Disease Detail


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ID DOID:80684
Name diffuse midline glioma, H3 K27M-mutant
Definition A histone mutated tumor that is characterized by the presence of histone H3 K27M mutation located throughout the midline structures of the central nervous system.
Source DiseaseOntology.org
Alt Ids
Path disease disease of cellular proliferation cancer cell type cancer high grade glioma histone mutated tumor diffuse midline glioma, H3 K27M-mutant


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Molecular Profile Therapy Indication/Tumor Type Response Type Profile Response Detail
PIK3CA E545K Trametinib diffuse midline glioma, H3 K27M-mutant sensitive detail...
FGFR1 N546K Alpelisib diffuse midline glioma, H3 K27M-mutant sensitive detail...
FGFR1 N546K Infigratinib diffuse midline glioma, H3 K27M-mutant sensitive detail...
Clinical Trial Phase Therapies Title Recruitment Status Covered Countries Other Countries
NCT04655404 Phase I Larotrectinib A Pilot Study of Larotrectinib for Newly-Diagnosed High-Grade Glioma With NTRK Fusion Recruiting USA | GBR | DEU | CAN | AUS 1
NCT04732065 Phase I ONC206 ONC206 for Treatment of Newly Diagnosed or Recurrent Diffuse Midline Gliomas, and Other Recurrent Malignant Brain Tumors (PNOC 023) Recruiting USA 1
NCT04804709 Phase I Panobinostat Non-Invasive Focused Ultrasound (FUS) With Oral Panobinostat in Children With Progressive Diffuse Midline Glioma (DMG) Active, not recruiting USA 0
NCT04808245 Phase I Atezolizumab + H3K27M peptide vaccine + Imiquimod A MultIceNTER Phase I Peptide VaCcine Trial for the Treatment of H3-Mutated Gliomas (INTERCEPT-H3) Recruiting DEU 0
NCT04870944 Phase Ib/II CBL0137 CBL0137 for the Treatment of Relapsed or Refractory Solid Tumors, Including CNS Tumors and Lymphoma Recruiting USA 0
NCT04908176 Phase I Avapritinib + Midazolam A Drug-drug Interaction Study of Avapritinib and Midazolam Recruiting USA 0
NCT04943848 Phase I rHSC-DIPGVax Balstilimab + rHSC-DIPGVax Balstilimab + rHSC-DIPGVax + Zalifrelimab rHSC-DIPGVax Plus Checkpoint Blockade for the Treatment of Newly Diagnosed DIPG and DMG Recruiting USA 0
NCT04978727 Phase I SurVaxM A Pilot Study of SurVaxM in Children Progressive or Relapsed Medulloblastoma, High Grade Glioma, Ependymoma and Newly Diagnosed Diffuse Intrinsic Pontine Glioma Recruiting USA | CAN 0
NCT05009992 Phase II GDC-0084 + ONC201 ONC201 + Panobinostat Combination Therapy for the Treatment of Diffuse Midline Gliomas Active, not recruiting USA | AUS 4
NCT05081180 Phase I Avelumab + Lenvatinib Study of Avelumab in Combination With Lenvatinib for Children With Primary CNS Tumors Recruiting USA | FRA | DEU | CAN 1
NCT05099003 Phase Ib/II Selinexor A Study of the Drug Selinexor With Radiation Therapy in Patients With Newly-Diagnosed Diffuse Intrinsic Pontine (DIPG) Glioma and High-Grade Glioma (HGG) Recruiting USA | CAN 1
NCT05135975 Phase II Cabozantinib A Study of Cabozantinib as a Maintenance Agent to Prevent Progression or Recurrence in High-Risk Pediatric Solid Tumors Recruiting USA 0
NCT05429502 Phase Ib/II Ribociclib + Temozolomide + Topotecan Study of Efficacy and Safety of Ribociclib (LEE011) in Combination With Topotecan and Temozolomide (TOTEM) in Pediatric Patients With Relapsed or Refractory Neuroblastoma and Other Solid Tumors Recruiting USA | ITA | FRA | ESP | DEU | AUS 1
NCT05476939 Phase III Everolimus ONC201 Biological Medicine for Diffuse Intrinsic Pontine Glioma (DIPG) Eradication 2.0 (BIOMEDE 2) Recruiting FRA 0
NCT05500508 Phase Ib/II AMXT1501 + Eflornithine Oral AMXT 1501 Dicaprate in Combination With IV DFMO Recruiting USA | AUS 0
NCT05518838 Expanded access OKN-007 Expanded Access to OKN-007 for Patients With Diffuse Midline Glioma, H3 K27-altered Available USA 0
NCT05544526 Phase I GD2 CAR T cells CAR T Cells to Target GD2 for DMG (CARMIGO) Recruiting GBR 0
NCT05580562 Phase III ONC201 ONC201 in H3 K27M-mutant Diffuse Glioma Following Radiotherapy (the ACTION Study) (ACTION) Recruiting USA | ITA | GBR | ESP | DEU | CAN | AUS 5
NCT05768880 Phase I SC-CAR4BRAIN Study of B7-H3, EGFR806, HER2, And IL13-Zetakine (Quad) CAR T Cell Locoregional Immunotherapy For Pediatric Diffuse Intrinsic Pontine Glioma, Diffuse Midline Glioma, And Recurrent Or Refractory Central Nervous System Tumors Recruiting USA 0
NCT05835687 Phase I B7-H3 CAR T cells Loc3CAR: Locoregional Delivery of B7-H3-CAR T Cells for Pediatric Patients With Primary CNS Tumors Recruiting USA 0
NCT06011109 Phase Ib/II Bevacizumab APG-157 Treatment of Patients With Recurrent High-Grade Glioma With APG-157 and Bevacizumab Not yet recruiting USA 0