• Diffuse Intrinsic Pontine Glioma (DIPG)


    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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    DIPG Treatment at Dana-Farber/Boston Children's

    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.

    How are diffuse intrinsic pontine gliomas classified?

    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:

    Low Grade

    High Grade

    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.

    What are the causes and symptoms of diffuse intrinsic pontine glioma?

    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:

    • Rapidly developing problems controlling eye movements, facial expressions, speech, chewing, and swallowing (due to problems in the cranial nerves)
    • Weakness in the arms and legs
    • Problems with walking and coordination

    How is diffuse intrinsic pontine glioma diagnosed?

    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:

    • Computerized tomography scan (also called a CT or CAT scan) – a diagnostic imaging procedure that uses a combination of X-rays and computer technology to produce cross-sectional images (often called slices), both horizontally and vertically, of the body. CT scans are more detailed than general X-rays.
    • Magnetic resonance imaging (MRI) – a diagnostic procedure that uses a combination of large magnets, radiofrequencies, and a computer to produce detailed images of organs and structures within the body. MRI provides greater anatomical detail than CT scans and does a better job of distinguishing between tumors, tumor-related swelling, and normal tissue.
    • Magnetic resonance spectroscopy (MRS) – a diagnostic test conducted along with an MRI. It can detect the presence of organic compounds around the tumor tissue that can identify the tissue as normal or tumor and may also be able to tell if the tumor is a glial tumor or if it is of neuronal origin (originating in a neuron, instead of an astrocytic or glial cell).
    • Biopsy – with recent advances in neuro-surgical techniques, it is now possible to safely biopsy diffuse intrinsic pontine glioma (when performed by a pediatric neuro-surgeon with specific expertise in performing biopsies for DIPG). Our team will discuss with you the option of a biopsy for your child. All children who receive a biopsy are also invited to participate in our ongoing research studies involving genetic tumor profiling. These studies may potentially guide personalized (precision) medicine.

    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.

    What are the treatments for a diffuse intrinsic pontine glioma?

    Your child’s physician will determine a specific course of treatment based on several factors, including:

    • Your child's age, overall health, and medical history
    • Type, location, and size of the tumor
    • Extent of your child’s disease
    • Your child's tolerance for specific medications, procedures, or therapies
    • How her doctors expects the disease to progress

    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:

    • Radiation therapy – This is the traditional therapy for newly diagnosed DIPGs. It uses high-energy rays (radiation) from a specialized machine to damage or kill cancer cells and shrink tumors. Conventional limited-field radiation produces responses in more than 90 percent of children with DIPGs. These responses are short-lived, however, lasting about six to nine months on average. Several trials to increase the dose of radiation therapy have been performed and none have improved survival.
    • Experimental chemotherapy – Chemotherapy and biologic therapy in combination with radiation therapy is actively being investigated as a treatment for this condition. Several trials evaluating new agents are either underway or have been recently completed. With a biopsy of the tumor at diagnosis, the selection of drugs targeted to your child’s tumor can be determined. Multiple clinical trials have demonstrated that routine chemotherapy does not increase survival rates for this condition.

    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.

    How are side effects of DIPG treatment managed?

    Side effects in the treatment of diffuse intrinsic pontine glioma can arise from biopsy, radiation, and chemotherapy.

    • Procedures should be performed in specialized centers where experienced neurosurgeons, working in the most technologically advanced settings, can provide the most precise molecular diagnosis while preserving normal brain tissue.
    • Radiation therapy often produces inflammation, which can temporarily worsen symptoms and dysfunction. To control this inflammation, steroids are sometimes necessary. New agents now are being tested that control the tumor- and radiation-associated swelling without causing the typical side effects of steroids.
    • Some of the chemotherapy agents are associated with fatigue, diarrhea, constipation, and headache. These side effects can be effectively managed under most circumstances.

    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.

    • Acupuncture/acupressure
    • Therapeutic touch
    • Massage
    • Herbs
    • Dietary recommendations

    Talk to your child’s physician about whether complementary or alternative medicine might be a viable option.

    What is the latest research on diffuse intrinsic pontine gliomas?

    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.

    Clinical trials

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

    What is the long-term outlook for diffuse intrinsic pontine glioma?

    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.

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