Ganglioneuroma

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Central nervous system

Definition

Ganglioneuroma is a tumor of the peripheral nervous system.

Causes, incidence, and risk factors

Ganglioneuromas are rare tumors that most frequently start in the autonomic nerve cells, which may be in any part of the body. The tumor are usually noncancerous (benign).

Ganglioneuromas usually occur in people ages 10 to 40. They grow slowly, and may release certain chemicals or hormones.

There are no known risk factors. However, the tumors may be associated with some genetic problems, such as neurofibromatosis type 1.

Symptoms

A ganglioneuroma usually causes no symptoms, and is only discovered when being examined or treated for another condition.

Symptoms depend on the location of the tumor and the type of chemicals released.

If the tumor is in the chest area (mediastinum), symptoms may include:

• Breathing difficulty
• Chest pain
• Compression of the windpipe (trachea)

If the tumor is lower down in the abdomen in the area called the retroperitoneal space, symptoms may include:

• Abdominal pain
• Bloating

If the tumor is near the spinal cord, it may cause:

• Compression of the spinal cord, which leads to pain and loss of strength or feeling in the legs, the arms, or both
• Spine deformity

These tumors may produce certain hormones, which can cause the following symptoms:

• Diarrhea
• Enlarged clitoris (women)
• High blood pressure
• Increased body hair
• Sweating

Signs and tests

The best tools to identify a ganglioneuroma are:

• CT scan
• MRI scan

Blood and urine tests may be done to determine if the tumor is producing hormones or other chemicals.

A biopsy or complete removal of the tumor may be needed to confirm the diagnosis.

Treatment

Treatment involves surgery to remove the tumor (if it is causing symptoms).

Expectations (prognosis)

Most ganglioneuromas are noncancerous. The expected outcome is usually good. A ganglioneuroma may, however, become cancerous and spread to other areas, or it may come back after removal.

Complications

If the tumor has been present for a long time and has pressed on the spinal cord or caused other symptoms, surgery to remove the tumor may not necessarily reverse the damage.

Compression of the spinal cord may result in loss of movement (paralysis), especially if the cause is not detected promptly.

Surgery to remove the tumor may also lead to complications in some cases.

Calling your health care provider

Call your health care provider if you or your child has symptoms that may be caused by this type of tumor.

References

Sovak MA, Aisner SC, Aisner J. Tumors of the Pleura and Mediastinum. In: Abeloff MD, Armitage JO, Niederhuber JE, Kastan MB, McKenna WG, eds. Abeloff's Clinical Oncology. 4th ed. Philadelphia, Pa; Churchill Livingstone Elsevier; 2008: chap 77.

Review Date: 9/22/2008

Reviewed by: Daniel B. Hoch, PhD, MD, Assistant Professor of Neurology, Harvard Medical School, Department of Neurology, Massachusetts General Hospital. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.

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