Diffuse Intrinsic Pontine Glioma (DIPG)

Health Line

5 hours ago

This rare, aggressive type of cancerous tumor that forms in the brain stem often develops in childhood and is difficult to treat.
A diffuse intrinsic pontine glioma (DIPG) is an aggressive type of cancerous tumor that forms in the brain stem. That's the area at the base of your brain that connects the brain to the spine. The brain stem controls most of your basic functions, including vision, hearing, talking, walking, eating, breathing, heart rate, and more.
Gliomas are tumors that grow from glial cells, which are found throughout the nervous system. They surround and support nerve cells, called neurons.
DIPG most often develops in children between the ages of 5 and 10. However, it can affect anyone at any age.
The condition is rare — about 300 children are diagnosed with DIPG every year in the United States. It is also difficult to treat and has a negative outlook.
How are these tumors graded?
Most DIPG tumors are high grade, either grades 3 or 4. Very few are grade 2, and those that are act similarly to high grade tumors.
Because DIPG tumors are located in such a sensitive area, a traditional biopsy of the tumor isn't always recommended. However, a stereotactic biopsy is an imaging technique that can provide pathology findings and guide treatment decisions.
What are the symptoms?
DIPG symptoms can vary depending on factors such as:
the location of the tumor
the size of the tumor
whether or not the tumor has spread
how fast the tumor grows
the child's age and stage of development
Symptoms may develop suddenly and include:
eye movement issues
vision problems
trouble talking, chewing, or swallowing
drooping on one side of the face
nausea and vomiting
a headache that develops in the morning or goes away after vomiting
weakness in the limbs
loss of balance
trouble walking
behavior changes
issues with learning
However, these symptoms could be a sign of other issues outside of DIPG, so it's important to consult your child's doctor to determine the underlying cause.
What causes DIPG?
The development of DIPG is associated with changes in the way glial cells grow and divide. However, the exact cause of the condition is unknown, and there are no known risk factors.
Scientists have identified a few genetic mutations associated with DIPG, but more research is needed to better understand the origins of this condition.
How's it diagnosed?
If DIPG is suspected, your child's doctor will ask about your child's personal and family medical history. They will then do a physical and neurological exam.
Your child's doctor may also run additional testing to help confirm a diagnosis of DIPG, such as:
MRI scan: This type of imaging uses radio waves and a large magnetic field to create images of the inside of the body. An MRI can help distinguish between a tumor and typical tissue and swelling that might be related to the tumor.
Stereotactic biopsy: This form of imaging may be used to provide information about the cancerous tumor and guide treatment decisions.
Immunohistochemistry: This lab test uses antibodies to check for specific antigens in a tissue sample to help distinguish one type of cancer from another to make an accurate diagnosis.
Sometimes, you may seek a second opinion to confirm your child's diagnosis and treatment approach.
How's it treated?
Your child's DIPG treatment plan will take factors into consideration, such as their overall health and whether the cancer is newly diagnosed or recurring. Treatment options may include:
Steroids: These medications are often given at diagnosis in preparation for radiation therapy.
Radiation therapy: This is the main treatment for DIPG. It involves using high-energy X-rays to kill cancer cells and shrink tumors. However, radiation is a temporary treatment and is not viewed as a cure for DIPG.
Chemotherapy: This treatment uses powerful chemicals to destroy cancer cells and is sometimes used along with radiation therapy. However, this combination treatment is also not a permanent cure.
Surgery: Surgical removal of tumors that are so close to the brain stem often isn't recommended. However, in children who have increased fluid around the brain or spinal cord, surgery can be used to place a shunt to carry fluid away from the brain and reduce pressure on the brain or spinal cord.
Palliative care: Because there's no cure for DIPG, treatment often involves strategies to help improve quality of life for both you and your child.
Clinical trials: Your child's doctor may recommend enrolling in a clinical trial to help advance the future of DIPG treatment.
What's the outlook?
A diagnosis of DIPG can be life-altering news. The condition is currently considered fatal.
Even with treatment, nearly every case of DIPG starts to grow again within 6 to 12 months. At that point, no further treatment is known to help improve survival.
The average survival is usually 9 months, and only 10% of children diagnosed with DIPG live for more than 2 years.
Researchers are trying to learn as much as they can about DIPG in hopes of unlocking a lasting treatment. They are also studying how to prevent these cancerous tumors from developing in children with the genetic mutations associated with them.
Various clinical trials are currently underway. To learn more about ongoing or upcoming DIPG clinic trials, you can visit ClinicalTrials.gov.