Diffuse Intrinsic Pontine Glioma (DIPG)

Fact: An estimated 300-350 children are diagnosed with DIPG each year

What is Diffuse Intrinsic Pontine Glioma (DIPG)?
"DIPG are highly aggressive and difficult to treat brain tumors found at the base of the brain. They are glial tumors, meaning they arise from the brain's glial tissue—tissue made up of cells that help support and protect the brain's neurons. These tumors are found in an area of the brainstem (the lowest, stem-like part of the brain) called the pons, which controls many of the body's most vital functions such as breathing, blood pressure, and heart rate." --  Boston Children's Hospital

There are about 300-350 new cases of DIPG diagnosed each year in the United States, usually in children under the age of 10. DIPG affect boys and girls equally.  DIPG primarily affects children between the ages of 5 and 7, and makes up 10-15 percent of all brain tumors in children, according to the website DIPGregistry.org. There are approximately 100-150 new cases of DIPG diagnosed each year in the United States, and 300-350 per year in all of North American and Europe. 
What causes DIPG?
Scientists do not yet know what causes DIPG, and the cancer is so rare that it hasn’t received nearly enough research funding to find a cause, a cure, or even better treatment options.  Survival rates have not improved much over the last few decades due to this lack of new research.

Who Gets DIPG?

While Diffuse Intrinsic Pontine Glioma is considered to be a childhood disease, it does occur in adults. DIPG in adults is extremely rare and even more poorly understood than in children. 
Grades and Diagnosis

DIPG falls into the Glioma staging system, so they can be classified according to the four stages below based on how the cells look under the microscope. The grades are from the least severe to the most severe.
  • Low Grade: Grade I or II means that the tumor cells are the closest to normal.
  • High Grade: Grade III or IV means that these are the most aggressive tumors.
The main issue with DIPG is that most of these tumors are not classified by grade because surgery to obtain tissue by biopsy or to remove the tumor is not safe because of the location of the tumor. When these tumors are biopsied, they are usually grade III or grade IV tumors, which tend to behave very aggressively. Most of the tumors are diagnosed by their appearance on MRI.
Symptoms of DIPG
Because the brainstem controls blood pressure, breathing, and a number of other critical life functions, DIPG can cause significant neurological problems. Pontine gliomas often affect the cranial nerves, so many of DIPG's early symptoms appear in the facial muscles, often affecting the eye and eyelid movements.The tumor grows so fast that symptoms often appear  suddenly and get worse quickly.  The most common symptoms of DIPG are:
  • Problems with eye movement. The most common one is difficulty looking to the side. Double vision is common because of the loss of alignment of the eyes. Other problems of eye movement include drooping eyelids and inability to close the eyes completely. Often the problems are seen on both eyes.
  • Facial weakness, drooping on one side of the face
  • Sudden appearance of hearing problems, including deafness.
  • Trouble chewing or swallowing, gagging while eating
  • Limb weakness, difficulty standing or walking, abnormal gaits, unbalanced limb movements
  • Headache
  • Nausea and vomiting from brain edema (swelling) or hydrocephalus. Hydrocephalus is a condition in which the flow of cerebrospinal fluid around the brain is blocked, causing an increase in pressure inside the skull. About 10 percent of DIPG patients have hydrocephalus at the time of diagnosis.
  • Most children with headaches do not have brain tumors, of course, but anyone with neurological symptoms like those listed above should be evaluated by a physician. Suspected or confirmed brain tumors should be referred to a neurosurgeon for evaluation.

Want to learn more?

The following organizations and/or websites offer additional information and education about DIPG Disease. They are listed in Alphabetical order without any preference or prejudice. Listing these organizations is not a recommendation or referral in any regard for seeking treatment or consultation or support for treatment.

About Kids Health
Aidans Avengers
American Brain Tumor Association
Boston Children's Hospital
Dana Farber Boston Children's
DIPG Registry
Pediatric Brain Tumor Foundation
St. Jude Children's Hospital
The Childhood Brain Tumor Foundation (CBTF)
The Cure Starts Now Foundation
The Lyla Nsouli Foundation for Children's Brain Cancer Research
Weill Cornell Brain & Spine Center 

**Information on this page taken from:
Boston Children's Hospital
St. Jude Children's Hospital

Weill Cornell Brain & Spine Center