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| ID | DOID:0080684 |
| Name | diffuse midline glioma, H3 K27-altered |
| Definition | A histone mutated tumor that is characterized by loss of histone H3 p.K28me3 (K27me3) methylation and located throughout the midline structures of the central nervous system. Methylation loss may be due to p.K28M (K27M) mutation in one of the histone H3 isoforms (H3.1, H3.2, or H3.3), overexpression of EZHIP, or mutation of the EGFR gene. |
| Source | DiseaseOntology.org |
| Alt Ids | |
| Xrefs |
ICDO:9385/3
|
| Path | disease disease of cellular proliferation cancer cell type cancer high grade glioma histone mutated tumor diffuse midline glioma, H3 K27-altered |
| Molecular Profile | Therapy | Indication/Tumor Type | Response Type | Profile Response Detail |
|---|---|---|---|---|
| PIK3CA E545K | Trametinib | diffuse midline glioma, H3 K27-altered | sensitive | detail... |
| FGFR1 N546K | Alpelisib | diffuse midline glioma, H3 K27-altered | sensitive | detail... |
| FGFR1 N546K | Infigratinib | diffuse midline glioma, H3 K27-altered | 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) | Terminated | 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 | NLD | 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) | Active, not 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 | 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 | Active, not 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) | 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 |
| NCT06946680 | Phase I | Autologous CXCR2-modified CD70 CAR-T cells | IL-8 Receptor-modified CD70 CAR T Cell Therapy in CD70+ Newly Diagnosed and Recurrent Pediatric High-grade Glioma (pHGG) and Newly Diagnosed Diffuse Intrinsic Pontine Glioma (ndDIPG) (Peds IMPACT) | Not yet recruiting | 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 | 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 |
| NCT07389278 | Phase Ib/II | ADI-PEG 20 + Temozolomide | Combination ADI-PEG 20, TMZ, and RT for Treatment of Newly Diagnosed High-grade Glioma (HGG) | Not yet recruiting | USA | 0 |