Study of Ribociclib and Everolimus in HGG and DIPG or Ribociclib and Temozolomide in DHG, H3G34-mutant

Conditions

• Anaplastic Astrocytoma
• Diffuse Hemispheric Glioma, H3 G34-Mutant
• Diffuse Intrinsic Pontine Glioma
• Diffuse Midline Glioma, H3 K27M-Mutant
• Glioblastoma
• Glioblastoma Multiforme
• High Grade Glioma
• Metastatic Brain Tumor
• WHO Grade III Glioma
• WHO Grade IV Glioma

Eligibility Criteria

Sex

ALL

Age

12 Months - 39 Years

Healthy Volunteers

Not accepted

Interventions

• Ribociclib — DRUG
  Ribociclib PO qd on days 1-21
• Everolimus — DRUG
  Everolimus PO qd on days 1-28
• Temozolomide (TMZ) — DRUG
  Temozolomide PO qd on days 1-5 for the first 13 cycles

Study Details

The goal of this study is to determine the efficacy of the 1) ribociclib and everolimus to treat pediatric and young adult patients newly diagnosed with a high-grade glioma (HGG), including DIPG, that have genetic changes in pathways (cell cycle, PI3K/mTOR) that these drugs target or 2) ribociclib and temozolomide to treat pediatric and young adult patients newly diagnosed with diffuse hemispheric glioma (DHG), H3G34-mutant. The main question the study aims to answer is whether the combinations of ribociclib and everolimus or ribociclib and temozolomide can prolong the life of patients diagnosed with HGG/DIPG or DHG H3G34-mutant.

Key Dates

Start date

Aug 22, 2024

Status verified

Oct 2025

Primary completion

Aug 31, 2029

Completion

Aug 28, 2034

Study Design

Enrollment

120 participants (estimated)

Allocation

NON_RANDOMIZED

Intervention model

PARALLEL

Primary purpose

TREATMENT

Arms

• Experimental: Stratum A (n=40)
  Patients with localized, intracranial, non-pontine, and non-thalamic HGG (who do not meet criteria for strata B, C, or D).
• Experimental: Stratum B (n=40)
  Patients with DIPG, defined as a tumor with pontine epicenter and diffuse involvement of at least 2/3 of the pons, with histopathology consistent with diffuse WHO grade 2-4 glioma (e.g., diffuse astrocytoma, anaplastic astrocytoma, glioblastoma, H3K27-altered diffuse midline glioma).
• Experimental: Stratum C (n=6-12)
  Patients with primary thalamic, spinal cord, and/or secondary (radiation-related) HGG.
• Experimental: Stratum D (n=6-12)
  Patients with metastatic/disseminated HGG, multifocal HGG, and/or gliomatosis cerebri who received craniospinal irradiation.
• Experimental: Stratum E (n=20)
  Patients with localized H3G34-mutant DHG

Primary Outcome Measure

Progression-Free Survival (PFS) in HGG (Part 2, Stratum A) [ Time Frame: From date on treatment until date of Progressive Disease or death due to any cause or date of last follow-up, assessed up to 60 months ]

Central Contacts

• Kelsey H Troyer, PhD
  16147223284
  [email protected]

Locations (11)

Find similar trials in Aurora, CO

Related Studies

• Molecular Analysis of Samples From Patients With Diffuse Intrinsic Pontine Glioma and Brainstem Glioma
  Recruiting · Children's National Research Institute · Washington D.C., District of Columbia
• Safety and Efficacy Study in Recurrent or Progressive Grade III or IV IDH1 Mutated Glioma
  PHASE1/PHASE2 · Recruiting · Neonc Technologies, Inc. · Los Angeles, California
• INdividualized Screening Trial of Innovative Glioblastoma Therapy (INSIGhT)
  PHASE2 · Recruiting · Patrick Wen, MD · Birmingham, Alabama
• International Diffuse Intrinsic Pontine Glioma (DIPG)/Diffuse Midline Glioma (DMG) Registry and Repository
  Recruiting · Children's Hospital Medical Center, Cincinnati · Cincinnati, Ohio