• Diffuse Intrinsic Pontine Glioma (DIPG) Overview

    Diffuse intrinsic pontine gliomas (DIPG) are highly aggressive and difficult to treat 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.

    DIPG Treatment at Dana-Farber/Boston Children's

    Children and adolescents with 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.

    Treatment & Care Options

    The treatment for diffuse intrinsic pontine glioma may include:

    • Radiation therapy, which is the primary therapy for newly diagnosed diffuse intrinsic pontine gliomas
    • Experimental chemotherapy, which combines chemotherapy and biologic therapy in combination with radiation therapy 

    Multiple clinical trials have demonstrated that routine chemotherapy does not increase survival rates for this condition. In addition, surgery is not an option because it can cause severe neurological damage and affect the body’s most vital functions. 

    Progressive or Recurrent Disease

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

    Long-term Outlook

    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.
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