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Ganglioglioma

What is a ganglioglioma tumor?

Gangliogliomas are rare tumors found in the brain and spinal cord.

They grow from groups of nerve cells (ganglion cells) and supportive cells (glial cells). These cells are in the central nervous system (CNS).

Gangliogliomas usually develop in the cerebrum. But they can occur anywhere in the central nervous system. Gangliogliomas tend to be small, low-grade tumors that don’t spread. But sometimes they are malignant , higher-grade tumors.

  • Most gangliogliomas occur in children and young adults.
  • They’re more common in males.
  • The cause is usually unknown.
Gangliogliomas are a type of brain tumor that most often develop in the cerebrum.

Gangliogliomas are a type of brain tumor that most often develop in the cerebrum.

What is the prognosis for ganglioglioma?

Children with ganglioglioma usually do well. They have a greater than 90% five-year survival rate. Doctors sometimes recommend watching small, non-growing stable tumors instead of surgery.

When doctors recommend treatment, biopsy/surgery is usually the preferred option. Radiation therapy may help if surgery can’t remove the tumor or it comes back. Chemotherapy and targeted therapy are options, too.

What are signs and symptoms of ganglioglioma?

Seizures are a key symptom of ganglioglioma.

Other symptoms include:

  • Headache
  • Nausea and vomiting
  • Fatigue
  • Weakness on one side of the body

Ganglioglioma symptoms depend on tumor size and location. Because gangliogliomas tend to grow slowly, symptoms may be gradual.

How is ganglioglioma diagnosed?

Doctors look for ganglioglioma in several ways.

  • A biopsy/surgery usually diagnoses ganglioglioma.
    • During a biopsy, a surgeon removes a small section of the tumor. A pathologist checks the tissue sample under a microscope to identify the tumor.
  • A health history and physical exam help doctors learn about:
    • Symptoms
    • General health
    • Past illness
    • Risk factors
  • A neurological exam measures different aspects of brain function like:
    • Memory
    • Vision
    • Hearing
    • Muscle strength
    • Balance
    • Coordination
    • Reflexes
  • An electroencephalogram measures the electrical activity of the brain. This test monitors and records seizure activity through scalp electrodes.
  • Imaging tests help identify the tumor, see how big the tumor is, and find affected areas.
    • Magnetic resonance imaging (MRI) uses radio waves and magnets to take pictures of the body. These images can give more information about the tumor. MRI is also done after surgery to see if any of the tumor remains.
    • Computed tomography (CT scan) uses X-rays to create images of the inside of the body. The machine takes many pictures to make a detailed image. Doctors can see small tumors because of these images.

Grading and staging of ganglioglioma

Gangliogliomas are generally low-grade tumors. In most cases, they are slow-growing and not considered malignant. But about 5-10% of ganglioglioma are more aggressive, high-grade tumors.

What is the ganglioglioma brain tumor prognosis?

Tumor grade is the chief factor that affects a patient’s prognosis.

Other factors that influence the chance of a cure include:

  • Whether surgery can remove the tumor
  • Location of the tumor
  • If the cancer is new or if it has come back (recurrent)

How are ganglioglioma brain tumors treated?

Surgery to remove the tumor is the primary treatment for ganglioglioma.

Radiation therapy may help if the tumor can’t be completely removed. It may also help if the tumor comes back. New treatments using targeted therapies are in clinical trials.

  1. Surgery

    The goal of treatment is removal the tumor. Surgery alone can often manage gangliogliomas.

  2. Radiation therapy

    Radiation therapy may help if the surgery can’t remove the entire tumor. It can also help if cancer returns. Doctors try to limit the use of radiation in low-grade tumors due to the risk of late effects.

    Radiation therapy is used in addition to surgery, especially if limited surgery was performed. This therapy uses beams of radiation, X-rays or protons, to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.

    Radiation therapy is used with limited surgery or when tumor recurs or when chemotherapy/targeted therapy fail. This therapy uses X-ray beam radiation to shrink tumors and kill cancer cells. Radiation works by damaging the DNA inside cancer cells.

  3. Chemotherapy

    Chemotherapy may help some patients if surgery can’t completely remove the tumor. Chemotherapy depends on the age of the child and the tumor’s location.

  4. Targeted therapy

    Targeted therapies work by acting on, or targeting, specific features of tumor cells. In some gangliogliomas, there may be a change to the BRAF gene in tumor cells. This is a V600E BRAF mutation.

    A mutation is a change in the cell’s DNA sequence. Mistakes during cell division can cause mutations. Or exposure to things in the environment that damage DNA may cause them.

    Mutations can be harmful, beneficial, or unnoticeable. Some mutations may lead to cancer or other diseases.

    The BRAF gene helps to control a vital protein for cell growth and function. Certain drugs can block this specific signaling pathway. These V600E BRAF inhibitors include vemurafenib and dabrafenib.

     

Life after ganglioglioma

Periodic imaging helps check patients after treatment. Follow-up care should include rehabilitation, psychological, and neurological consultations as needed.

Many patients will take anti-seizure medication after surgery. Patients can sometimes taper and stop taking them after a period.

A recurrence is more likely if the tumor wasn’t completely removed. 

—
Reviewed: October 2021

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