diffuse midline glioma, H3 K27M-mutant
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ID DOID:0080684
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
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ICDO:9385/3
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
NCT03585465 Phase Ib/II Capecitabine + Cyclophosphamide + Nivolumab + Vinblastine Capecitabine + Cyclophosphamide + Vinblastine Cyclophosphamide + Nivolumab + Vinblastine Capecitabine + Nivolumab Nivolumab in Combination With Metronomic Chemotherapy in Paediatrics Refractory / Relapsing Solid Tumors (Metro-PD1) Active, not recruiting FRA | BEL 0
NCT04099797 Phase I C7R-GD2.CAR-T cells Cyclophosphamide + Fludarabine C7R-GD2.CAR T Cells for Patients With GD2-expressing Brain Tumors (GAIL-B) Recruiting USA 0
NCT04196413 Phase I Cyclophosphamide + Fludarabine Cyclophosphamide + Fludarabine + Rituximab GD2 CAR T cells GD2 CAR T Cells in Diffuse Intrinsic Pontine Gliomas (DIPG) & Spinal Diffuse Midline Glioma(DMG) Recruiting USA 0
NCT04655404 Phase I Larotrectinib A Pilot Study of Larotrectinib for Newly-Diagnosed High-Grade Glioma with NTRK Fusion Recruiting USA | NZL | DEU | CAN | AUS 0
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 | CHE 0
NCT04804709 Phase I Panobinostat Non-Invasive Focused Ultrasound (FUS) With Oral Panobinostat in Children With Progressive Diffuse Midline Glioma (DMG) Terminated 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) Active, not 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 | AUS 0
NCT04908176 Phase I Avapritinib + Midazolam A Drug-drug Interaction Study of Avapritinib and Midazolam Completed 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 Active, not recruiting USA | CAN 0
NCT05004116 Phase Ib/II Irinotecan + Repotrectinib + Temozolomide A Study of Repotrectinib in Combination with Chemotherapy in Children and Young Adults with Solid Tumor Cancer Recruiting USA 0
NCT05009992 Phase II Dordaviprone DNX-2401 Combination Therapy for the Treatment of Diffuse Midline Gliomas (PNOC022) Recruiting USA | NZL | NLD | ISR | CHE | AUS 0
NCT05081180 Phase I Avelumab + Lenvatinib Study of Avelumab in Combination With Lenvatinib for Children With Primary CNS Tumors Active, not recruiting FRA | DEU | CAN 1
NCT05096481 Phase II PEP-CMV + Temozolomide PEP-CMV Vaccine Targeting CMV Antigen to Treat Newly Diagnosed Pediatric HGG and DIPG and Recurrent Medulloblastoma Recruiting USA 0
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 | NZL | CAN | AUS 0
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 Terminated USA | ITA | GBR | DEU 0
NCT05476939 Phase III Everolimus Dordaviprone Biological Medicine for Diffuse Intrinsic Pontine Glioma (DIPG) Eradication 2.0 (BIOMEDE 2) Recruiting SWE | FRA | ESP | DNK 0
NCT05478837 Phase I Cyclophosphamide + Fludarabine KIND T cells Genetically Modified Cells (KIND T Cells) for the Treatment of HLA-A*0201-Positive Patients With H3.3K27M-Mutated Glioma (PNOC018) Recruiting USA 0
NCT05500508 Phase Ib/II AMXT1501 + Eflornithine Oral AMXT 1501 Dicaprate in Combination With IV DFMO Terminated USA | AUS 0
NCT05518838 Expanded access OKN-007 Expanded Access to OKN-007 for Patients With Diffuse Midline Glioma, H3 K27-altered No longer 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 Dordaviprone ONC201 in H3 K27M-mutant Diffuse Glioma Following Radiotherapy (the ACTION Study) (ACTION) Recruiting USA | NLD | ITA | ISR | GBR | ESP | DNK | DEU | CHE | CAN | BRA | AUT | AUS | ARG 4
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 Active, not 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
NCT05843253 Phase II Ribociclib + Temozolomide Everolimus + Ribociclib Study of Ribociclib and Everolimus in HGG and DIPG or Ribociclib and Temozolomide in DHG, H3G34-mutant Recruiting USA | NZL | NLD | GBR | DEU | CAN | AUS 0
NCT06011109 Phase Ib/II Bevacizumab APG-157 Treatment of Patients With Recurrent High-Grade Glioma With APG-157 and Bevacizumab Recruiting USA 0
NCT06297512 Phase II Doxorubicin + Temozolomide Evaluate the Role of Anthracycline After Radio Therapy in Patients With Glioblastoma (pGBM). Recruiting ITA 0
NCT06305910 Phase I CD200AR-L CD200AR-L and Allogeneic Tumor Lysate Vaccine Immunotherapy for Recurrent HGG and Newly Diagnosed DMG/DIPG in Children and Young Adults Recruiting USA 0
NCT06413706 Phase II Abemaciclib + Temozolomide Temozolomide A Study Comparing Abemaciclib Plus Temozolomide to Temozolomide Monotherapy in Children and Young Adults With High-grade Glioma Following Radiotherapy Recruiting USA | ROU | NLD | ITA | FRA | ESP | DNK | BEL | AUS 1
NCT06521567 Phase Ib/II Cobolimab + Dostarlimab-gxly A Study of Cobolimab Plus Dostarlimab in Pediatric and Young Adult Participants With Cancer Active, not recruiting USA | ITA | FRA | ESP | DNK | CZE 0
NCT06624371 Phase I Atovaquone Atovaquone Combined with Radiation in Children with Malignant Brain Tumors (AflacBT2303) Recruiting USA 0
NCT06639607 Phase Ib/II Nivolumab + PEP-CMV + Temozolomide PEP-CMV + Nivolumab for Newly Diagnosed Diffuse Midline Glioma/High-grade Glioma and Recurrent Diffuse Midline Glioma/High-grade Glioma, Medulloblastoma, and Ependymoma (PRiME II) Not yet recruiting USA 0
NCT06838676 Phase II ACT001 ACT001 for the Treatment of Diffuse Intrinsic Pontine Gliomas and H3K27-altered High Grade Gliomas Recruiting USA | NZL | DEU | CAN | AUS 0
NCT06894979 Phase I AZD1390 Testing the Addition of an Anti-Cancer Drug, AZD1390, During Radiation Therapy for Newly Diagnosed High Grade Glioma, Diffuse Midline Glioma, or Diffuse Intrinsic Pontine Glioma Recruiting USA 0
NCT06896110 Phase I Azacitidine + Nivolumab Intrathecal Azacitidine and Nivolumab in Patients with Recurrent High-grade Glioma Enrolling by invitation USA 0
NCT07076498 Phase I M032 Engineered HSV-1 M032 for the Treatment of Children and Adults With Newly Diagnosed Diffuse Midline Glioma After Standard of Care Radiation Not yet recruiting USA 0
NCT07223034 Phase I lutetium Lu 177 vipivotide tetraxetan + Temozolomide A Study of 177Lu-PSMA-617 in People With Gliomas Recruiting USA 0