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OncoMatch/Clinical Trials/NCT05009992

Combination Therapy for the Treatment of Diffuse Midline Gliomas

Is NCT05009992 recruiting? Yes, currently enrolling (May 2026). This Phase 2 trial studies multiple treatments including ONC201 and Paxalisib for diffuse intrinsic pontine glioma.

Phase 2RecruitingUniversity of California, San FranciscoNCT05009992Data as of May 2026

Treatment: ONC201 · Paxalisib · DNX-2401This phase II trial determines if the combination of ONC201 with different drugs is effective for treating participants with diffuse midline gliomas (DMGs). Despite years of research, little to no progress has been made to improve outcomes for participants with DMGs, and there are few treatment options. This trial will utilize an adaptive platform design in that the different treatment arms for each cohort will be opened and closed based on ongoing preclinical investigation as well as evolving outcome data from the trial. Novel agents will be continuously added to this study as pre-clinical data emerge to suggest additive or synergistic activity when combined ONC201. Should a novel agent not have an RP2D at the time of incorporation into this study, a phase 1 lead-in will be performed prior to initiation of combination therapy (via study amendment).

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Extracted eligibility criteria

Cancer type

Diffuse Intrinsic Pontine Glioma

Glioblastoma

Biomarker criteria

Required: BRAF V600E

BRAFV600E

Required: PDGFRA mutation

PDGFRA (DNA point mutation or amplification with >=5 copy numbers)

Required: PDGFRA amplification (>=5 copy numbers)

PDGFRA (DNA point mutation or amplification with >=5 copy numbers)

Required: FGFR1 mutation

FGFR1 (DNA point mutation, gene fusions, or amplification with >=5 copy numbers)

Required: FGFR1 fusion

FGFR1 (DNA point mutation, gene fusions, or amplification with >=5 copy numbers)

Required: FGFR1 amplification (>=5 copy numbers)

FGFR1 (DNA point mutation, gene fusions, or amplification with >=5 copy numbers)

Required: NF1 mutation

NF1

Allowed: H3F3A (H3 K27M) K27M

diffuse midline glioma Histone 3 lysine 27 - mutant (H3K27M)

Allowed: HIST1H3B K27M

diffuse midline glioma Histone 3 lysine 27 - mutant (H3K27M)

Allowed: H3F3A (H3 K27M) wild-type

WHO grade III and IV H3 wildtype gliomas

Disease stage

Required: Stage WHO GRADE III, WHO GRADE IV (WHO)

Excluded: Stage GRADE II

WHO grade III and IV H3 wildtype gliomas

Prior therapy

Cannot have received: radiation therapy

Exception: Cohorts 1A/1B, 4A^1/4B^1, 5^1 (pre-radiation) only

Prior exposure to radiation therapy

Cannot have received: re-irradiation

Exception: Cohorts 3A/3B, 4A^3/4B^3, 5^3 (progression) only

Prior exposure to re-irradiation for tumor progression

Cannot have received: ONC201 (upfront setting, Chimerix trials except PNOC022/expanded access) (ONC201)

Exception: Participants who received ONC201 as part of PNOC022 or expanded access programs are allowed

Participants who participated in trials investigating ONC201 in the upfront setting will not be eligible at any time, with the exception if participants received ONC201 as part of PNOC022 or other expanded access programs such as German sources of ONC201.

Lab requirements

Blood counts

ANC >= 750/mm^3; Platelet count >= 75,000/mm^3 (transfusion independent, not receiving platelet transfusions for at least 7 days prior to enrollment)

Kidney function

Creatinine clearance or radioisotope GFR >= 70 mL/min/1.73 m^2 OR serum creatinine within normal limits for age

Liver function

Bilirubin <= 1.5 x ULN for age (Cohort 6: <= 3 x ULN, Gilbert's syndrome <= 6 x ULN or direct bilirubin <= 3 x ULN); ALT <= 3 x ULN (Cohort 6: <= 5 x ULN); AST <= 5 x ULN (Cohort 6: <= 5 x ULN); Serum albumin >= 2 g/dL

Cardiac function

No history of congestive heart failure or family history of long QT syndrome; QTC < 470 msec; Shortening fraction >= 27% if at risk; No exercise intolerance due to pulmonary insufficiency; pulse oximetry > 92% on room air

See cohort-specific organ function requirements above.

Structured fields extracted by AI. May contain errors — verify against the official protocol.

US trial sites

  • University of Alabama at Birmingham · Birmingham, Alabama
  • Children's Hospital Los Angeles · Los Angeles, California
  • University of California, San Diego / Rady Children's Hospital, San Diego · San Diego, California
  • University of California, San Francisco · San Francisco, California
  • Children's National Hospital · Washington D.C., District of Columbia

Showing up to 5 US sites. See all sites on ClinicalTrials.gov →

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