Diffuse Intrinsic Pontine Glioma (DIPG)

July 30, 2025

This page was reviewed under our medical and editorial policy by Leo David Wang, M.D., Ph.D., associate professor, Department of Pediatrics, City of Hope^® Cancer Center Duarte. 

About 200 to 300 children are diagnosed with a diffuse intrinsic pontine gliomas tumor (DIPG) each year in the United States, and most of them are between 5-10 years of age.

What Is DIPG?

Diffuse intrinsic pontine glioma (DIPG) is a rare, aggressive ​​type of childhood cancer that develops in the brain stem, the area at the base of the brain connecting the brain to the spinal cord. It forms in the area of the brainstem called the pons, responsible for vital functions like breathing, heart rate, hearing, seeing, talking, walking, eating and more.

As a glioma, DIPG grows from the brainstem's glial cells, which surround and support the brain's nerve cells.

Though DIPG may affect anyone at any age, this rare ​pediatric cancer mainly affects children between the ages of 5 and 10.

DIPG Symptoms 

Diffuse intrinsic pontine glioma symptoms depend on the tumor’s location and size, how far and fast the tumor has spread, and the child’s age and development stage.

Symptoms of DIPG may appear rapidly, with early signs and symptoms often seen in the eyes and eyelids, including:

• Difficulty with eye movement (the eye is turned inward)
• Drooping eyelids
• Vision problems, including double vision

DIPG tumors may grow rapidly, causing other symptoms without warning, such as:

• Problems hearing, talking, chewing and swallowing
• Drooping on one side of the face
• Headache (occurs in the morning or goes away after vomiting)
• Nausea and vomiting
• Weakness in the arms and legs
• Difficulty with balance and walking
• Behavior changes
• Trouble learning in school

Causes

It’s unclear why some people develop DIPG. Researchers continue to study the causes and risk factors of diffuse intrinsic pontine glioma.

Cancer develops when cells grow and divide uncontrollably due to changes (mutations) in their DNA. With DIPG, the exact cause of cell changes is often unknown, but researchers are working to better understand the role of specific genetic mutations and genetic disorders in these rare tumors. Most DIPG tumors bear a characteristic histone mutation known as H3K27M, although there are other important mutations as well.

DIPG Diagnosis

In addition to a comprehensive family medical history, physical exam and neurological exam to measure various aspects of brain function, a doctor may order other tests to help confirm a DIPG diagnosis, including those listed below.

Computed tomography (CT): This is an X-ray procedure that takes many pictures as the camera rotates around the body.

Magnetic resonance imaging (MRI): The primary imaging test used to diagnose DIPG, MRI uses a strong magnet, radio waves and a computer to produce a series of detailed images of locations inside the brain. In some cases, a contrast material called gadolinium is injected into a vein to enhance the scan.

Biopsy: This procedure is recommended to surgically remove suspicious tissue from tumor for further lab examination to diagnose DIPG and to identify genetic changes. Usually, a stereotactic biopsy, involving precise imaging navigation to the tumor, is performed.

DIPG Treatment

Diffuse intrinsic pontine glioma is challenging to treat because of its location in the brain, how quickly it advances and the way it spreads into healthy tissue. Doctors weigh various factors when determining a treatment plan, including the child’s overall health and whether the cancer is new or has returned.

Types of treatment for DIPG may include those listed below. ​​

Radiation therapy: This treatment, which uses high-energy X-rays or other forms of radiation to destroy cancer cells, is the standard of care for children who are newly diagnosed with DIPG. While not a cure, radiation therapy can temporarily slow or shrink the tumor.

​Chemotherapy: This approach uses drugs to destroy cancer cells and is sometimes used along with radiation therapy. Currently, there are not a lot of chemotherapy agents that have shown effectiveness for most kinds of DIPG, but research focused on more tumor-specific treatment, including different drug combinations, is evolving.

Surgery: This is rarely a treatment option for childhood DIPG due to the tumor’s proximity to the brain stem. If there’s fluid buildup around the brain or spinal cord, a surgeon may place a shunt (a long, thin tube) to drain extra fluid from the brain.

Much of DIPG patient care focuses on managing symptoms and supporting quality of life. But numerous clinical trials are underway studying new approaches, such as immunotherapies and cellular therapies like CAR T cell therapy, which have shown promise in killing DIPG cancer cells.

Who Treats DIPG?

With diffuse intrinsic pontine glioma, specialization matters. That’s why it’s critical to seek care from pediatric cancer experts and clinical providers who focus on treating children with brain tumors, starting with a pediatric oncologist.

The pediatric oncologist manages DIPG care and collaborates with other specialists, including:

• Pediatricians, who focus on preventing, diagnosing and treating childhood diseases and injuries
• Neurosurgeons, who perform surgery on the brain, spine and other parts of the nervous system
• Neuropathologists, who examine tissue samples to help diagnose diseases of the nervous system
• Radiation oncologists, who use high-energy beams of radiation to destroy cancerous tissue
• Neuro-oncologists, who specialize in diagnosing and treating brain tumors and other tumors of the nervous system
• Neurologists, who concentrate on diagnosing and treating brain, spinal cord and nervous system disorders
• Neuroradiologists, who utilize imaging tools and interventional techniques focused on the nervous system
• Endocrinologists, who diagnose and treat disorders of the endocrine system — hormone-producing glands and organs
• Supportive care providers, who offer an array of physical, psychological and emotional support to patients and their loved ones

DIPG Survival Rate

When discussing prognosis, or the likely outcome of diffuse intrinsic pontine glioma, doctors consider a variety of factors, including tumor location and spread, the child’s age at diagnosis and how long they’ve experienced symptoms prior to diagnosis.

• The five-year survival rate is about 2%.
• The median survival ranges from 9 to 15 months.
• Less than 10% of children survive beyond two years.

Survival rates may be complicated. That’s why it’s important to discuss them with a pediatric oncologist.