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

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Ganglioneuroma		0003005
30%-79% of people have these symptoms
Headache	Headaches	0002315
Hemianopia		0012377
Spinal cord tumor	Tumor of the spinal cord	0010302
5%-29% of people have these symptoms
Abnormal prolactin level		0040086
Abnormality of brainstem morphology	Abnormal shape of brainstem	0002363
Accelerated skeletal maturation	Advanced bone age Early bone maturation [ more ]	0005616
Adrenocorticotropic hormone excess		0011749
Amenorrhea	Abnormal absence of menstruation	0000141
Decreased female libido	Decreased female sex drive	0030018
Distal muscle weakness	Weakness of outermost muscles	0002460
Focal-onset seizure	Seizure affecting one half of brain	0007359
Growth hormone excess		0000845
Hyperhidrosis	Excessive sweating Increased sweating Profuse sweating Sweating Sweating profusely Sweating, increased [ more ]	0000975
Impotence	Difficulty getting a full erection Difficulty getting an erection [ more ]	0000802
Paresthesia	Pins and needles feeling Tingling [ more ]	0003401
Pituitary null cell adenoma		0011761
Scoliosis		0002650
1%-4% of people have these symptoms
Abnormal cerebellum morphology	Abnormality of the cerebellum Cerebellar abnormalities Cerebellar abnormality Cerebellar anomaly [ more ]	0001317
Dementia	Dementia, progressive Progressive dementia [ more ]	0000726
Excessive daytime somnolence	Excessive daytime sleepiness More than typical sleepiness during day [ more ]	0001262
Pituitary prolactin cell adenoma		0006767
Polyphagia	Voracious appetite	0002591
Syringomyelia	Fluid-filled cyst in spinal cord	0003396

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Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 10/1/2020

Do you have updated information on this disease? We want to hear from you.

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

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s 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: 1-800-886-2282
Telephone: +1-773-577-8750
Fax: +1-773-577-8738
E-mail: info@abta.org
Website: https://www.abta.org/
Children's Brain Tumor Foundation
274 Madison Avenue, Suite 1004
New York, NY 10016
Toll-free: 1-866-228-4673
E-mail: info@cbtf.org
Website: http://www.cbtf.org
Children's Tumor Foundation (CTF)
120 Wall Street, 16th floor
New York, NY 10005-3904
Toll-free: 1-800-323-7938
Telephone: +1-212-344-6633
Fax: +1-212-747-0004
E-mail: info@ctf.org
Website: https://www.ctf.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
55 Chapel St., Suite 200
Newton, MA 02458
Telephone: +1-617-924-9997
Fax: +1-617-924 -9998
E-mail: http://braintumor.org/our-mission/contact-us/
Website: http://braintumor.org/
Pediatric Brain Tumor Foundation
302 Ridgefield Court
Asheville, NC 28806
Toll-free: 800-253-6530
Telephone: +1-828-665-6891
Fax: +1-828-665-6894
E-mail: info@curethekids.org
Website: http://www.curethekids.org/

Do you know of an organization? We want to hear from you.

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

News

Rare Diseases Are Not Rare - Gallery of Creative Work Raises Awareness of Rare Diseases
September 1, 2020
NIH-Supported Research Survey to Examine Impact of COVID-19 on Rare Diseases Community
May 22, 2020
NCATS Translational Approach Addresses COVID-19
May 21, 2020

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

Have a question? Contact a GARD Information Specialist.

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.