Diffuse Intrinsic Pontine Glioma

What is a diffuse intrinsic pontine glioma?

Diffuse intrinsic pontine gliomas (DIPGs) 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 called the pons, which controls many of the body's most vital functions such as breathing, blood pressure and heart rate.

Approximately 300 children are diagnosed with DIPGs each year, usually between the ages of 5 and 9. Although the prognosis for DIPGs remains very poor but new research, with the help of clinical trials, may provide the key to improved treatment options.

Unlocking a treatment for DIPG

Katherine Warren, MD, clinical director for Pediatric Neuro-Oncology at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, has been researching cancer of the central nervous system (CNS)  for more than two decades.

 Katherine Warren, MD, clinical director for Pediatric Neuro-Oncology at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center.

What are the symptoms of diffuse intrinsic pontine gliomas?

The symptoms of diffuse intrinsic pontine glioma (DIPG) usually develop very rapidly prior to diagnosis, reflecting the fast growth of these tumors. Most patients start experiencing symptoms less than three months — and often less than three weeks — before diagnosis. The most common symptoms include:

  • rapidly developing problems controlling eye movements, facial expressions, speech, chewing, and swallowing
  • weakness in the arms and legs
  • problems with walking and coordination

What causes DIPG?

For a long time, little was understood about DIPGs because clinicians feared they could not be safely biopsied. However, recent and ongoing research at Dana-Farber/Boston Children's Cancer and Blood Disorders Center has led to important discoveries, allowing us to learn more about certain genetic mutations that may cause DIPG.

How we care for DIPGs

Children and adolescents with DIPGs are treated at Dana-Farber/Boston Children's Cancer and Blood Disorders Center through our Glioma Program, one of the world’s largest pediatric glioma treatment programs. Our brain tumor specialists have extensive expertise in treating many types of gliomas, including DIPGs.

Our areas of research for DIPG

Clinical and basic scientists at both Dana-Farber Cancer Institute and Boston Children’s Hospital are conducting numerous research studies to help clinicians better understand and treat DIPGs, leading a national phase II clinical trial on the genetics of diffuse intrinsic pontine glioma. Using advanced surgical techniques, a surgical biopsy of the tumor was performed on participating patients. Samples were then analyzed at the Broad Institute, Dana-Farber Cancer Institute, and Harvard Medical School as well as with collaborators at McGill University in Montreal, Canada, in order to understand the unique molecular characteristics of each tumor. Dana-Farber/Boston Children’s researchers are using initial findings from that study to develop a follow-up DIPG clinical trial. This new trial will tailor treatments to the specific genetic mutations within each individual patient’s tumor, which hopefully will improve outcomes for children with DIPG.

For many children with rare or hard-to-treat conditions, clinical trials may provide new options.