Diffuse Intrinsic Pontine Glioma

A rare, aggressive brain tumor that forms in the GLIAL CELLS in the PONS.

Also Known As:

Brain Tumor, DIPG; DIPG Brain Tumor; DIPG Brain Tumors; DIPG, Diffuse Intrinsic Pontine Glioma

Networked: 536 relevant articles (23 outcomes, 71 trials/studies)

Relationship Network

Disease Context: Research Results

Neoplasms: 1324753
- Neoplasms by Site: 4
  - Nervous System Neoplasms: 235
    - Central Nervous System Neoplasms: 818
      - Brain Neoplasms: 25077
        Infratentorial Neoplasms: 208
        Brain Stem Neoplasms: 260
        Diffuse Intrinsic Pontine Glioma: 536
- Neoplasms by Histologic Type: 2
Nervous System Diseases: 14178
- Central Nervous System Diseases: 4213
  - Brain Diseases: 15694
    - Brain Neoplasms: 25077
      - Infratentorial Neoplasms: 208
        Brain Stem Neoplasms: 260
        Diffuse Intrinsic Pontine Glioma: 536
- Nervous System Neoplasms: 235
  - Central Nervous System Neoplasms: 818
    - Brain Neoplasms: 25077
      - Infratentorial Neoplasms: 208
        Brain Stem Neoplasms: 260
        Diffuse Intrinsic Pontine Glioma: 536

Related Diseases

1.	Glioma (Gliomas)
2.	Neoplasms (Cancer)
3.	Brain Neoplasms (Brain Tumor)
4.	Glioblastoma (Glioblastoma Multiforme)
5.	Hydrocephalus (Hydrocephaly)

Experts

1.	van Vuurden, Dannis G: 30 articles (01/2022 - 01/2013)
2.	Fouladi, Maryam: 28 articles (12/2022 - 03/2014)
3.	Broniscer, Alberto: 25 articles (01/2022 - 11/2010)
4.	Hulleman, Esther: 25 articles (01/2022 - 07/2011)
5.	Nazarian, Javad: 24 articles (01/2022 - 05/2012)
6.	Kaspers, Gertjan J L: 23 articles (06/2021 - 02/2013)
7.	Bouffet, Eric: 22 articles (01/2022 - 02/2008)
8.	Warren, Katherine E: 22 articles (01/2022 - 10/2008)
9.	Hawkins, Cynthia: 21 articles (01/2022 - 03/2010)
10.	Monje, Michelle: 21 articles (01/2022 - 07/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Diffuse Intrinsic Pontine Glioma:

1.	dipinacoline glutamateIBA 01/01/2017 - "Targeting epigenetic changes in diffuse intrinsic pontine glioma (DIPG) may provide a novel treatment option for patients. " 01/01/2023 - "Concomitant chemoradiation followed by repeat (dose-deescalated) irradiation has become standard of care in treating childhood diffuse intrinsic pontine glioma (DIPG) during first line treatment and at first progression. " 01/01/2023 - "This study aimed to describe 11C-methionine (11C-MET) PET imaging characteristics in patients with paediatric diffuse intrinsic pontine glioma (DIPG) and correlate them with survival and H3 K27M mutation status. " 01/01/2020 - "In this study, we further validated the feasibility of this method by modeling pediatric diffuse intrinsic pontine glioma (DIPG) and two types of adult GBM (GBM1, GBM551), in mice with intact immune systems and immunodeficient mice. " 01/01/2020 - "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. "
2.	nimotuzumabIBA 11/01/2014 - "Phase 2 study of safety and efficacy of nimotuzumab in pediatric patients with progressive diffuse intrinsic pontine glioma." 04/01/2011 - "Response to nimotuzumab in a child with a progressive diffuse intrinsic pontine glioma." 05/01/2019 - "Nimotuzumab and radiotherapy for treatment of newly diagnosed diffuse intrinsic pontine glioma (DIPG): a phase III clinical study." 08/01/2013 - "Craniospinal irradiation with concurrent temozolomide and nimotuzumab in a child with primary metastatic diffuse intrinsic pontine glioma. " 01/01/2022 - "Follow-up ranged from 2.4-66 mo with four studies reporting diffuse intrinsic pontine glioma (DIPG) patients and only one study reporting nimotuzumab usage in pediatric high-grade glioma (HGG) patients with better outcome in HGG patients than DIPG. "
3.	Histone Deacetylase InhibitorsIBA 01/01/2018 - "OBJECTIVE The pan-histone deacetylase inhibitor panobinostat has preclinical efficacy against diffuse intrinsic pontine glioma (DIPG), and the oral formulation has entered a Phase I clinical trial. " 01/01/2022 - "A phase I/II trial of vorinostat (suberoylanilide hydroxamic acid), an oral histone deacetylase inhibitor, was conducted in children with newly diagnosed diffuse intrinsic pontine glioma (DIPG) through the Children's Oncology Group (COG) to: 1) determine the recommended phase II dose (RP2D) of vorinostat given concurrently with radiation therapy; 2) document the toxicities of continuing vorinostat as maintenance therapy after radiation; and 3) to determine the efficacy of this regimen by comparing the risk of progression or death with a historical model from past COG trials. " 01/01/2022 - "We found that pediatric glioblastoma (PED-GBM) cell lines from diffuse intrinsic pontine glioma (DIPG) carrying the H3K27M mutation or from diffuse hemispheric glioma expressing the H3G34R mutation are sensitive to the combination of vorinostat (a histone deacetylase inhibitor) and PARP-1 inhibitors. "
4.	Chimeric Antigen ReceptorsIBA 01/01/2023 - "HER2 chimeric antigen receptor T cell immunotherapy is an effective treatment for diffuse intrinsic pontine glioma." 02/01/2023 - "Based on adoptive cellular therapy's clinical success against childhood leukemia and the preclinical efficacy against pediatric CNS tumors, chimeric antigen receptor (CAR) T cells offer hope of improving outcomes for recurrent tumors and universally fatal diseases such as diffuse intrinsic pontine glioma (DIPG). "
5.	Bevacizumab (Avastin)FDA Link 01/01/2022 - "Does a Bevacizumab-based regime have a role in the treatment of children with diffuse intrinsic pontine glioma? " 01/01/2013 - "We report on 2 patients with diffuse intrinsic pontine glioma treated with radiation, followed by temozolamide 200 mg/m/d for 5 days every 28 days and bevacizumab 10 mg/kg/dose every 14 days. " 01/01/2013 - "Prolonged survival after treatment of diffuse intrinsic pontine glioma with radiation, temozolamide, and bevacizumab: report of 2 cases." 01/01/2023 - "Bevacizumab for the early clinical progression of diffuse intrinsic pontine glioma." 02/01/2015 - "Patterns of progression in pediatric patients with high-grade glioma or diffuse intrinsic pontine glioma treated with Bevacizumab-based therapy at diagnosis."
6.	PanobinostatIBA 11/02/2023 - "PNOC015: Repeated convection-enhanced delivery of MTX110 (aqueous panobinostat) in children with newly diagnosed diffuse intrinsic pontine glioma." 01/01/2020 - "Characterizing the pharmacokinetics of panobinostat in a non-human primate model for the treatment of diffuse intrinsic pontine glioma." 01/01/2017 - "Pre-Clinical Study of Panobinostat in Xenograft and Genetically Engineered Murine Diffuse Intrinsic Pontine Glioma Models." 06/01/2015 - "Panobinostat active against diffuse intrinsic pontine glioma." 08/01/2023 - "A Phase I Trial of Panobinostat in Children with Diffuse Intrinsic Pontine Glioma: A Report from the Pediatric Brain Tumor Consortium (PBTC-047)."
7.	Gemcitabine FDA Link 01/01/2018 - "Correction to: A phase I/II study of gemcitabine during radiotherapy in children with newly diagnosed diffuse intrinsic pontine glioma." 11/01/2017 - "A phase I/II study of gemcitabine during radiotherapy in children with newly diagnosed diffuse intrinsic pontine glioma." 11/01/2017 - "The purpose of this phase I/II, open-label, single-arm trial is to investigate the safety, tolerability, maximum tolerated dose and preliminary efficacy of the potential radiosensitizer gemcitabine, administered concomitantly to radiotherapy, in children with newly diagnosed diffuse intrinsic pontine glioma (DIPG). "
8.	Vascular Endothelial Growth Factor A (Vascular Endothelial Growth Factor)IBA 06/01/2017 - "Measurable Supratentorial White Matter Volume Changes in Patients with Diffuse Intrinsic Pontine Glioma Treated with an Anti-Vascular Endothelial Growth Factor Agent, Steroids, and Radiation." 01/01/2018 - "Inadequate tumor uptake of the vascular endothelial growth factor antibody bevacizumab could explain lack of effect in diffuse intrinsic pontine glioma. " 01/01/2016 - "The role of the VEGF inhibitor bevacizumab in the treatment of diffuse intrinsic pontine glioma (DIPG) is unclear. " 06/01/2021 - "This study investigates the safety, tolerability, and preliminary efficacy of combined treatment with VEGF inhibitor bevacizumab, topoisomerase I inhibitor irinotecan, and EGFR inhibitor erlotinib in children with progressive diffuse intrinsic pontine glioma (DIPG). "
9.	Vorinostat FDA Link 01/01/2022 - "Phase I/II trial of vorinostat and radiation and maintenance vorinostat in children with diffuse intrinsic pontine glioma: A Children's Oncology Group report." 01/01/2022 - "A phase I/II trial of vorinostat (suberoylanilide hydroxamic acid), an oral histone deacetylase inhibitor, was conducted in children with newly diagnosed diffuse intrinsic pontine glioma (DIPG) through the Children's Oncology Group (COG) to: 1) determine the recommended phase II dose (RP2D) of vorinostat given concurrently with radiation therapy; 2) document the toxicities of continuing vorinostat as maintenance therapy after radiation; and 3) to determine the efficacy of this regimen by comparing the risk of progression or death with a historical model from past COG trials. " 01/01/2022 - "We found that pediatric glioblastoma (PED-GBM) cell lines from diffuse intrinsic pontine glioma (DIPG) carrying the H3K27M mutation or from diffuse hemispheric glioma expressing the H3G34R mutation are sensitive to the combination of vorinostat (a histone deacetylase inhibitor) and PARP-1 inhibitors. "
10.	Irinotecan (Camptosar)FDA LinkGeneric 04/27/2020 - "Characterization of the Blood-Brain Barrier Integrity and the Brain Transport of SN-38 in an Orthotopic Xenograft Rat Model of Diffuse Intrinsic Pontine Glioma." 06/01/2021 - "A phase I/II study of bevacizumab, irinotecan and erlotinib in children with progressive diffuse intrinsic pontine glioma." 06/01/2021 - "This study investigates the safety, tolerability, and preliminary efficacy of combined treatment with VEGF inhibitor bevacizumab, topoisomerase I inhibitor irinotecan, and EGFR inhibitor erlotinib in children with progressive diffuse intrinsic pontine glioma (DIPG). "

Therapies and Procedures

1.	Radiotherapy 01/01/2022 - "Hypofractionated Radiation Therapy For Diffuse Intrinsic Pontine Glioma: A Noninferiority Randomized Study Including 253 Children." 01/01/2018 - "Eligible patients were aged 3-21 years and had diffuse intrinsic pontine glioma as diagnosed by consensus of a multidisciplinary paediatric neuro-oncology team; a Lansky (patients <16 years of age) or Karnofsky (patients ≥16 years) performance score of at least 50 at study entry; a minimum weight of 8 kg; and had completed external beam radiation therapy (54·0-59·4 Gy at 1·8 Gy per fraction over 30-33 fractions) at least 4 weeks but no more than 14 weeks before enrolment. " 04/01/2023 - "Radiotherapy for Diffuse Intrinsic Pontine Glioma: Insufficient but Indispensable." 01/01/2023 - "Suggestions for Escaping the Dark Ages for Pediatric Diffuse Intrinsic Pontine Glioma Treated with Radiotherapy: Analysis of Prognostic Factors from the National Multicenter Study." 01/01/2021 - "Model-based evaluation of image-guided fractionated whole-brain radiation therapy in pediatric diffuse intrinsic pontine glioma xenografts."
2.	Drug Therapy (Chemotherapy) 06/01/2011 - "The biology of diffuse intrinsic pontine glioma remains poorly understood and the dismal prognosis has not changed despite various attempts to add chemotherapy to standard radiation. " 04/01/2021 - "Effective treatment of diffuse intrinsic pontine glioma (DIPG) remains a formidable challenge due to inadequate penetration of the blood-brain barrier (BBB) by systemically administered chemotherapies. " 01/01/2020 - "Hundreds of systemic chemotherapy trials in diffuse intrinsic pontine glioma (DIPG) have not improved survival, potentially due to lack of intratumoral penetration, which has not previously been assessed in humans. " 10/01/2019 - "Children with diffuse intrinsic pontine glioma (DIPG) succumb to disease within 2 years of diagnosis despite treatment with ionizing radiation (IR) and/or chemotherapy. " 10/01/2018 - "The use of MRgFUS in the brainstem has not previously been shown but could provide value in the treatment of tumours such as Diffuse Intrinsic Pontine Glioma (DIPG) where the intact BBB has contributed to the limited success of chemotherapy. "
3.	Therapeutics 01/01/2023 - "First-in-human sonodynamic therapy with ALA for pediatric diffuse intrinsic pontine glioma: a phase 1/2 study using low-intensity focused ultrasound : Technical communication." 01/01/2022 - "Targeted therapy for pediatric diffuse intrinsic pontine glioma: a single-center experience." 01/01/2022 - "Oncolytic Viral Therapy Is Feasible in Diffuse Intrinsic Pontine Glioma." 01/01/2020 - "Targeting ALK2: An Open Science Approach to Developing Therapeutics for the Treatment of Diffuse Intrinsic Pontine Glioma." 01/01/2020 - "Combined Therapy of AXL and HDAC Inhibition Reverses Mesenchymal Transition in Diffuse Intrinsic Pontine Glioma."
4.	Re-Irradiation 01/01/2019 - "A Phase 1/2 Trial of Reirradiation for Diffuse Intrinsic Pontine Glioma." 01/01/2023 - "Second course of re-irradiation in pediatric diffuse intrinsic pontine glioma : A case study." 03/01/2022 - "Sequential Diffusion Tensor Imaging and Magnetic Resonance Spectroscopy in Patients Undergoing Reirradiation for Progressive Diffuse Intrinsic Pontine Glioma." 04/30/2021 - "Consecutive patients of recurrent diffuse intrinsic pontine glioma treated with re-irradiation (January 2015-September 2019) were reviewed retrospectively to describe the clinical-response-based approach followed for the dose and volume decision. " 04/30/2021 - "We present our institutional approach for re-irradiation in diffuse intrinsic pontine glioma and their outcomes. "
5.	Immunotherapy 08/24/2022 - "Immunogenic Cell Death Enhances Immunotherapy of Diffuse Intrinsic Pontine Glioma: From Preclinical to Clinical Studies." 11/08/2023 - "Clinical experience with immunotherapy in patients with diffuse intrinsic pontine glioma." 01/18/2023 - "Immune Microenvironment and Immunotherapies for Diffuse Intrinsic Pontine Glioma." 01/01/2023 - "A combined immunopeptidomics, proteomics, and cell surface proteomics approach to identify immunotherapy targets for diffuse intrinsic pontine glioma." 01/01/2019 - "Characterization of the immune microenvironment of diffuse intrinsic pontine glioma: implications for development of immunotherapy."