National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Gangliocytoma

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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.

The following information may help to address your question:


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.[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

What are the signs and symptoms of gangliocytomas?

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

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.[3]
Last updated: 5/11/2015

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.[3] 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.[3]
Last updated: 5/11/2015

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.[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

We hope this information is helpful. We strongly recommend you discuss this information with your doctor. If you still have questions, please contact us.

Warm regards,
GARD Information Specialist

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  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.