Diffuse intrinsic pontine gliomas (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.
Diffuse intrinsic pontine gliomas account for 10 percent of all childhood central nervous system tumors. Approximately 300 children in the U.S. are diagnosed with DIPG each year. While DIPGs are usually diagnosed when children are between the ages of 5 and 9, they can occur at any age in childhood. These tumors occur in boys and girls equally and do not generally appear in adults.
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Children and adolescents with diffuse intrinsic pontine gliomas (DIPG) are treated at Dana-Farber/Boston Children's through our Glioma Program, one of the world’s largest pediatric glioma treatment programs. Our brain tumor specialists have extensive expertise in treating all types of gliomas, including DIPGs. Continue reading to learn more about DIPG, read our overview on brain tumors, or visit the Glioma Program to learn about our expertise and treatment options.
There are four stages or “grades” of gliomas, according to how the cells look under a microscope. Ordered from least severe to most severe, they are:
When DIPGs are biopsied, they are usually grade III or grade IV. Occasionally, they are grade II, but because of their location in the brain they are still considered malignant. That being said, diffuse intrinsic pontine gliomas usually progress like grade IV glioblastoma multiforme tumors. They are very aggressive tumors and grow by invading normal brain tissue.
For a long time, little was understood about diffuse intrinsic pontine gliomas because clinicians feared that DIPGs could not be safely biopsied. However, recent and ongoing research at Dana-Farber/Boston Children's has led to important discoveries, and we are learning more about certain genetic mutations that might be causing DIPG.
The symptoms of 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:
The first step in treating your child is forming an accurate and complete diagnosis. Diffuse intrinsic pontine glioma is most commonly diagnosed from imaging studies.
To start, your child’s doctor will perform a complete medical and physical examination. In addition, your child’s physician may order some of the following imaging tests:
After we complete all necessary
tests, our experts meet to review and discuss what they have learned about your
child's condition. Then, we will meet with you and your family to discuss the
results and outline the best pediatric cancer treatment options for your child.
Your child’s physician will determine a specific course of
treatment based on several factors, including:
There are a number of treatments we may recommend. Some of them
help to treat the tumor while others are intended to address complications of
the disease or side effects of the treatment.
Treatment for DIPG may include:
Unfortunately, complete surgical removal is not an option in the
treatment of these tumors, because of their location in the brainstem. Surgery in this
part of the brain can cause severe neurological damage and affect the body’s
most vital functions; only biopsies can be performed safely.
Side effects in the treatment of diffuse intrinsic pontine glioma can arise from the biopsy, radiation, and chemotherapy.
Many specialized brain tumor treatment centers, including Dana-Farber/Boston Children's, have specialists who deliver complementary or alternative medicines. These treatments, which may help control pain and the side effects of therapy, include the following.
Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.
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 diffuse intrinsic pontine gliomas (DIPGs).
We have been
leading a national phase II clinical trial on the genetics of diffuse intrinsic
pontine glioma. (Accrual for the trial closed in November 2015 and data is now
being reviewed.) 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. (Learn
more about this DIPG research.) Dana-Farber/Boston Children’s
researchers are using initial findings from that study to develop a follow-up
DIPG clinical trial. The 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 provide new options.
Unfortunately, the prognosis for DIPGs remains very poor, although a small percentage of patients survive this disease and new DIPG research may provide the key to improved treatment options. Your child’s physician will discuss treatment options with you, including experimental clinical trials and supportive care.
Dana-Farber/Boston Children's Cancer and Blood Disorders Center offers a number of resources and support services to help you and your family throughout treatment for diffuse intrinsic pontine glioma (DIPG).
When appropriate, our Pediatric Advanced Care Team (PACT) offers supportive treatments intended to optimize the quality of life and promote healing and comfort for children with a life-threatening illness. PACT also can provide psychosocial support and help arrange end-of-life care, if necessary.
On May 22, 2017, Mark Kieran, MD, PhD, discussed what's new in research and treatment for pediatric brain tumors on Facebook Live. Watch the archive above.
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For more than 15 years, pediatric neuro-oncologist Mariella Filbin, MD, PhD, has been on a scientific crusade to understand DIPGs.