Diffuse intrinsic pontine glioma

Diffuse intrinsic pontine glioma, often called DIPG, is a type of brain tumor that grows in the brainstem — specifically in a region called the pons. The pons is a critical part of the brain that controls many basic body functions, including breathing, heart rate, swallowing, eye movement, and balance. Because the tumor grows in a diffuse pattern, meaning it spreads throughout the tissue rather than forming a single lump, it cannot be safely removed with surgery. DIPG mainly affects children, typically between the ages of 5 and 10, though it can occur in younger children and teenagers as well. The tumor grows quickly and puts pressure on the brainstem, causing a range of serious symptoms. The most common early signs include problems with facial muscle control, difficulty walking, and double vision. As the tumor grows, children may develop weakness on one side of the body, trouble swallowing, and problems with speech. Treatment options for DIPG are very limited. Radiation therapy is the current standard of care and can temporarily shrink the tumor and relieve symptoms, but it does not cure the disease. Chemotherapy has not been shown to significantly improve outcomes in most cases. In recent years, researchers have discovered that most DIPG tumors carry a specific genetic change called the H3 K27M mutation, which has opened new doors for targeted therapies. Clinical trials are actively exploring new treatments, including ONC201, which has shown early promise for tumors with this mutation.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.Does my child's tumor have the H3 K27M mutation, and what does that mean for treatment options?,Are there any clinical trials that my child might be eligible for?,What are the goals of radiation therapy, and what side effects should we expect?,When should we involve palliative care, and what does that involve?,How will we know if the tumor is responding to treatment or growing again?,What support services — such as physical therapy, speech therapy, or psychological support — are available for our family?,Should we seek a second opinion at a specialized DIPG center?

Common questions about Diffuse intrinsic pontine glioma