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Ganglioglioma

What is Ganglioglioma?

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) in the central nervous system (CNS). Most gangliogliomas occur in children and young adults. They account for less than 2% of all CNS tumors. Gangliogliomas tend to be small, low grade tumors that do not spread. However, they can sometimes be malignant, higher grade tumors.

The most common place for gangliogliomas to develop is in the cerebrum, usually in the temporal lobe. However, they can occur anywhere in the central nervous system.

Surgery is the main treatment for gangliogliomas. Radiation therapy may be used if the tumor can’t be removed or comes back. Chemotherapy and targeted therapy may also be used. Doctors sometimes recommend watching small, low grade tumors if they do not appear to be causing problems. Children with low grade ganglioglioma usually do well and have a greater than 90% survival rate.

What are Signs and Symptoms of Ganglioglioma?

Symptoms depend on tumor size and location. Because gangliogliomas tend to grow slowly, symptoms often have a gradual onset. Seizures are a main symptom of ganglioglioma.

Ganglioglioma symptoms include:

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

How is Ganglioglioma Diagnosed?

Doctors evaluate ganglioglioma in several ways.  

  • A health history and physical exam helps doctors learn about symptoms, general health, past illness, and risk factors.
    • Most gangliogliomas occur in older children and young adults. They occur slightly more often in males compared to females. Usually, the cause is unknown.
  • A neurological exam measures different aspects of brain function including memory, vision, hearing, muscle strength, balance, coordination, and reflexes.
  • An electroencephalogram (EEG), measures electrical activity of the brain. This test monitors and records seizure activity through scalp electrodes.
  • Imaging tests are used to help identify the tumor, see how big the tumor is, and find out what brain areas may be affected.
    • Magnetic resonance imaging (MRI) uses radio waves and magnets to make detailed pictures of the body. The images made by MRI can give more information about the type of tumor. MRI is also done after surgery to see if any tumor remains.
    • Computed tomography (CT scan) uses X-rays to create cross-sectional images of the organs and tissues inside the body. The machine takes many pictures to make a very detailed image. The images are taken as a series of “slices” of the body and are saved to a computer. These slices or sections can allow very small tumors to be seen.
  • A biopsy is usually used to diagnose ganglioglioma. In a biopsy, a small sample of the tumor is removed during surgery. A pathologist looks at the tissue sample under a microscope to identify the specific type of tumor.

Grading and Staging of Ganglioglioma

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

What is the Prognosis for Ganglioglioma?

For children with ganglioglioma, the most important factor that affects prognosis is the tumor grade.

Other factors that influence chance of cure include:

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

How is Ganglioglioma Treated?

The main treatment for ganglioglioma is surgery to remove as much of the tumor as possible. Radiation therapy may be used if the tumor cannot be completely removed or if the tumor recurs. New treatments using targeted therapies are being studied in clinical trials.

  1. The goal of treatment is gross total resection of the tumor. Gangliogliomas can often be managed with surgery alone and then followed with imaging.

  2. Radiation therapy may be used if surgery cannot completely remove the tumor or if the cancer returns. Doctors try to limit the use of radiation in low grade tumors due to the risk of late effects.

  3. Chemotherapy may also be used in certain cases if the tumor is not fully removed with surgery. Chemotherapy depends on the age of the child and location of the tumor. Low grade gangliogliomas are treated with the same chemotherapy treatment plans used for low grade astrocytomas.

  4. Targeted therapies work by acting on, or targeting, specific features of tumor cells. In some gangliogliomas, there appears to be a change in the BRAF gene in cells of the tumor. This change is called a V600E BRAF mutation. The BRAF gene helps to control a protein important 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 is used to monitor patients after treatment. Like other low grade gliomas, there is higher risk of recurrence if the tumor was not fully resected. Follow-up care should include appropriate rehabilitation, psychological, and neurological consultations as needed. Many patients will take anti-seizure medication after surgery, but medications can often be tapered and stopped after a period of time.

More: Life After Brain Tumors


Reviewed: June 2018