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

This study is for children, teenagers, and young adults with certain brain tumours. To be considered, you generally need to be between 12 months (1 year) and 39 years old. However, for the very first part of the study testing ribociclib and everolimus, you need to be under 21 years old.

Your tumour must be a newly diagnosed High-Grade Glioma (HGG), which includes DIPG, or a specific type called Diffuse Hemispheric Glioma (DHG, H3G34-mutant). Doctors will need to confirm the exact type of tumour from a biopsy or surgery sample. In the case of DIPG, it must be located in a specific area of the brain called the pons and show certain features on scans.

If you join this study, you will be taking specific medications, either a combination of ribociclib and everolimus, or ribociclib and temozolomide. These drugs are usually given after you have completed initial radiotherapy. The study will happen in different stages. First, a small group of patients will help doctors find the safest and most effective dose of the study drugs. This involves regular check-ups, blood tests, and scans to see how your body reacts to the medication and how the tumour changes.

Once the right dose is found, more patients will join the study to see how well the treatment works over a longer period. This will also involve regular hospital visits for assessments, scans (like MRI), and blood tests to monitor your health, look for any side effects, and track the tumour's response. The duration of your participation will depend on how your tumour responds to the treatment and your general health, but it will involve ongoing follow-up for a significant period.