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  3. Gangliocytoma
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Gangliocytoma


Title


Categories:
Nervous System Diseases; Rare Cancers

Summary Summary


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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


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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.

Showing of 24 |
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
Erectile dysfunction
[ more ]
0000802
Paresthesia
Pins and needles feeling
Tingling
[ more ]
0003401
Pituitary null cell adenoma 0011761
Scoliosis
Abnormal curving of the spine
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
More than typical sleepiness during day
0001262
Pituitary prolactin cell adenoma 0006767
Polyphagia
Voracious appetite
0002591
Syringomyelia
Fluid-filled cyst in spinal cord
0003396
Showing of 24 |
Do you have more information about symptoms of this disease? We want to hear from you.
Last updated: 12/1/2019
Do you have updated information on this disease? We want to hear from you.

Prognosis Prognosis


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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


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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


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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.

GARD Answers GARD Answers


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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


  1. Adesina A, Rauch R. Ganglioglioma and Gangliocytoma. Atlas of Pediatric Brain Tumors. Springer International Publishing; 2010;
  2. 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.
  3. 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.
  4. Tumor Grading and Staging. American Brain Tumor Association. 2014; http://www.abta.org/brain-tumor-information/diagnosis/grading-staging.html.
  5. Jörg-Christian Tonn, Manfred Westphal, J. T. Rutka. Ganglioglioma and Gangliocytoma. Oncology of CNS Tumors: Second Edition. Springer; February, 2010; 196-198.
Do you know of a review article? We want to hear from you.
You can help advance
rare disease research!
You can help advance rare disease research!
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