Perlmutter Cancer Center

This is a study testing a new medicine called Selinexor (small-molecule drug) in children and young adults who were recently diagnosed with a brain tumor called diffuse intrinsic pontine glioma (DIPG) or high-grade glioma (HGG). Selinexor works as a selective inhibitor of nuclear export (SINE) by blocking a protein called XPO1, which may help keep cancer cells from growing and may kill them. Selinexor will be given along with regular radiation therapy to help shrink the tumors.

This study has two parts: dose-finding phase and efficacy phase. In Part 1(dose finding phase), the doctors will try to find the dose of Selinexor that works best but does not cause too many bad side effects. In Part 2 (efficacy phase),doctors will use the best dose found in Part 1 to see how well it is shrinking the tumor. All patients will be given radiation therapy in combination with the Selinexor for a total of eight weeks. After a two week rest period, Selinexor maintenance therapy will be given alone. All patients will have medical check ups and blood samples taken to see how their bodies are handling the study medicine. The study team will have special scans done for all patients to see how the cancer is responding to the study medicines. The study team will be monitoring the patients for potential side effects and safety concerns.