What Is Diffuse Intrinsic Pontine Glioma (DIPG)?

A Rare and Life-Limiting Brain Cancer Affecting Children

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Diffuse intrinsic pontine glioma (DIPG) is a rare, aggressive, and deadly type of brain cancer that primarily affects children under the age of 10. DIPG is diffuse in nature, which means the tumor isn’t well contained and takes over healthy brain tissue.

Young girl with head scarf and stuffed animal looking out a window

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The tumor grows in the brainstem, which sits at the back of the neck where the head meets the spinal cord. It’s made up of glial cells. These cells act as a kind of shield around other brain matter, particularly neurons, which transmit signals from the brain to all parts of the body.

With DIPG, glial cells grow out of control, eventually squeezing and damaging the brain. This brain damage causes serious and life-threatening consequences. 

How Rare Is DIPG?

Only about 200 to 300 new DIPG cases are diagnosed each year in the U.S. It most often strikes between the ages of 6 and 9 and is slightly more common in boys than girls. While rare, DIPG is also deadly. Many of those with DIPG survive less than one year. Ninety percent will die within two years of diagnosis.

DIPG Symptoms

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

  • Double or blurred vision. This is oftentimes one of the first symptoms. In some cases, the eyes may appear to be looking in different directions or look crossed (called strabismus).
  • 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.
  • Digestive issues such as nausea and vomiting. These can be due to hydrocephalus, which is a buildup of fluid that puts dangerous pressure on the brain.
  • Headaches, particularly in the morning. These tend to go away after vomiting.
  • Facial muscles that droop and sag, making the face look lopsided. Facial paralysis and droopy eyelids are also common.

Disease Progression and End of Life

DIPG symptoms can go from disabling to life-ending in a matter of weeks to months. Some of the most common symptoms in the last three months of life are:

  • Problems breathing
  • Problems walking
  • Difficulty swallowing
  • Trouble talking

DIPG and End of Life

Research shows that the top five symptoms children with DIPG experience in the last three months of life are problems moving (in one study, 100% of children experienced this), difficulty swallowing, difficulty talking, fatigue, and vision difficulties.

Seventeen percent of the children in one study were placed on ventilators so they could breath, and nearly half of the study participants received drugs for pain and agitation.

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.

Causes

Scientists know that DIPG occurs when something goes wrong with cell division and reproduction (called cell mutation), but no one knows why. Unlike other cancers, DIPG doesn’t seem to run in families and isn’t thought to be caused by environmental factors like exposure to chemicals or pollutants. 

Because DIPG is so rare, much isn’t known about it. But recent research shows that 80% of DIPG tumors have a mutation in the gene 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, hence, for these mutations to occur. That may help explain why DIPG affects young children so disproportionately.

Diagnosis

The first thing a doctor will do to diagnose DIPG is take a medical history and perform a physical exam. Your child’s healthcare provider will ask you about symptoms your child is experiencing and perform neurological testing, looking at balance and weakness in the arms and legs.

They may also look at reflexes using a Babinski reflex test, in which an area on the bottom of the foot is stroked. Babies respond to the stimulus by bringing their big toe upward and spreading their toes apart. But the reflex isn’t normal in kids over 2 and can signal brain or spinal cord damage.

DIPG is also diagnosed with imaging tests. A healthcare provider may order:

  • Computerized tomography scan (CT or CAT scan): This provides both horizontal and vertical images of the brain. It offers a more detailed image than X-ray.
  • Magnetic resonance imaging (MRI): This uses radio 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 around the tumor. It can also identify whether the tumor is made of glial cells or something different.

Biopsy

Rarely an MRI or other diagnostic test is inconclusive. But if it is, your child’s doctor may recommend a biopsy—particularly a stereotactic brainstem biopsy—to make a definitive diagnosis.

A stereotactic brainstem biopsy uses a CT scan or MRI along with computers and what’s known as fiducial markers, or small metal objects placed on the scalp, to more precisely pinpoint the tumor and take a sample.

Examining bits of the tissue can help scientists develop more individualized and targeted treatment. It can also “grade” the tumor, from I to IV. Grade I tumors contain tissue that’s close to normal. Grade III or IV means the tumor is aggressive.

Treatment

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 problematic.

Chemotherapy isn’t a good option either, primarily because the drugs used can’t cross the blood-brain barrier in enough quantities to have any effect on the tumor. 

To treat DIPG tumors, doctors mostly rely on radiation therapy, which uses specialized high-energy X-rays to damage cancer cells so they stop dividing or die off. This is generally used in children older than 3. The radiation is delivered five days a week for about six weeks.

It 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. When the tumor grows back, more radiation can be given to extend life and reduce symptoms.

Palliative and Hospice Care

As the end of life nears, many families look into palliative 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. But 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 surrounding tissue. The downside: Very few centers in this country use this type of radiation.
  • 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 works along the same lines: A tiny catheter delivers chemotherapy to an artery in the brain. The benefit of both is that cancer-fighting drugs, at higher concentrations, 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 8-10 months after diagnosis, although every child is different. About 10% of kids will survive two years, and 2% will live five years.

DIPG and Life Expectancy

When compared to others with DIPG, those who tend to have a longer life expectancy are those who:

  • Are younger than 3 years of age or older than 10
  • Experience symptoms longer before being diagnosed
  • Have the H3.1K27M (a Histone 3) mutation
  • Receive EGFR inhibitors (drugs that stop or slow cell growth) at diagnosis

Coping

A diagnosis of DIPG is devastating. 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. Read them a bedtime story using FaceTime or visit them at school every Monday and Friday 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. Better surgical techniques, targeted therapies, and more effective drug-delivery systems are in development and showing promise. And the longer-term 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.

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