• 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 that they arise in the brain’s glial or supportive tissue. 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 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.

    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 Cancer and Blood Disorders Center through our Glioma Program. Continue reading to learn more about DIPG or visit the Glioma Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    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

    DIPG are most commonly diagnosed from diagnostic imaging studies, including:

    • Computerized tomography (CT or CAT) scan
    • Magnetic resonance imaging
    • Magnetic resonance spectroscopy, which can detect the presence of organic compounds around the tumor tissue that can identify the tissue as normal or tumor and may be able to distinguish glial tumors from those that originate in neurons

    The location of these tumors and their tendency to invade into normal tissue make biopsies complicated, although a biopsy may be performed as part of a clinical trial in order to identify the optimal therapy and confirm the diagnosis. After all tests are completed, doctors will be able to outline the best treatment options.

    DIPG Treatment and Care Options

    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 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. Multiple clinical trials have demonstrated that routine chemotherapy does not increase survival rates for this condition.

    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. 

    DIPG Research and Clinical Trials

    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?

    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 University, 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.

    Long-term Outlook for Children with DIPG

    Unfortunately, the prognosis for DIPG remains very poor, although a small percentage of patients can survive this disease. The median survival is nine to 12 months, even with treatment.

    However, clinical trials and experimental therapies are available for children DIPG. Current trials include novel medications as well as new methods for the delivery of more traditional agents.

    Resources and Support

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

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