Glomus jugulare tumor Health Article

Definition

A glomus jugulare tumor is a tumor that can affect the ear, upper neck, base of the skull, and the surrounding blood vessels and nerves.

Alternative Names

Tumor - Jacobson's nerve; Jacobson's nerve tumor; Tumor - temporal bone; Paraganglioma; Glomus tympanicum

Causes, incidence, and risk factors

A glomus jugulare tumor grows in the bones of the skull, where nerves are located. Specifically, the tumor occurs in part of the jugular vein (a large vein in the neck) or along a certain nerve on the side of the skull (temporal bone).

Both of these areas contain glomus bodies, which are nerve fibers that normally respond to change in body temperature or blood pressure. A person with a glomus jugulare tumor may have hearing loss, dizziness, or a feeling of a loud pulsing in the ear.

These tumors usually occur later in life, around age 60 or 70, but can appear at any age. The cause of a glomus jugulare tumor is unknown. Usually, there are no known risk factors. Glomus tumors have been associated with changes (mutations) in a gene responsible for succinate dehydrogenase (SDHD), an enzyme.

Symptoms

Symptoms may include:

• Difficulty swallowing (dysphagia)
• Hearing problems
• Hoarseness
• Pain
• Weakness or loss of movement in the face (cranial nerve palsy)

Signs and tests

Glomus jugulare tumors are diagnosed by physical examination and various scans including:

• Cerebral angiography (injection of dye so that the blood vessels of the neck and brain can be seen with x-rays)
• CT scan
• MRI scan

Treatment

Glomus jugulare tumors are rarely cancerous and do not tend to spread to other parts of the body. However, treatment may be needed to relieve symptoms. The main treatment is surgery. Surgery is complex and usually done by both a neurosurgeon and a head and neck surgeon.

In some cases, a procedure called embolization is performed before surgery to shrink the tumor.

After surgery, radiation therapy may be used to treat any part of the tumor that could not be removed completely.

Glomus tumors can be treated with stereotactic radiosurgery.

Expectations (prognosis)

Patients who have surgery or radiation tend to do well. More than 90% of those with glomus jugulare tumors are cured.