Phase 12 Study of Selinexor with Radiation Therapy for Children and Young Adults Newly Diagnosed with Diffuse Intrinsic Pontine Glioma DIPG or High-Grade Glioma HGG

What this Trial Is About

Researchers are studying children and young adults with newly diagnosed diffuse intrinsic pontine glioma DIPG or high-grade glioma HGG that have a specific genetic change called H3 K27M mutation. This phase III trial evaluates the safety, side effects, and best dose of the drug selinexor combined with standard radiation therapy, and aims to determine how well this combination shrinks tumors in these patients. DIPG is a high-risk brain tumor located in a critical area of the brainstem, and HGG refers to rapidly growing glioma tumors in the brain or spine. Participants receive standard radiation therapy five days a week for 5 to 7 weeks. Beginning on day 4 or 5 of radiation, selinexor is given orally on specific days during this period. After a two-week rest, patients enter a maintenance phase where selinexor is taken orally on days 1, 8, 15, and 22 of each 28-day cycle, for up to 24 cycles if there is no disease progression or unacceptable side effects. Magnetic resonance imaging MRI scans and possibly a biopsy are done before treatment and during follow-up. During the study, participants are monitored closely with MRIs and clinical evaluations to assess side effects and tumor response. Researchers measure the maximum tolerated dose of selinexor, event-free survival, overall survival, and response rates over up to five years. After treatment, patients are followed every three months during the first year, then every six months for two years, and annually for years four and five to track long-term outcomes and safety.

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