Ganglioglioma is a rare brain tumor that usually begins in the temporal lobe but it can also occur in other parts of the brain and spinal cord.
A ganglioglioma is a rare brain tumor that grows from nerve cells (neurons) and supportive cells (glial cells). Gangliogliomas usually develop in the temporal lobe, a brain area located behind the ears. This area is important for memory, understanding language, and processing emotions. But gangliogliomas can also occur in other parts of the brain and spinal cord and affect a variety of brain functions.
Most gangliogliomas occur in children and young adults. About 95% of gangliogliomas are low-grade gliomas. These tumors generally grow slowly and are noncancerous, meaning they do not spread to other parts of the body. But a small number of gangliogliomas (5–10%) are malignant (cancerous), high-grade tumors.
Treatment usually involves surgery to remove as much of the tumor as possible. If the whole tumor cannot be removed or if it starts to grow again, doctors may use treatments like radiation, chemotherapy, or targeted therapy.
Most people with ganglioglioma do well after treatment, especially if the tumor is found early. Low-grade gangliogliomas rarely recur (come back) after complete surgical removal.
Symptoms of ganglioglioma
Ganglioglioma symptoms depend on the tumor’s size and location. Because these tumors tend to grow slowly, symptoms may be gradual.
Signs and symptoms of ganglioglioma may include:
Seizures are the most common symptom. Seizures happen because gangliogliomas can disrupt normal brain activity in the areas where they grow.
A biopsy to look at a sample of the tumor under a microscope to identify the type and grade of glioma.
Genetic and molecular testing of the tumor to look for specific changes (mutations) in tumor cells. These changes help doctors make the correct diagnosis and choose the best treatment.
Types of ganglioglioma
Most gangliogliomas are low-grade tumors that can occur in any part of the brain or spinal cord. They grow slowly, are not cancerous, and have a low risk of coming back (recurring).
About 5–10% of gangliogliomas are high-grade tumors, known as anaplastic gangliogliomas. These tend to grow faster and require stronger treatment.
Desmoplastic infantile ganglioglioma is a rare type of ganglioglioma that occurs in very young children under age 2. It often appears as a large tumor but is typically low grade and responds well to treatment.
Treatment of ganglioglioma
Treatment usually involves surgery to remove as much of the tumor as possible. If the whole tumor cannot be removed or if it comes back, ganglioglioma might be treated with radiation, chemotherapy, or targeted therapy. If the tumor is not causing symptoms and is not growing, doctors may recommend regular imaging and monitoring instead of surgery.
Surgery for ganglioglioma
The goal of surgery is to remove as much of the tumor as possible. Surgery alone can often treat gangliogliomas.
Radiation therapy for ganglioglioma
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.
Chemotherapy for ganglioglioma
Chemotherapy may help some patients if surgery cannot completely remove the tumor. Chemotherapy depends on the age of the child and the tumor’s location.
Targeted therapy drugs for ganglioglioma
Targeted therapies work by acting on, or targeting, certain features of tumor cells. These drugs look for changes in the cells that help the tumor grow. Unlike regular chemotherapy, targeted therapy attacks only certain parts of the tumor, which can help protect healthy cells.
Some gangliogliomas have changes (mutations) in a gene called BRAF. One common change is called the BRAF V600E mutation. If your child’s tumor has this mutation, doctors might use targeted therapy to help stop the tumor from growing.
Targeted therapy drugs for ganglioglioma with a BRAF V600E mutation include vemurafenib and dabrafenib. These are called BRAF inhibitors because they block the BRAF protein. Sometimes, another drug called a MEK inhibitor (such as trametinib) is used along with a BRAF inhibitor to make the treatment work better.
Prognosis for ganglioglioma
Children with ganglioglioma usually do well. Over 90% survive at least 5 years after diagnosis.
Factors that influence the chance of a cure include:
Tumor grade
If the tumor can be completely removed with surgery
Location of the tumor
If the cancer is new or if it has come back (recurrent)
Support for patients with ganglioglioma
Anti-seizure medicines are often prescribed after surgery to prevent seizures. If no seizures occur over time, doctors may gradually reduce the dosage and eventually stop the medication.
Regular imaging is used to monitor patients after treatment.
Questions to ask your care team
What type of ganglioglioma does my child have?
Is the tumor low grade or high grade?
Where is the tumor located, and how does its location affect treatment?
Can the tumor be completely removed with surgery?
Will my child need radiation or chemotherapy? What are the risks or side effects?
Are there clinical trials or targeted therapies that might help?
What are the chances of the tumor coming back?
Should we consider genetic testing for BRAF or other mutations?
How often will imaging or check-ups be needed after treatment?
Will my child need anti-seizure medication after surgery? For how long?
What are the possible long-term effects of treatment on my child’s brain function and development?
Key points about ganglioglioma
Ganglioglioma is a rare brain tumor made of nerve and supportive cells in the central nervous system.
Most gangliogliomas are low-grade, slow-growing, and noncancerous.
These tumors usually develop in the temporal lobe, which controls memory, language, and emotions.
Seizures are the most common symptom, but other symptoms include headaches, nausea, vomiting, fatigue, and weakness.
Surgery is the main treatment, aiming to remove as much of the tumor as possible. Radiation, chemotherapy, or targeted therapy may be used if the tumor cannot be fully removed or comes back.
Genetic changes, like the BRAF mutation, may play a role in some gangliogliomas.
Children with ganglioglioma usually have an excellent prognosis, with over 90% surviving at least 5 years after diagnosis.
Follow-up care includes imaging, rehabilitation, and psychological support. Anti-seizure medicines may be needed after surgery.
Low-grade gliomas are the most common brain and spinal cord tumor in children. Learn about symptoms, diagnosis, and treatment of pediatric low-grade glioma.
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