diffuse midline glioma, H3 K27M-mutant
diseaseOn this page
Summary
diffuse midline glioma, H3 K27M-mutant (MONDO:0957196) is a cancer and 27 clinical trials. Top therapeutic interventions include everolimus, atovaquone, and eflornithine. A subtype of diffuse midline glioma, H3 K27-altered — broader associated-gene and molecular evidence is on the parent page (see Disease family below).
At a glance
- Classification: Cancer
- Clinical trials: 27
Clinical features
No curated clinical features (Orphanet) for this disease.
Identifiers
Disease identifiers
| Field | Value |
|---|---|
| Canonical name | diffuse midline glioma, H3 K27M-mutant |
| Mondo ID | MONDO:0957196 |
| DOID | DOID:0080684 |
| NCIT | C129309 |
| UMLS | C4289688 |
| MedGen | 927220 |
| GARD | 0026785 |
| Is cancer (heuristic) | yes |
Disease family
This is a subtype of diffuse midline glioma, H3 K27-altered. Genetic, therapeutic, and trial evidence is largely curated at the broader-term level — see the parent page for the associated-gene cohort and molecular evidence.
Classification path: cancer › nervous system cancer › central nervous system cancer › brain cancer › infratentorial cancer › brainstem cancer › brain stem glioma › childhood brain stem glioma › diffuse intrinsic pontine glioma › diffuse midline glioma, H3 K27-altered › diffuse midline glioma, H3 K27M-mutant
Genetics & variants
GWAS landscape
No GWAS associations recorded — common-variant (GWAS) studies don’t cover this disease (typical for Mendelian / rare diseases). See the curated gene cohort and Mendelian overlap below.
Variant details and genetic-evidence tiers
No tiered GWAS variants or ClinVar records for this disease.
Genes & proteins
No associated-gene cohort resolved for this disease. Atlas builds the molecular and therapeutic sections — associated genes, protein families, druggability, pathways, interactions, and drug associations — by aggregating over a disease’s associated genes (resolved via GWAS / GenCC / ClinVar / CIViC), and none resolved here. This is expected for antibody-mediated, autoimmune, or otherwise non-gene-defined conditions; the curated evidence for this disease is its clinical features, GWAS susceptibility, and clinical trials (above).
Function
No pathway enrichment — requires an associated-gene cohort.
Therapeutics
No druggable-target or therapeutic data for this disease’s cohort.
Clinical trials & evidence
Clinical trials
Clinical trials: 27.
Phase distribution (across all retrieved trials)
| Phase | Trials |
|---|---|
| PHASE1 | 13 |
| PHASE2 | 5 |
| Not specified | 5 |
| PHASE1/PHASE2 | 3 |
| PHASE3 | 1 |
Top trials by phase / activity
| NCT | Phase | Status | Title |
|---|---|---|---|
| NCT05476939 | PHASE3 | RECRUITING | Biological Medicine for Diffuse Intrinsic Pontine Glioma (DIPG) Eradication 2.0 |
| NCT05009992 | PHASE2 | RECRUITING | Combination Therapy for the Treatment of Diffuse Midline Gliomas |
| NCT05077735 | PHASE2 | RECRUITING | Stereotactic Biopsy Split-Course Radiation Therapy in Diffuse Midline Glioma, SPORT-DMG Study |
| NCT05843253 | PHASE2 | RECRUITING | Study of Ribociclib and Everolimus in HGG and DIPG or Ribociclib and Temozolomide in DHG, H3G34-mutant |
| NCT06161974 | PHASE2 | RECRUITING | Study of Olutasidenib and Temozolomide in HGG |
| NCT07389278 | PHASE1/PHASE2 | NOT_YET_RECRUITING | Combination ADI-PEG 20, TMZ, and RT for Treatment of Newly Diagnosed High-grade Glioma (HGG) |
| NCT07589257 | PHASE2 | RECRUITING | A Study of VRT106 in Combination With Radiotherapy in Adult Patients With Diffuse Midline Glioma / Diffuse Intrinsic Pontine Glioma |
| NCT02960230 | PHASE1/PHASE2 | COMPLETED | H3.3K27M Peptide Vaccine With Nivolumab for Children With Newly Diagnosed DIPG and Other Gliomas |
| NCT05500508 | PHASE1/PHASE2 | TERMINATED | Oral AMXT 1501 Dicaprate in Combination With IV DFMO |
| NCT04541082 | PHASE1 | RECRUITING | Phase I Study of Oral ONC206 in Recurrent and Rare Primary Central Nervous System Neoplasms |
| NCT04943848 | PHASE1 | RECRUITING | rHSC-DIPGVax Plus Checkpoint Blockade for the Treatment of Newly Diagnosed DIPG and DMG |
| NCT05478837 | PHASE1 | ACTIVE_NOT_RECRUITING | Genetically Modified Cells (KIND T Cells) for the Treatment of HLA-A*0201-Positive Patients With H3.3K27M-Mutated Glioma |
| NCT05544526 | PHASE1 | RECRUITING | CAR T Cells to Target GD2 for DMG |
| NCT05762419 | PHASE1 | RECRUITING | FUS Etoposide for DMG |
| NCT05835687 | PHASE1 | RECRUITING | Loc3CAR: Locoregional Delivery of B7-H3-CAR T Cells for Pediatric Patients With Primary CNS Tumors |
| NCT06305910 | PHASE1 | RECRUITING | CD200AR-L and Allogeneic Tumor Lysate Vaccine Immunotherapy for Recurrent HGG and Newly Diagnosed DMG/DIPG in Children and Young Adults |
| NCT06357377 | PHASE1 | NOT_YET_RECRUITING | A Study of the Safety, Dosing, and Delivery of NEO100 in Patients With Pediatric Brain Tumors |
| NCT06624371 | PHASE1 | RECRUITING | Atovaquone Combined With Radiation in Children With Malignant Brain Tumors |
| NCT01922076 | PHASE1 | COMPLETED | Adavosertib and Local Radiation Therapy in Treating Children With Newly Diagnosed Diffuse Intrinsic Pontine Gliomas |
| NCT04771897 | PHASE1 | TERMINATED | A Study of BXQ-350 in Children With Newly Diagnosed Diffuse Intrinsic Pontine Glioma (DIPG) or Diffuse Midline Glioma (DMG) |
| NCT04804709 | PHASE1 | TERMINATED | Non-Invasive Focused Ultrasound (FUS) With Oral Panobinostat in Children With Progressive Diffuse Midline Glioma (DMG) |
| NCT06806228 | PHASE1 | SUSPENDED | Phase I Pilot Study to Evaluate the Anti-glioblastoma Effect of S-Gboxin in Standard Treatment of Glioblastoma/Diffuse Midline Glioma and Response to Treatment (Regardless of Mutation Status) |
| NCT03101813 | Not specified | RECRUITING | International Diffuse Intrinsic Pontine Glioma (DIPG)/Diffuse Midline Glioma (DMG) Registry and Repository |
| NCT05259605 | Not specified | RECRUITING | Observational Study for Assessing Treatment and Outcome of Patients With Primary Brain Tumours Using cIMPACT-NOW and 2021 WHO Classification |
| NCT05839379 | Not specified | RECRUITING | Targeted Pediatric High-Grade Glioma Therapy |
| NCT06093165 | Not specified | NOT_YET_RECRUITING | RE-irradiation of Diffuse MIdline Glioma paTients |
| NCT05518838 | Not specified | NO_LONGER_AVAILABLE | Expanded Access to OKN-007 for Patients With Diffuse Midline Glioma, H3 K27-altered |
Drugs tested across these trials (top 30)
| Molecule | Max phase | Trials referencing |
|---|---|---|
| EVEROLIMUS | 4 | 2 |
| ATOVAQUONE | 4 | 1 |
| EFLORNITHINE | 4 | 1 |
| OLUTASIDENIB | 4 | 1 |
| RIBOCICLIB | 4 | 1 |
| DORDAVIPRONE | 3 | 2 |
| BALSTILIMAB | 3 | 1 |
| DISUFENTON SODIUM | 3 | 1 |
| PEGARGIMINASE | 3 | 1 |
| ADAVOSERTIB | 2 | 1 |
| PAXALISIB | 2 | 1 |
| PERILLYL ALCOHOL | 2 | 1 |
| TASADENOTUREV | 2 | 1 |
| ZALIFRELIMAB | 2 | 1 |
| AMXT-1501 | 1 | 1 |
| ONC-206 | 1 | 1 |
| CHEMBL2369717 | 0 | 1 |
| CHEMBL344227 | 0 | 1 |
| CHEMBL471792 | 0 | 1 |
| CHEMBL4787699 | 0 | 1 |