CD47-SIRPα interaction using anti-CD47 antibodies has shown promise in preclinical xenografts of various human malignancies. We demonstrate the effect of a humanized anti-CD47 antibody, Hu5F9-G4, on five aggressive and etiologically distinct pediatric brain tumors: group 3 medulloblastoma (primary and metastatic), atypical teratoid rhabdoid tumor, primitive neuroectodermal tumor, pediatric glioblastoma, and diffuse intrinsic pontine glioma...
March 15, 2017: Science Translational Medicine
https://www.readbyqxmd.com/read/28291423/preclinical-evaluation-of-convection-enhanced-delivery-of-liposomal-doxorubicin-to-treat-pediatric-diffuse-intrinsic-pontine-glioma-and-thalamic-high-grade-glioma
#4
A Charlotte P Sewing, Tonny Lagerweij, Dannis G van Vuurden, Michaël H Meel, Susanna J E Veringa, Angel M Carcaboso, Pieter J Gaillard, W Peter Vandertop, Pieter Wesseling, David Noske, Gertjan J L Kaspers, Esther Hulleman
OBJECTIVE Pediatric high-grade gliomas (pHGGs) including diffuse intrinsic pontine gliomas (DIPGs) are primary brain tumors with high mortality and morbidity. Because of their poor brain penetrance, systemic chemotherapy regimens have failed to deliver satisfactory results; however, convection-enhanced delivery (CED) may be an alternative mode of drug delivery. Anthracyclines are potent chemotherapeutics that have been successfully delivered via CED in preclinical supratentorial glioma models. This study aims to assess the potency of anthracyclines against DIPG and pHGG cell lines in vitro and to evaluate the efficacy of CED with anthracyclines in orthotopic pontine and thalamic tumor models...
February 17, 2017: Journal of Neurosurgery. Pediatrics
https://www.readbyqxmd.com/read/28283507/inhibition-of-ezh2-or-bet-suppresses-diffuse-intrinsic-pontine-glioma
#5
(no author information available yet)
H3K27M-expressing diffuse intrinsic pontine gliomas (DIPG) are dependent on residual PRC2 activity.
March 10, 2017: Cancer Discovery
https://www.readbyqxmd.com/read/28274399/radiation-and-subsequent-reirradiation-outcomes-in-the-treatment-of-diffuse-intrinsic-pontine-glioma-and-a-systematic-review-of-the-reirradiation-literature
#6
Christopher Freese, Vinita Takiar, Maryam Fouladi, Mariko DeWire, John Breneman, Luke Pater
PURPOSE: Diffuse intrinsic pontine glioma (DIPG) is a devastating pediatric disease, with a median survival of <1 year. Here, we review our institution's DIPG experience over an 8-year interval and perform a systematic review of the literature, specifically evaluating reports of reirradiation (reRT) for DIPG. METHODS AND MATERIALS: We retrospectively reviewed the medical records of 26 patients who underwent definitive intensity modulated radiation therapy (IMRT) for DIPG at a single institution between 2007 and 2015...
March 2017: Practical Radiation Oncology
https://www.readbyqxmd.com/read/28263309/ezh2-is-a-potential-therapeutic-target-for-h3k27m-mutant-pediatric-gliomas
#7
Faizaan Mohammad, Simon Weissmann, Benjamin Leblanc, Deo P Pandey, Jonas W Højfeldt, Itys Comet, Chunqin Zheng, Jens Vilstrup Johansen, Nicolas Rapin, Bo T Porse, Andrey Tvardovskiy, Ole N Jensen, Nagore G Olaciregui, Cinzia Lavarino, Mariona Suñol, Carmen de Torres, Jaume Mora, Angel M Carcaboso, Kristian Helin
Diffuse intrinsic pontine glioma (DIPG) is an aggressive brain tumor that is located in the pons and primarily affects children. Nearly 80% of DIPGs harbor mutations in histone H3 genes, wherein lysine 27 is substituted with methionine (H3K27M). H3K27M has been shown to inhibit polycomb repressive complex 2 (PRC2), a multiprotein complex responsible for the methylation of H3 at lysine 27 (H3K27me), by binding to its catalytic subunit EZH2. Although DIPGs with the H3K27M mutation show global loss of H3K27me3, several genes retain H3K27me3...
February 27, 2017: Nature Medicine
https://www.readbyqxmd.com/read/28263307/therapeutic-targeting-of-polycomb-and-bet-bromodomain-proteins-in-diffuse-intrinsic-pontine-gliomas
#8
Andrea Piunti, Rintaro Hashizume, Marc A Morgan, Elizabeth T Bartom, Craig M Horbinski, Stacy A Marshall, Emily J Rendleman, Quanhong Ma, Yoh-Hei Takahashi, Ashley R Woodfin, Alexander V Misharin, Nebiyu A Abshiru, Rishi R Lulla, Amanda M Saratsis, Neil L Kelleher, C David James, Ali Shilatifard
Diffuse intrinsic pontine glioma (DIPG) is a highly aggressive pediatric brainstem tumor characterized by rapid and uniform patient demise. A heterozygous point mutation of histone H3 occurs in more than 80% of these tumors and results in a lysine-to-methionine substitution (H3K27M). Expression of this histone mutant is accompanied by a reduction in the levels of polycomb repressive complex 2 (PRC2)-mediated H3K27 trimethylation (H3K27me3), and this is hypothesized to be a driving event of DIPG oncogenesis...
February 27, 2017: Nature Medicine
https://www.readbyqxmd.com/read/28254596/robot-assisted-stereotactic-biopsy-of-diffuse-intrinsic-pontine-glioma-a-single-centre-experience
#9
Andrea Carai, Angela Mastronuzzi, Alessandro De Benedictis, Raffaella Messina, Antonella Cacchione, Evelina Miele, Franco Randi, Giacomo Esposito, Andrea Trezza, Giovanna Stefania Colafati, Alessandra Savioli, Franco Locatelli, Carlo Efisio Marras
BACKGROUND: Diffuse Intrinsic Pontine Glioma (DIPG) is a childhood tumor with dismal prognosis. Emerging molecular signatures have paved the way for stereotactic biopsy in selected Centers. We present our experience in DIPG stereotactic needle biopsy using the Robotic Stereotactic Assisted system (ROSA™) in a consecutive pediatric series. METHODS: All stereotactic biopsy procedures for DIPG performed during the last year at our Institution were considered. All procedures were carried out using the ROSA™ surgical assistant through a precoronary approach...
February 27, 2017: World Neurosurgery
https://www.readbyqxmd.com/read/28251325/second-re-irradiation-for-dipg-progression-re-considering-old-strategies-with-new-approaches
#10
Andres Morales La Madrid, Vicente Santa-María, Ofelia Cruz Martinez, Jaume Mora, Patricia Puerta Roldan, Antonio Guillen Quesada, Mariona Suñol Capella, Carmen de Torres Gomez-Pallete, Alvaro Lassaletta, Normand Laperriere, Salvador Villà, Eric Bouffet
Diffuse intrinsic pontine glioma (DIPG) is an aggressive infiltrative glioma for which no curative therapy is available. Radiation therapy (RT) is the only potentially effective intervention in delaying tumor progression, but only transiently. At progression, re-irradiation is gaining popularity as an effective palliative therapy. However, at second progression, exclusive symptomatic treatment is usually offered. Here we report two patients with DIPG at second progression who were treated with a second re-irradiation course with good response...
March 1, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28236065/glioblastoma-spheroids-produce-infiltrative-gliomas-in-the-rat-brainstem
#11
Zhiping Zhou, Neal Luther, Ranjodh Singh, John A Boockvar, Mark M Souweidane, Jeffrey P Greenfield
PURPOSE: Diffuse intrinsic pontine glioma (DIPG) is universally fatal without proven therapy other than radiation therapy for palliation. Representative animal models will play an essential role in the preclinical stage of future therapy development. To address the shortage of representative models, we created a novel infiltrative brainstem glioma model in rats based on glioblastoma spheroids. METHODS: Cells dissociated from glioblastoma spheroids grown from surgical specimens were implanted into the brainstem of NIH nude rats...
February 24, 2017: Child's Nervous System: ChNS: Official Journal of the International Society for Pediatric Neurosurgery
https://www.readbyqxmd.com/read/28178670/new-in-vivo-avatars-of-diffuse-intrinsic-pontine-gliomas-dipg-from-stereotactic-biopsies-performed-at-diagnosis
#12
Alexandre Plessier, Ludivine Le Dret, Pascale Varlet, Kévin Beccaria, Joëlle Lacombe, Sébastien Mériaux, Françoise Geffroy, Laurence Fiette, Patricia Flamant, Fabrice Chrétien, Thomas Blauwblomme, Stéphanie Puget, Jacques Grill, Marie-Anne Debily, David Castel
Diffuse Instrinsic Pontine Glioma is the most aggressive form of High Grade Gliomas in children. The lack of biological material and the absence of relevant models have hampered the development of new therapeutics. Their extensive infiltration of the brainstem renders any surgical resection impossible and until recently biopsies were considered not informative enough and therefore not recommended. Thus, most models were derived from autopsy material. We aimed to develop relevant in vivo DIPG models that mimic this specific disease and its molecular diversity from tumor material obtained at diagnosis...
February 2, 2017: Oncotarget
https://www.readbyqxmd.com/read/28161497/survival-benefit-for-patients-with-diffuse-intrinsic-pontine-glioma-dipg-undergoing-re-irradiation-at-first-progression-a-matched-cohort-analysis-on-behalf-of-the-siop-e-hgg-dipg-working-group
#13
Geert O Janssens, Lorenza Gandola, Stephanie Bolle, Henry Mandeville, Monica Ramos-Albiac, Karen van Beek, Helen Benghiat, Bianca Hoeben, Andres Morales La Madrid, Rolf-Dieter Kortmann, Darren Hargrave, Johan Menten, Emilia Pecori, Veronica Biassoni, Andre O von Bueren, Dannis G van Vuurden, Maura Massimino, Dominik Sturm, Max Peters, Christof M Kramm
BACKGROUND: Overall survival (OS) of patients with diffuse intrinsic pontine glioma (DIPG) is poor. The purpose of this study is to analyse benefit and toxicity of re-irradiation at first progression. METHODS: At first progression, 31 children with DIPG, aged 2-16 years, underwent re-irradiation (dose 19.8-30.0 Gy) alone (n = 16) or combined with systemic therapy (n = 15). At initial presentation, all patients had typical symptoms and characteristic MRI features of DIPG, or biopsy-proven high-grade glioma...
March 2017: European Journal of Cancer
https://www.readbyqxmd.com/read/28120771/diffuse-intrinsic-pontine-gliomas-in-children-interest-of-robotic-frameless-assisted-biopsy-a-technical-note
#14
H A Coca, H Cebula, M Benmekhbi, M P Chenard, N Entz-Werle, F Proust
INTRODUCTION: Diffuse intrinsic pontine gliomas (DIPG) constitute 10-15% of all brain tumors in the pediatric population; currently prognosis remains poor, with an overall survival of 7-14 months. Recently the indication of DIPG biopsy has been enlarged due to the development of molecular biology and various ongoing clinical and therapeutic trials. Classically a biopsy is performed using a stereotactic frame assisted procedure but the workflow may sometimes be heavy and more complex especially in children...
December 2016: Neuro-Chirurgie
https://www.readbyqxmd.com/read/28116951/neonatal-respiratory-failure-caused-by-congenital-diffuse-intrinsic-pontine-glioma
#15
Katie M Satrom, Rachel A Phelan, Christopher L Moertel, H Brent Clark, Dana E Johnson, Thomas N George
The authors present a case of diffuse intrinsic pontine glioma presenting in a newborn with stridor and respiratory distress that progressed to respiratory failure. Magnetic resonance imaging (MRI) of the brain revealed findings compatible with the diagnosis of diffuse intrinsic pontine glioma. The family pursued palliative care and postmortem examination confirmed WHO grade III astrocytoma.
January 1, 2017: Journal of Child Neurology
https://www.readbyqxmd.com/read/28110411/development-of-the-siope-dipg-network-registry-and-imaging-repository-a-collaborative-effort-to-optimize-research-into-a-rare-and-lethal-disease
#16
Sophie E M Veldhuijzen van Zanten, Joshua Baugh, Brooklyn Chaney, Dennis De Jongh, Esther Sanchez Aliaga, Frederik Barkhof, Johan Noltes, Ruben De Wolf, Jet Van Dijk, Antonio Cannarozzo, Carin M Damen-Korbijn, Jan A Lieverst, Niclas Colditz, Marion Hoffmann, Monika Warmuth-Metz, Brigitte Bison, David T W Jones, Dominik Sturm, Gerrit H Gielen, Chris Jones, Esther Hulleman, Raphael Calmon, David Castel, Pascale Varlet, Géraldine Giraud, Irene Slavc, Stefaan Van Gool, Sandra Jacobs, Filip Jadrijevic-Cvrlje, David Sumerauer, Karsten Nysom, Virve Pentikainen, Sanna-Maria Kivivuori, Pierre Leblond, Natasha Entz-Werle, Andre O von Bueren, Antonis Kattamis, Darren R Hargrave, Péter Hauser, Miklos Garami, Halldora K Thorarinsdottir, Jane Pears, Lorenza Gandola, Giedre Rutkauskiene, Geert O Janssens, Ingrid K Torsvik, Marta Perek-Polnik, Maria J Gil-da-Costa, Olga Zheludkova, Liudmila Shats, Ladislav Deak, Lidija Kitanovski, Ofelia Cruz, Andres Morales La Madrid, Stefan Holm, Nicolas Gerber, Rejin Kebudi, Richard Grundy, Enrique Lopez-Aguilar, Marta Zapata-Tarres, John Emmerik, Tim Hayden, Simon Bailey, Veronica Biassoni, Maura Massimino, Jacques Grill, William P Vandertop, Gertjan J L Kaspers, Maryam Fouladi, Christof M Kramm, Dannis G van Vuurden
Diffuse intrinsic pontine glioma (DIPG) is a rare and deadly childhood malignancy. After 40 years of mostly single-center, often non-randomized trials with variable patient inclusions, there has been no improvement in survival. It is therefore time for international collaboration in DIPG research, to provide new hope for children, parents and medical professionals fighting DIPG. In a first step towards collaboration, in 2011, a network of biologists and clinicians working in the field of DIPG was established within the European Society for Paediatric Oncology (SIOPE) Brain Tumour Group: the SIOPE DIPG Network...
January 21, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28108511/biomarker-based-pet-imaging-of-diffuse-intrinsic-pontine-glioma-in-mouse-models
#17
Susanne Kossatz, Brandon Carney, Melanie E Schweitzer, Giuseppe Carlucci, Vesselin Miloushev, Uday B Maachani, Prajwal Rajappa, Kayvan Keshari, David J Pisapia, Wolfgang A Weber, Mark M Souweidane, Thomas Reiner
Diffuse intrinsic pontine glioma (DIPG) is a childhood brainstem tumor with a universally poor prognosis. Here, we characterize on a positron emission tomography (PET) probe for imaging DIPG in vivo. In human histological tissues, the probes target, poly(ADP)ribose polymerase 1 (PARP1), was highly expressed in DIPG compared to normal brain. PET imaging allowed for the sensitive detection of DIPG in a genetically engineered mouse model (GEMM), and probe uptake correlated to histologically determined tumor infiltration...
January 20, 2017: Cancer Research
https://www.readbyqxmd.com/read/28107439/development-of-a-dipg-orthotopic-model-in-mice-using-an-implantable-guide-screw-system
#18
Miguel Marigil, Naiara Martinez-Velez, Pablo D Domínguez, Miguel Angel Idoate, Enric Xipell, Ana Patiño-García, Marisol Gonzalez-Huarriz, Marc García-Moure, Marie-Pierre Junier, Hervé Chneiweiss, Elías El-Habr, Ricardo Diez-Valle, Sonia Tejada-Solís, Marta M Alonso
OBJECTIVE: In this work we set to develop and to validate a new in vivo frameless orthotopic Diffuse Intrinsic Pontine Glioma (DIPG) model based in the implantation of a guide-screw system. METHODS: It consisted of a guide-screw also called bolt, a Hamilton syringe with a 26-gauge needle and an insulin-like 15-gauge needle. The guide screw is 2.6 mm in length and harbors a 0.5 mm central hole which accepts the needle of the Hamilton syringe avoiding a theoretical displacement during insertion...
2017: PloS One
https://www.readbyqxmd.com/read/28093680/the-international-diffuse-intrinsic-pontine-glioma-registry-an-infrastructure-to-accelerate-collaborative-research-for-an-orphan-disease
#19
Joshua Baugh, Ute Bartels, James Leach, Blaise Jones, Brooklyn Chaney, Katherine E Warren, Jenavieve Kirkendall, Renee Doughman, Cynthia Hawkins, Lili Miles, Christine Fuller, Tim Hassall, Eric Bouffet, Adam Lane, Darren Hargrave, Jacques Grill, Lindsey M Hoffman, Chris Jones, Alex Towbin, Sharon A Savage, Michelle Monje, Xiao-Nan Li, David S Ziegler, Sophie Veldhuijzen van Zanten, Christof M Kramm, Dannis G van Vuurden, Maryam Fouladi
Diffuse intrinsic pontine glioma (DIPG), a rare, often fatal childhood brain tumor, remains a major therapeutic challenge. In 2012, investigators, funded by the DIPG Collaborative (a philanthropic partnership among 29 private foundations), launched the International DIPG Registry (IDIPGR) to advance understanding of DIPG. Comprised of comprehensive deidentified but linked clinical, imaging, histopathological, and genomic repositories, the IDIPGR uses standardized case report forms for uniform data collection; serial imaging and histopathology are centrally reviewed by IDIPGR neuro-radiologists and neuro-pathologists, respectively...
January 16, 2017: Journal of Neuro-oncology
https://www.readbyqxmd.com/read/28052119/pre-clinical-study-of-panobinostat-in-xenograft-and-genetically-engineered-murine-diffuse-intrinsic-pontine-glioma-models
#20
Tammy Hennika, Guo Hu, Nagore G Olaciregui, Kelly L Barton, Anahid Ehteda, Arjanna Chitranjan, Cecilia Chang, Andrew J Gifford, Maria Tsoli, David S Ziegler, Angel M Carcaboso, Oren J Becher
BACKGROUND: Diffuse intrinsic pontine glioma (DIPG), or high-grade brainstem glioma (BSG), is one of the major causes of brain tumor-related deaths in children. Its prognosis has remained poor despite numerous efforts to improve survival. Panobinostat, a histone deacetylase inhibitor, is a targeted agent that has recently shown pre-clinical efficacy and entered a phase I clinical trial for the treatment of children with recurrent or progressive DIPG. METHODS: A collaborative pre-clinical study was conducted using both a genetic BSG mouse model driven by PDGF-B signaling, p53 loss, and ectopic H3...
2017: PloS One
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