Actively Recruiting

Phase 1
Age: 2Years - 21Years
All Genders
NCT04732065

ONC206 for Treatment of Newly Diagnosed, Recurrent Diffuse Midline Gliomas, and Other Recurrent Malignant CNS Tumors

Led by Sabine Mueller, MD, PhD · Updated on 2026-03-17

208

Participants Needed

6

Research Sites

309 weeks

Total Duration

On this page

Sponsors

S

Sabine Mueller, MD, PhD

Lead Sponsor

J

Jazz Pharmaceuticals

Collaborating Sponsor

AI-Summary

What this Trial Is About

This phase I trial studies the effects and best dose of ONC206 alone or in combination with radiation therapy in treating patients with diffuse midline gliomas that is newly diagnosed or has come back (recurrent) or other recurrent primary malignant CNS tumors. ONC206 is a recently discovered compound that may stop cancer cells from growing. This drug has been shown in laboratory experiments to kill brain tumor cells by causing a so called "stress response" in tumor cells. This stress response causes cancer cells to die, but without affecting normal cells. ONC206 alone or in combination with radiation therapy may be effective in treating newly diagnosed or recurrent diffuse midline gliomas and other recurrent primary malignant CNS tumors.

CONDITIONS

Official Title

ONC206 for Treatment of Newly Diagnosed, Recurrent Diffuse Midline Gliomas, and Other Recurrent Malignant CNS Tumors

Who Can Participate

Age: 2Years - 21Years
All Genders

Eligibility Criteria

Eligible

You may qualify if you...

  • Children and young adults aged 2 to 21 years with DMG, H3K27 altered, who have completed at least one prior therapy including focal radiation (Arm A).
  • Newly diagnosed children and young adults aged 2 and older with DMG, H3K27 altered, including spinal cord DMGs (Arm B).
  • Children and young adults aged 2 to 21 years with progressing DMG, H3K27 altered, who have not yet been treated for progression and are candidates for re-irradiation (Arm C).
  • Children and young adults aged 2 to 21 years with recurrent primary malignant CNS tumors, excluding DMGs, with progression and no prior treatment for progression (Arm D).
  • Tumor tissue confirmation consistent with diagnosis is required.
  • Participants must have recovered from acute side effects of prior therapy.
  • Specific washout periods from prior therapies must be met before starting study treatment.
  • Participants must be on stable or decreasing steroid doses for at least 3 days before registration.
  • Adequate blood counts and organ function tests within specified limits.
  • Well-controlled seizure disorder allowed.
  • Agreement to use adequate contraception during study participation for females of child-bearing potential and males.
  • Karnofsky or Lansky performance score of 50 or higher.
  • Willingness to provide adequate tumor tissue.
  • Ability to understand and sign informed consent/assent.
  • Enrollment on PNOC COMP if available.
Not Eligible

You will not qualify if you...

  • Patients with thalamic or cerebellar DMG H3K27 altered without concurrent therapy other than temozolomide (Arms A & B).
  • Prior participation in ONC201 trials in the upfront setting (Arms C & D).
  • Current use of other investigational or anti-cancer drugs.
  • Known immune system disorders requiring systemic immunosuppressive therapy.
  • Uncontrolled infections.
  • Pregnant or breastfeeding females; positive pregnancy test before therapy.
  • Active illicit drug use or alcoholism.
  • History of allergic reactions to similar compounds as ONC206.
  • Inability to follow study procedures due to language, psychological, or cognitive issues.
  • Illnesses affecting ONC206 absorption.
  • Use of strong CYP3A4, 2D6, 1A2, 2C9, or 2C19 inhibitors or inducers within 14 days prior and during study.

AI-Screening

AI-Powered Screening

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Trial Site Locations

Total: 6 locations

1

University of California, San Francisco

San Francisco, California, United States, 94143

Actively Recruiting

2

Emory University

Atlanta, Georgia, United States, 30322

Actively Recruiting

3

University of Michigan

Ann Arbor, Michigan, United States, 48109

Actively Recruiting

4

Children's Hospital of Philadelphia

Philadelphia, Pennsylvania, United States, 19104

Actively Recruiting

5

Princess Máxima Center for Pediatric Oncology

Utrecht, Netherlands, 3584

Actively Recruiting

6

The University Children's Hospital in Zurich

Zurich, Canton of Zurich, Switzerland, 8032

Actively Recruiting

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Research Team

P

PNOC Operations

CONTACT

How is the study designed?

Study Type

INTERVENTIONAL

Masking

NONE

Allocation

NON_RANDOMIZED

Model

PARALLEL

Primary Purpose

TREATMENT

Number of Arms

4

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