Diffuse Intrinsic Pontine Glioma (DIPG)

  • A childhood brain tumor is always a very serious matter, and a diffuse intrinsic pontine glioma (a type of brainstem glioma) is no exception. Diffuse intrinsic pontine gliomas (DIPGs) are highly aggressive and difficult-to-treat brain tumors found at the base of the brain.

    Below is more detailed information about diffuse intrinsic pontine gliomas. If you would like to read more general information about brain tumors first, see the overview on brain tumors.

    How Dana-Farber/Boston Children’s approaches diffuse intrinsic pontine glioma

    If your child is cared for at Boston Children’s, she’ll be seen in the Glioma Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric oncology program through Dana-Farber Cancer Institute and Boston Children’s Hospital that provides—in one specialized program—all the services of both a leading cancer center and a specialized pediatric hospital.


  • What are diffuse intrinsic pontine gliomas?

    They are glial tumors, meaning that they arise in the brain's glial tissues—tissues made up of cells that help support and protect the brain's neurons. These tumors are found in an area of the brain stem (the lowest, stem-like part of the brain) called the pons, which controls many of the body’s most vital functions.

    Diffuse intrinsic pontine gliomas (DIPGs) account for 10 to 15 percent of all childhood central nervous system tumors. While they 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.

    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

    Doctors do not generally take biopsies of DIPGs, which makes it difficult to determine the stage of one of these tumors. When they 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.

    Boston Children's Hospital and Dana-Farber Cancer Institute are conducting many research studies that will help clinicians better understand and treat diffuse intrinsic pontine glioma.

    Causes

    What causes diffuse intrinsic pontine glioma?

    The cause of DIPG is not currently understood. There are no known factors or conditions that make your child more or less likely to develop this type of tumor.

    Symptoms

    What are the symptoms of a diffuse intrinsic pontine glioma?

    Symptoms usually develop very rapidly prior to diagnosis, reflecting the fast growth of these tumors. Most patients start experiencing symptoms less than three months—often less than three weeks—before diagnosis.

    While each child may experience symptoms of a DIPG differently, some of the most common 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

    Keep in mind that the symptoms of a brain tumor may resemble other, more common medical conditions. Always consult your child's physician for a diagnosis.

  • 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 scan 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).

    Because of their location and their tendency to invade normal tissue, taking biopsies of DIPGs is complicated, although a biopsy may be performed as part of a clinical trial in order to identify the best possible therapy and to confirm the diagnosis.   

    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.

  • Children with diffuse intrinsic pontine glioma (DIPG) are treated through the Glioma Program at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center, an integrated pediatric hematology and oncology partnership between Dana-Farber Cancer Institute and Boston Children’s Hospital. Working together, we provide more specialists, more programs, and more clinical trials than almost any other pediatric center treating cancer and blood disorders in the world.

    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.

    What are the treatments for a diffuse intrinsic pontine glioma?

    Treatment for DIPG may include:

    • radiation therapy – This is the primary 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.

    In addition, there are trials evaluating whether new ways of delivering the traditional drugs might improve responses. Multiple clinical trials have demonstrated that routine chemotherapy does not increase survival rates in this condition.

    Unfortunately, complete surgical removal is not an option in the treatment of these tumors, because they occur in the brainstem. Surgery in this part of the brain can cause severe neurological damage and affect the body’s most vital functions. When needed, it must be performed by an experienced pediatric neurosurgical team such as the one in the Dana-Farber/Boston Children’s team.

    How are side effects of pediatric cancer 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 now 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.

    What is the expected outcome after treatment for diffuse intrinsic pontine glioma?

    Unfortunately, the prognosis for DIPGs remains very poor, although a small percentage of patients survive this disease. Your child’s physician will discuss treatment options with you, including experimental clinical trials and supportive care.

    What about progressive or recurrent disease?

    Clinical trials and experimental therapies are available for patients with relapsed DIPG. Current trials include new medications as well as new methods for the delivery of more traditional agents.

    Resources and support

    There are also a number of resources and support services at Dana-Farber/Boston Children's Cancer and Blood Disorders Center to help you and your family through this difficult time.

    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 life-threatening illness. PACT also can provide psychosocial support and help arrange end-of-life care, if necessary.

  • Clinical and basic scientists at both Boston Children’s Hospital and Dana-Farber Cancer Institute are conducting numerous research studies to help clinicians better understand and treat diffuse intrinsic pontine gliomas (DIPGs).

    What is the latest research on diffuse intrinsic pontine gliomas and malignant gliomas?

    Dana-Farber/Boston Children's Cancer and Blood Disorders Center is a member of the Pediatric Oncology Therapeutic Experimental Investigators Consortium (POETIC), a collaborative clinical research group offering experimental therapies to patients with relapsed or refractory disease. It is also a member of the Department of Defense’s (DOD) Neurofibromatosis Clinical Trial Consortium.

    We are now leading an international phase II clinical trial on the genetics of diffuse intrinsic pontine glioma. Using advanced surgical techniques, a surgical biopsy of the tumor is performed on participating patients. Samples are then analyzed at the Broad Institute of Massachusetts Institute of Technology and Harvard, in order to understand the unique molecular characteristics of each tumor. This research will allow us to tailor treatment to each individual patient and hopefully improve outcomes for children with DIPG.

    Clinical trials

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

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