How rare are gangliocytomas?

The incidence of gangliocytomas has been reported to range from 0.1 to 0.5% among all central nervous system (CNS) tumors.

Last updated on 05-01-20

What is gangliocytoma?

Gangliocytoma is a rare type of central nervous system (CNS) tumor made up of mature neurons. Gangliocytomas may occur in all age groups but most often occur in people between the ages of 10 and 30. The most common site is the temporal lobe of the brain, but they can arise anywhere in the CNS including the cerebellum, brainstem, floor of the third ventricle, and spinal cord. They are among the most frequent tumors associated with epilepsy. Signs and symptoms may depend on the tumor's location and may include seizures (most commonly); increased brain pressure; endocrine disorders; and focal symptoms. Gangliocytomas are generally slow-growing and usually do not become malignant. Treatment involves surgical removal of the tumor.

Click here to view a separate page about dysplastic gangliocytoma of the cerebellum (also called Lhermitte-Duclose disease).

Last updated on 05-01-20

What is known about gangliocytomas of the spine?

Gangliocytomas are rarely found in the spine, accounting for fewer than 10% of all gangliocytomas. They occur more frequently in the thoracolumbar region, but cases involving the cervical spine (through the neck area) have also been described. Depending on the location of the tumor, people with spinal gangliocytomas may have symptoms such as radiculopathy (a condition of the nerve roots), paraparesis (partial paralysis of the legs), or cauda equina syndrome. Scoliosis has been described as a presenting sign in people with thoracic dumbbell gangliocytomas.

The intramedullary form of spinal gangliocytoma is exceedingly rare. Most of the cases in the literature report a favorable prognosis after a complete resection of the mass, but a good prognosis even after subtotal resection, without post-operative adjuvant treatment, has also been reported.

Last updated on 05-01-20

What is the long-term outlook for people with gangliocytoma?

Gangliocytoma is considered a low-grade tumor (WHO Grade I) with a benign course. Grade I tumors are considered the least malignant tumors and are usually associated with long-term survival. They grow slowly. Surgery alone is often an effective treatment for a Grade I tumor.

Complete resection (removal) of supratentorial gangliocytomas can be achieved in more than 75% of cases. Even after subtotal (partial) resection, clinically relevant re-growth of the tumor is rare. Therefore, although total resection is optimal, the outlook is reportedly good even after subtotal resection.

Morbidity and mortality related to surgery for gangliocytomas generally depends on the location of the tumor. Resection performed in some areas may carry significantly more risk than in others.

Last updated on 05-01-20

Name: Children's Tumor Foundation CTF 120 Wall Street, 16th floor
New York, NY, 10005-3904, United States
Phone: +1-212-344-6633 Toll Free: 1-800-323-7938 Fax : +1-212-747-0004 Email: Url:
Name: American Brain Tumor Association 8550 W. Bryn Mawr Ave, Ste 550
Chicago, IL, 60631, United States
Phone: +1-773-577-8750 Toll Free: 1-800-886-2282 Fax : +1-773-577-8738 Email: Url:
Name: National Brain Tumor Society 55 Chapel St., Suite 200
Newton, MA, 02458, United States
Phone: +1-617-924-9997 Fax : +1-617-924 -9998 Email: Url:
Name: Pediatric Brain Tumor Foundation 302 Ridgefield Court
Asheville, NC, 28806 , United States
Phone: +1-828-665-6891 Toll Free: 800-253-6530 Fax : +1-828-665-6894 Email: Url:
Name: Children's Brain Tumor Foundation 274 Madison Avenue, Suite 1004
New York, NY, 10016 , United States
Toll Free: 1-866-228-4673 Email: Url:
Name: International Brain Tumour Alliance 10 Carrodus Street, Fraser, ACT, 2615
Email: Url:

Connect with other users with Gangliocytoma on the RareGuru app

Do you have information about a disease, disorder, or syndrome? Want to suggest a symptom?
Please send suggestions to RareGuru!

The RareGuru disease database is regularly updated using data generously provided by GARD, the United States Genetic and Rare Disease Information Center.

People Using the App

Join the RareGuru Community

To connect, share, empower and heal today.

People Using the App