• Ganglioglioma Overview

    Gangliogliomas are rare, low-grade tumors that affect both glial cells (responsible for providing the structural support of the central nervous system) and neuronal cells (the functioning component of the central nervous system).  These tumors occur most commonly in the cerebrum, the part of the brain that controls motor, sensory and higher mental function. They rarely spread to other parts of the brain or transform into higher-grade tumors. Children with certain genetic syndromes, including neurofibromatosis 1 and tuberous sclerosis, are at high risk of developing glial tumors, including gangliogliomas; however, most of these tumors develop spontaneously.

    Ganglioglioma Treatment at Dana-Farber/Boston Children's

    Children with gangliogliomas are treated at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center through our Glioma Program. Continue reading to learn more about gangliogliomas or visit the Glioma Program homepage to learn about our expertise and treatment options for this condition.

    Symptoms & Diagnosis

    Since gangliogliomas grow relatively slowly, a child may have been having symptoms for many months prior to diagnosis, or symptoms may appear more suddenly. Many children with gangliogliomas first present with seizures. Other symptoms are associated with increased pressure in the brain, including:

    • Headache – generally upon awakening in the morning
    • Nausea and vomiting – often worse in the morning and improving throughout the day
    • Fatigue
    • Weakness on one side of the body

    A physician may order a number of different tests to best diagnose the tumor. In addition to a physical exam, medical history and neurological exam (which tests reflexes, muscle strength, eye and mouth movement, coordination and alertness), those tests may include:

    • Computerized tomography (CT or CAT) scan
    • Magnetic resonance imaging (MRI)
    • Biopsy 
    • Electroencephalogram

    After all tests are completed, doctors will be able to outline the best treatment options.

    Treatment & Care Options

    A child’s physician will determine a specific course of treatment based on several factors. Some therapies will treat the tumor while others are intended to address complications of the disease or side effects of the treatment. These treatments include:

    • Neurosurgery – to remove as much of the tumor as safely possible; surgery is often limited due to the deep, central placement of these tumors within the brain
    • Chemotherapy – either before surgery to shrink the tumor or to kill cancer cells
    • Radiation therapy – precisely targeted treatment to control local growth of tumors; not recommended unless the child’s tumor has re-grown due to potential long-term side effects of therapy

    Progressive or Recurrent Disease

    The recommended treatment for progressive or recurrent ganglioglioma is radiation therapy. Clinical trials and experimental therapies are also available for children with recurrent tumors that are not responsive to standard therapies.

    Long-term Outlook

    Gangliogliomas are associated with a high rate of survival. The survival rate is not as high if the tumor can’t be removed completely during surgery or if the tumor returns.