Ganglioneuroma

Definition
Ganglioneuroma is a usually-benign tumor of the peripheral nervous system.

Causes, incidence, and risk factors

Ganglioneuromas are rare tumors that occur in approximately 1 in 100,000 children. They most frequently arise in association with autonomic nerve cells, which may be in any part of the body.

Ganglioneuromas most frequently occur in people between age 10 and 40. They are slow-growing masses and may secrete neurochemicals or hormones. Most commonly, they are asymptomatic and discovered in the course of a normal exam or during treatment for some other condition.

Though there are no known risk factors, some reports suggests that they may be associated with some genetic problems, for instance, neurofibromatosis type 1.

Symptoms

Symptoms depend on the location of the mass and what type of neurochemicals it is secreting.

If the tumor is in the mediastinum (chest), it may cause chest pain, difficulty breathing, or compression of the trachea. If the tumor is in the retro-peritoneal space (lower in the abdomen), it may result in abdominal pain and distension. If the tumor is near the spinal cord, it may cause spine deformity and possible compression of the spinal cord.

Active hormone secretion may produce diarrhea, sweating, high blood pressure, and symptoms of virilization - increased body hair or enlarged clitoris in females - if testosterone is being secreted.

Signs and tests

The best methods to identify this tumor are imaging techniques such as CAT scans (special type of x-rays) or MRI scans (a big magnet used to generate pictures) of the area in question.

To determine if the tumor produces certain substances in excess (mostly hormones), blood and urine tests to detect those substances may be done. Ultimately, a biopsy or complete removal of the tumor may be necessary to confirm the diagnosis.

Treatment
Treatment involves the removal of the tumor by surgery (if it is symptomatic).

Expectations (prognosis)
Prognosis is usually good, because this tumor is generally benign. Rarely, a ganglioneuroma may become malignant and metastasize (spread) or recur.

Complications
Complications may occur as a result of surgery. If the tumor has been present for a long time and is causing symptoms (such as spinal cord compression), removal of the tumor may not necessarily reverse the deficits. Compression of the spinal cord may result in paralysis, especially if the cause is not detected promptly.

Calling your health care provider
Call your health care provider if you or your child experiences any of the above symptoms that may be caused by this type of tumor.

Johns Hopkins patient information

Last revised: December 6, 2012
by Dave R. Roger, M.D.

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