What is Diffuse Intrinsic Pontine Glioma?
Diffuse Intrinsic Pontine Glioma is a type of brain tumor that comes from glial cells. This is also known as Diffuse Midline Glioma (DMG). Normally glial cells surround, protect, and support other cells in the brain. But in a tumor, they continue to grow without stopping and eventually compress and hurt other parts of the brain.
DIPG was formerly classified as a tumor in the pons. The World Health Organization now recognizes this tumor in other locations such as the pons, thalamus, spinal cord and cerebellum. These parts of the brain control vital functions such as swallowing, balance, eye movements, sensation, and the passing of information from the brain to the rest of body, controlling movements in the arms and legs.
DIPG occurs most often in elementary school-age children, but it can affect children of any age. About 100 to 300 children in the U.S. are diagnosed with DIPG each year.
DIPG tends to grow quickly, so most children are diagnosed soon after their symptoms start. The most common symptoms are:
DIPG is often diagnosed by a child’s symptoms along with a picture of their brain using magnetic resonance imaging (MRI). The location, appearance and symptoms of the tumor usually identify it as DIPG. A biopsy, or surgery to remove a piece of the tumor, may be performed to provide confirmation of diagnosis.
Treatment options for DIPG are limited. The tumors cannot be entirely removed because they grow alongside and in between normal cells that control essential functions. Radiation can help shrink the tumor, but it cannot make the tumor go away permanently. There are some medical options that may also slow tumor growth and the possibility of enrollment on a clinical trial.
Research continues on finding new medications to help stop or slow the growth of DIPG. So far, because it is hard to get the medicine across the blood-brain barrier, none of them has worked very well.
Our Treatment Team
Cincinnati Children’s has long been one of the nation’s leading pediatric centers for brain tumor treatment and research. Research into finding the most effective treatments for high-grade gliomas and DIPG is a high priority. Our physician-scientists do their utmost to ensure that valid scientific discoveries move quickly and safely from the laboratory to the bedside to benefit our patients.
Because the diagnosis of DIPG affects patients and families in so many ways, our treatment team includes a range of specialists who support families during and after treatment, including:
One of the significant barriers to learning more about DIPG has been a lack of tumor tissue for study. Recently, Cincinnati Children’s helped lead a group of hospitals and research organizations known as the DIPG Collaborative in creating an international DIPG registry. This collaborative effort among physicians and researchers from North America, Canada, Europe and Australia collects health information and tumor samples donated by families of DIPG patients around the world.
Because of this, researchers are learning more about the biology and genetic makeup of the disease. This can help them design treatments that are more effective. Scientists have had some success developing new medicines in laboratory models of DIPG, and are better ways to deliver those medicines to the tumor. Researchers at Cincinnati Children’s are continuing to study drugs that could target a pathway activated by DIPG.