RecruitingMalignant Glioma

A Study of the Drug Selinexor With Radiation Therapy in Patients With Newly-Diagnosed Diffuse Intrinsic Pontine (DIPG) Glioma and High-Grade Glioma (HGG)

Eligible age

1–21 yrs

Accepts

All genders

Locations

42 states

Healthy volunteers

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About this study

This phase I/II trial tests the safety, side effects, and best dose of selinexor given in combination with standard radiation therapy in treating children and young adults with newly diagnosed diffuse intrinsic pontine glioma (DIPG) or high-grade glioma (HGG) with a genetic change called H3 K27M mutation. It also tests whether combination of selinexor and standard radiation therapy works to shrink tumors in this patient population. Glioma is a type of cancer that occurs in the brain or spine. Glioma is considered high risk (or high-grade) when it is growing and spreading quickly. The term, risk, refers to the chance of the cancer coming back after treatment. DIPG is a subtype of HGG that grows in the pons (a part of the brainstem that controls functions like breathing, swallowing, speaking, and eye movements). This trial has two parts. The only difference in treatment between the two parts is that some subjects treated in Part 1 may receive a different dose of selinexor than the subjects treated in Part 2. In Part 1 (also called the Dose-Finding Phase), investigators want to determine the dose of selinexor that can be given without causing side effects that are too severe. This dose is called the maximum tolerated dose (MTD). In Part 2 (also called the Efficacy Phase), investigators want to find out how effective the MTD of selinexor is against HGG or DIPG. Selinexor blocks a protein called CRM1, which may help keep cancer cells from growing and may kill them. It is a type of small molecule inhibitor called selective inhibitors of nuclear export (SINE). Radiation therapy uses high energy to kill tumor cells and shrink tumors. The combination of selinexor and radiation therapy may be effective in treating patients with newly-diagnosed DIPG and H3 K27M-Mutant HGG.

Sponsor: National Cancer Institute (NCI)

You may qualify if…

✓ PRE ENROLLMENT: Patients must be =\< 25 years of age at the time of enrollment on APEC14B1 part A central nervous system (CNS)/high grade glioma (HGG) pre-enrollment eligibility screening
✓ Please note:
✓ This required age range applies to pre-enrollment eligibility for all HGG patients. Individual treatment protocols may have different age criteria.
✓ Non-DIPG patients with tumors that do not harbor an H3K27M-mutation and are \>= 18 years of age will not be eligible to enroll on ACNS1821 (Step 1).
✓ PRE ENROLLMENT: Patient is suspected of having localized, newly diagnosed HGG, excluding metastatic disease, OR patient has an institutional diagnosis of DIPG
✓ Please note: there are specific radiographic criteria for DIPG patient enrollment on ACNS1821 (Step 1)
✓ As of February 14, 2025, stratum DIPG and stratum DMG have closed to accrual, and no patients will be enrolled on these strata after Amendment #4.
✓ PRE ENROLLMENT:

You may not qualify if…

✕ STEP 1: Patients must not have received any prior therapy for their central nervous system (CNS) malignancy except for surgery and steroid medications.
✕ STEP 1: Patients who are currently receiving another investigational drug are not eligible.
✕ STEP 1: Patients who are currently receiving other anti-cancer agents are not eligible.
✕ STEP 1: Patients \>=18 years of age who have H3 K27M-wild type HGG.
✕ STEP 1: Patients who have an uncontrolled infection.
✕ STEP 1: Patients who have received a prior solid organ transplantation.
✕ STEP 1: Patients with grade \> 1 extrapyramidal movement disorder.
✕ STEP 1: Patients with known macular degeneration, uncontrolled glaucoma, or cataracts.