What Is Diffuse Intrinsic Pontine Glioma (DIPG)?

Diffuse intrinsic pontine glioma (DIPG) is a rare, aggressive, and deadly type of brain cancer that primarily affects children under the age of 10.

The tumor grows in the brainstem, which sits at the back of the neck where the brain meets the spinal cord. It’s made up of glial cells, which makes it a type of glioma. Glial cells act as supportive cells around neurons.

With DIPG, glial cells grow out of control, eventually squeezing and damaging the healthy brain tissue. This resulting brain damage leads to serious and life-threatening consequences. 

DIPG Symptoms

DIPG is a fast-growing tumor, with symptoms typically appearing suddenly and quickly becoming dramatically worse. These tumors occur in the pons, an area of the brainstem that is responsible for many of the body’s essential functions, such as breathing, blood pressure control, and eye movement.

Common DIPG symptoms include:

• Double vision or blurred vision is often one of the first symptoms.
• In some cases, the eyes may appear to be looking in different directions or look crossed.
• Problems with walking, maintaining balance, and coordination. Parents may notice jerky movements in their children or weakness on one side of the body.
• Difficulty chewing and swallowing. Some children may drool.
• Nausea and vomiting. These can be due to hydrocephalus, which is a dangerous buildup of fluid that puts pressure on the brain.
• Headaches, particularly in the morning. These may go away after sudden vomiting.
• Facial or eyelid muscles that droop and sag, making the face look lopsided.

Disease Progression and End of Life

The effects of DIPG can become life-threatening in a matter of weeks to months.

Some of the most common symptoms in the last three months of life are:

• Fatigue
• Impaired coordination and walking
• Difficulty swallowing or speaking
• Vision difficulties
• Difficulty breathing
• Confusion or change in consciousness

These problems become progressively worse as time goes on. In the last days of life, children with DIPG have increasing problems with breathing and consciousness.

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Causes

DIPG doesn’t run in families and isn’t thought to be caused by environmental factors—like exposure to chemicals or pollutants. 

Recent research shows that 80% of DIPG tumors have a mutation in the gene that codes for a protein called histone H3. Histones are proteins that allow DNA to wrap around a chromosome and fit into the nucleus of a cell.

Other research links about 25% of DIPG cases to mutations in a gene called ACVR1.

Early childhood is a prime time for brain development and, that may help explain why DIPG affects young children so disproportionately.

Diagnosis

If your child has symptoms of DIPG, their doctor will ask you about the symptoms that your child is experiencing and perform neurological testing, looking at balance and movement in the arms and legs.

DIPG is usually diagnosed with imaging tests.

Your child's doctor may order:

• Computerized Tomography (CT or CAT scan): This provides both horizontal and vertical images of the brain with more detail than X-ray.
• Magnetic resonance imaging (MRI): This uses magnetic frequencies to look at the brain and can distinguish between tumors, swelling, and normal tissue. 
• Magnetic resonance spectroscopy (MRS): This measures the chemical makeup of the tissue in and around the tumor.

Biopsy

If your child's imaging test is inconclusive, their doctor may recommend a biopsy to make a definitive diagnosis.

A stereotactic brainstem biopsy is done with the use of imaging, along with with aid of small metal markers placed on the scalp to precisely pinpoint the tumor and take a sample.

The tissue sample will be examined so your child's doctors can define and grade the tumor. Grading is based on tumor potential for invasiveness and ranges from grade I to IV. Grade I tumors contain tissue that’s close to normal. Grade III or IV tumors are substantially different from normal tissue. These designations help direct the treatment plan.

Treatment

DIPG tumors are mainly treated with radiation therapy, which uses specialized high-energy X-rays to prevent cancer cells from dividing. This is generally used in children older than 3. The radiation is delivered five days a week for about six weeks.

Radiation therapy improves symptoms in about 80% of cases and can prolong life by two to three months. Children who are too young or too sick to lie still while it’s being administered must undergo anesthesia to receive the radiation.

If the tumor grows back, more radiation can be given to reduce symptoms and extend a child's life.

It’s important to note that there is no cure for DIPG, and therapies used to treat other types of tumors are often not effective.

Given the fact that DIPG doesn’t produce a solid mass, but instead creeps into the nerve fibers of the pons, standard neurosurgery isn’t a viable option. The delicate area of the brain in which these tumors are found also makes surgery especially dangerous.

Many chemotherapy drugs can’t cross the blood-brain barrier efficiently enough to have a therapeutic effect on the tumor. 

Palliative and Hospice Care

As the end of life nears, many families look into palliative care or hospice care to help them grapple with tough issues and give their children the most comfortable end-of-life experience.

A palliative or hospice care team can help you decide what measures to take if your child:

• Stops eating and drinking
• Becomes disoriented
• Has breathing problems
• Becomes inordinately sleepy and unresponsive

All these end-of-life symptoms are extremely difficult for any parent to witness. A hospice care team can guide you on what to expect, tell you if your child is feeling pain or distress, and advise you on what comfort measures to take—for all involved.

Advanced Drug Research

For over 40 years, there’s been little advancement in DIPG treatments. Some progress is now being made. More testing and trials are needed, but some promising advances include:

• Newer radiation therapies: Proton beam radiation (PRT) uses high-energy beams that more precisely target tumors while sparing healthy surrounding tissue.
• Better drug-delivery methods: Convection enhanced delivery (CED) uses a catheter (a flexible, hollow tube) to deliver drugs directly to the tumor. Intra-arterial chemotherapy uses a tiny catheter to deliver chemotherapy to an artery in the brain. The benefit of these methods is that high concentrations of cancer-fighting drugs can go directly where they’re needed.
• Immunotherapy: Immunotherapy helps the body’s own immune system recognize and fight cancer cells. One technique under investigation is chimeric antigen receptor (CAR) T-cell immunotherapy. It takes infection-fighting T-cells from the blood of a child with DIPG, mixes it with a virus that helps the T-cells identify and attach to cancer cells, then puts the cells back into the patient where the T-cells can locate and destroy the offending cancer cells.

Prognosis

Typically, children with DIPG survive an average of eight to 10 months after diagnosis, although every child is different. About 10% of kids will survive two years, and 2% will live five years.

Coping

A diagnosis of DIPG can be devastating for a child and their familiy. Managing the pain and grief is essential for you, your child, and your family.

Here, some suggestions:

• Answer your child’s questions about what’s happening honestly. Give them as much straightforward information as you think they can understand and seem to want to hear. 
• Give your child choices. DIPG takes away so much from them, including freedom—to play, go to school, be a child. Give your child back some control by allowing them to make simple choices, such as what to eat, what to wear, what book to read. If your child is old enough, also involve them in treatment decisions.
• Expect—and talk about—behavior changes. Just like adults, children deal with grief in a variety of ways. Your child may become agitated, withdrawn, or anxious. You may notice changes in eating and sleeping patterns. Ask your child how they’re feeling and what would help them feel better.
• Cry when you need to—even if it’s in front of your child or other children you have. Letting them see you cry can make it easier for them to express their true feelings. But don’t rage or wail. That can be frightening to children.
• Set up routines with your other children at home. Your sick child is understandably demanding the majority of your attention. While you can’t spend as much time as normal with your other children, try to set up meaningful routines with them. If you are spending many evenings at the hospital, for example, read a bedtime story with your other children using FaceTime or visit them at school on a set day every week for a lunch date.
• Encourage your other children to talk about their feelings. Reassure them that nothing they did caused DIPG. Use role-play to help them formulate answers to difficult questions others might ask. For example, you can ask “If Alex or Tony ask you at soccer practice how Will is doing, what would you say?” Help your child find the words that express their feelings without making them overly emotional. One response could be: “He’s in the hospital and pretty sick. I don’t know when he’s coming home. But I’d rather just concentrate on soccer right now.”
• Do things now that can help keep your child’s memory alive later. Some ideas include planting a tree (ask your child which kind they’d like the best), taking a family vacation, and having your child write letters (or dictate them to you) to send to family and friends. Write about things the child loves or favorite memories they have of the person you’re writing to. 

A Word From Verywell

DIPG is a deadly and devastating disease. But there’s reason for hope. Targeted therapies and more effective drug-delivery systems are in development and showing promise. And the survival rate is inching up, from a 1% five-year survival rate to a 2% one today. That’s a small but significant jump to any family with a child with DIPG.