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  • Ganglioglioma

    Ganglioglioma is low-grade tumor of mixed cell type. It is a type of brain tumor that contains properties of 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). It is very rare.

    • Ganglioglioma occurs most commonly in the cerebrum (the part of the brain that controls motor, sensory and higher mental function), but may occur in any part of the brain and spinal cord.
    • In rare cases, ganglioglioma may undergo transformation into a higher grade, more malignant tumor.
    • Children with certain genetic syndromes, including neurofibromatosis 1 and tuberous sclerosis, are at higher risk of developing glial tumors, including gangliogliomas; however, most of these tumors develop spontaneously.

    Ganglioglioma Treatment at Dana-Farber/Boston Children's

    Children and adolescents with ganglioglioma are treated at Dana-Farber/Boston Children's through the Brain Tumor Center, one of the largest and most experienced pediatric brain tumor treatment programs in the world. Our brain tumor specialists have extensive expertise in treating all types of brain tumors, including ganglioglioma. Our patients receive care from neuro-oncologists, neurosurgeons, neurologists, and pediatric subspecialists.

    What are ganglioglioma symptoms?

    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:

    Common ganglioglioma symptoms may include:

    • Increased pressure within the brain
    • 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

    Ganglioglioma symptoms may resemble those of other conditions or medical problems. Always consult your child's physician for a diagnosis.

    How is ganglioglioma diagnosed?

    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:

    • physical examination – the child may demonstrate evidence of increased pressure in the brain
    • 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. For ganglioglioma, a CT scan of the brain is usually done.
    • 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. For ganglioglioma, an MRI of the brain and spine is usually done, however gangliogliomas of the brain rarely spread to the spine.
    • electroencephalogram (EEG) – for children experiencing seizures, this procedure records the brain's continuous, electrical activity by means of electrodes attached to the scalp, and helps identify and localize seizure activity to a particular section of the brain
    • biopsy – a tissue sample from the tumor will be taken through a needle during a simple surgical procedure performed by a surgeon to confirm the diagnosis

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

    What are ganglioglioma treatment options?

    A child’s physician will determine a specific course of ganglioglioma 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. 

    Ganglioglioma 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

    Specific ganglioglioma treatments will be determined by your child's physician based on:

    • your child's age, overall health, and medical history
    • type, location, and size of the tumor
    • extent of the disease
    • your child's tolerance for specific medications, procedures, or therapies
    • how your child's doctors expects the disease to progress
    • your opinion or preference

    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.