Ganglioglioma

What is a ganglioglioma?

A ganglioglioma is a rare type of brain tumor made up of a mixture of neuronal cells and glial cells. Neuronal cells form neurons in your brain, which carry information throughout your body. Glial cells form the structure of your central nervous system and interact closely with neurons, helping them function correctly.

Gangliogliomas are also known as mixed neuronal-glial tumors. They most commonly affect children and young adults. Gangliogliomas are typically small, slow-growing and noncancerous (benign). They usually don’t spread to other parts of your body. Even though these tumors are benign, they can still cause problems and require treatment.

Most gangliogliomas are low-grade gliomas that aren’t cancerous and have a low risk of returning (recurrence). A glioma is a brain or spinal cord tumor that develops when glial cells grow out of control. Sometimes, gangliogliomas are classified as high-grade gangliogliomas, which means they can turn cancerous (malignant). These are aggressive and have a high risk of recurrence.

What are the types of gangliogliomas?

Gangliogliomas are found most often in your temporal lobes. But they may occur in any part of your brain, including your:

• Cerebrum.
• Brainstem.
• Cerebellum.
• Thalamus.
• Hypothalamus.

They may also occur in your spinal cord.

A desmoplastic infantile ganglioglioma is a very rare type of ganglioglioma that occurs before the age of 2 in the upper part of a baby’s brain.

How common are gangliogliomas?

Each year, more than 4,000 children ages 19 and younger in the U.S. receive a brain or spinal cord tumor diagnosis. Gangliogliomas are very rare. They account for about 1% to 2% of all brain tumors in children and young adults.

What are the symptoms of gangliogliomas?

Gangliogliomas most often occur near your temporal lobes, which are located near your ears. Because of their position in your brain, they tend to cause epilepsy. So, seizures may be your first clue that a ganglioglioma is present.

Other ganglioglioma symptoms usually develop slowly. Depending on the location of the tumor in your brain, symptoms may include:

• Headaches, especially upon waking in the morning.
• Nausea and vomiting.
• Difficulty swallowing.
• Double vision.
• Unsteady gait (walk).
• Fatigue.
• Dizziness.
• Weakness on one side of your body.

What causes gangliogliomas?

Most gangliogliomas develop randomly with no known cause. Researchers have discovered a genetic mutation in the BRAF gene that occurs in 10% to 60% of all gangliogliomas, but the mutation happens in a range of other tumors as well.

What are the risk factors for this condition?

Certain genetic conditions increase the risk of children developing glial tumors, including gangliogliomas. These conditions include:

• Neurofibromatosis 1 (NF1).
• Tuberous sclerosis.
• Turcot syndrome.
• Li-Fraumeni syndrome.
• von Hippel-Lindau syndrome.

In addition, brain and spinal cord tumors occur more frequently in children assigned male at birth (AMAB) than in children assigned female at birth (AFAB). These tumors occur in white children more than any other racial/ethnic group.

How are gangliogliomas diagnosed?

Your child’s healthcare provider will perform a physical exam and ask you about your child’s symptoms. They’ll go over your child’s medical history and perform a neurological exam as well. The neuro exam tests your child’s reflexes, muscle strength, mouth and eye movement, and alertness and coordination. Your child’s provider will likely order additional tests to make a diagnosis. These tests may include:

• Computed tomography (CT) scan: This test uses a series of X-rays and a computer to produce images of your child’s brain.
• Brain magnetic resonance imaging (MRI) scan: This test uses magnets, radio waves and a computer to produce images of your child’s brain.
• Electroencephalogram (EEG): This test measures and records your child’s brain’s electrical signals and activity.
• Biopsy: Depending on your child’s situation, their provider may refer to a neurosurgeon to perform a biopsy to obtain samples of tissue that a pathologist will examine under a microscope and send for additional testing (such as genomic testing).

How do you treat ganglioglioma?

To make sure your child receives the best possible treatment, they should receive care at a pediatric medical center. They should see a board-certified pediatric oncologist or neuro-oncologist with extensive experience in treating gangliogliomas.

Your child’s ganglioglioma treatment will depend on several factors, including:

• Your child’s overall health, age and medical history.
• The type, location and size of the tumor.
• Severity of the tumor.
• Your child's tolerance for specific therapies, procedures or medications.
• How your child’s providers expect the tumor to progress.

The first line of treatment is usually surgery to remove the tumor. Your child’s provider will remove as much of the tumor as possible. Surgery is sometimes limited due to the placement of the tumor in your child’s brain. If your child’s provider can’t remove the entire tumor and it’s high-grade, they may proceed with radiation therapy.

Radiation therapy is a procedure that uses high-energy X-rays and other types of radiation to destroy cancer cells and/or stop them from growing and dividing. Radiation therapy can precisely target the tumor. Your child’s provider may recommend radiation therapy for progressive or recurrent ganglioglioma.

Your child’s healthcare provider may also choose to use chemotherapy, a drug treatment that kills or shrinks cancer cells. They may also use targeted therapy, a drug treatment that attacks a specific marker in the tumor. Many gangliogliomas have specific markers that targeted therapy can destroy, such as BRAF mutations.

Providers may recommend clinical trials for children with recurrent tumors that aren’t responsive to other therapies.

Can gangliogliomas be prevented?

Researchers don’t know why gangliogliomas occur in some children and not others. There’s no way to prevent the condition.

What is the ganglioglioma prognosis?

The overall five-year survival rate for a ganglioglioma is more than 90%. That means the likelihood of your child being alive five years after they’ve received their diagnosis is very good. Survival depends on many factors, including the tumor grade, your child’s age and the location of the tumor.

What is the likelihood of relapse?

More than 95% of gangliogliomas are considered low-grade and won’t return after a complete tumor removal. Higher-grade gliomas like grade 3 gangliogliomas are more likely to recur (return) after surgery.

When should my child see their healthcare provider?

If your child experiences a seizure or any other ganglioglioma symptoms, they should see their healthcare provider for an evaluation. Ganglioglioma symptoms may look like the symptoms of other health conditions, so it’s important to see your child’s provider for a proper diagnosis.

What questions should I ask my child’s healthcare provider?

If your child has a ganglioglioma, you probably have many questions. Some questions you may want to ask your child’s provider include:

• What type of ganglioglioma does my child have?
• Is it cancerous?
• How will the tumor affect my child’s brain and health?
• What are the treatment options?
• Can you recommend a support group for families with children with gangliogliomas?