Gangliocytoma

Title

Categories:

Nervous System Diseases; Rare Cancers

Summary Summary

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.^[1]^[2]

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

Last updated: 5/11/2015

Symptoms Symptoms

Signs and symptoms caused by the presence of a gangliocytoma can vary depending on the tumor's location. Seizures are the most common symptom. Other symptoms may include increased brain pressure, endocrine disorders, and focal symptoms.^[1]^[2] Gangliocytomas can also be asymptomatic (cause no symptoms) and may be diagnosed incidentally on imaging studies.^[3]

Last updated: 5/11/2015

Prognosis Prognosis

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.^[4]

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.^[5]

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.^[5]

Last updated: 5/12/2015

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Organizations Organizations

Nonprofit support and advocacy groups bring together patients, families, medical professionals, and researchers. These groups often raise awareness, provide support, and develop patient-centered information. Many are the driving force behind research for better treatments and possible cures. They can direct people to research, resources, and services. Many groups also have experts who serve as medical advisors. Visit their website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Brain Tumor Association
8550 W. Bryn Mawr Ave, Ste 550
Chicago, IL 60631
Toll-free: 800-886-2282
Telephone: 847-827-9910
E-mail: info@abta.org
Website: http://www.abta.org
Brain Tumor Foundation for Children, Inc.
6065 Roswell Road, Suite 505
Atlanta, GA 30328-4015
Telephone: 404-252-4107
E-mail: http://www.braintumorkids.org/web/contactus.asp
Website: http://www.braintumorkids.org
Children's Brain Tumor Foundation
274 Madison Avenue, Suite 1004
New York, NY 10016
Toll-free: 866-228-4673
E-mail: info@cbtf.org
Website: http://www.cbtf.org
International Brain Tumour Alliance
10 Carrodus Street,
Fraser, ACT, 2615
Australia
E-mail: chair@theibta.org
Website: http://www.theibta.org/
National Brain Tumor Society
124 Watertown St, Suite 2D
Watertown, MA 02472
Toll-free: 800-770-8287
Telephone: 617-924-9997
E-mail: info@braintumor.org
Website: http://www.braintumor.org
Pediatric Brain Tumor Foundation of the United States
302 Ridgefield Court
Asheville, NC 28806
Toll-free: 800-253-6530
Telephone: 828-665-6891
E-mail: pbtfus@pbtfus.org
Website: http://www.pbtfus.org
The Children's Tumor Foundation
120 Wall Street, 16th floor
New York, NY 10005-3904
Toll-free: 800-323-7938
Telephone: 212-344-6633
Fax: 212-747-0004
E-mail: info@ctf.org/
Website: http://www.ctf.org/

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Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

In-Depth Information

The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Gangliocytoma. Click on the link to view a sample search on this topic.

News & Events News & Events

NCATS Co-Sponsored Conferences

Overcoming Barriers to International Clinical Trials for Rare Cancers Friday, December 10, 2010
Location: Bethesda, Maryland
Description: The goals of this conference were to introduce key institutional players to the topic of international clinical trials in rare cancers and to establish an ongoing dialogue. Participants left the meeting with a set of specific priorities that need to be enacted to promote these trials. The meeting promoted consensus on the way that resources are prioritized to address rare cancers. Participants were asked to convey the content of the meeting to their constituencies and to follow up with pilot concepts.

Contact: Jack Welch, M.D., Ph.D., jack.welch@nih.govjack.welch@nih.gov

Co-funding Institute(s): National Cancer Institute, Office of Rare Diseases Research
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GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

My daughter was diagnosed with a primary intramedullary spinal cord tumor. She had surgery to remove the tumor. They could only manage partial debulking of the tumor. The histopathology report revealed it is a rare tumor called gangliocytoma. There is little information about this tumor anywhere and I was hoping you could provide information. See answer

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References References

Adesina A, Rauch R. Ganglioglioma and Gangliocytoma. Atlas of Pediatric Brain Tumors. Springer International Publishing; 2010;
Türeyen K, Senol N, Sav A. Gangliocytoma associated with focal cortical dysplasia in a young-adult: a case report. Turk Neurosurg. July, 2008; 18(3):259-263.
Jacob JT, Cohen-Gadol AA, Scheithauer BW, Krauss WE. Intramedullary spinal cord gangliocytoma: case report and a review of the literature. Neurosurg Rev. October, 2005; 28(4):326-329.
Tumor Grading and Staging. American Brain Tumor Association. 2014; http://www.abta.org/brain-tumor-information/diagnosis/grading-staging.html.
Jörg-Christian Tonn, Manfred Westphal, J. T. Rutka. Ganglioglioma and Gangliocytoma. Oncology of CNS Tumors: Second Edition. Springer; February, 2010; 196-198.