Glomus jugulare tumor

Answer 1

Definition

A glomus jugulare tumor is a tumor of a part of the temporal bone in the skull. This tumor can affect the ear, upper neck, base of the skull, and the surrounding blood vessels and nerves.

Alternative Names

Paraganglioma - glomus jugulare

Causes, incidence, and risk factors

A glomus jugulare tumor grows in the temporal bone of the skull, in an area called the jugular foramen. The jugular foramen is also where the jugular vein and several important nerves exit the skull.

This area contains glomus bodies, which are nerve fibers that normally respond to changes in body temperature or blood pressure.

These tumors usually occur later in life, around age 60 or 70, but they 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 the enzyme succinate dehydrogenase (SDHD).

Symptoms

Symptoms may include:

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

Signs and tests

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

• Cerebral angiography
• 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 is usually done by both a neurosurgeon and a head and neck surgeon (neurotologist).

In some cases, a procedure called embolization is performed before surgery to prevent the tumor from bleeding too much during surgery.

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

Some 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.

Complications

The most common complications are due to nerve damage, which may be caused by the tumor itself or damage during surgery. Nerve damage can lead to:

• Change in voice
• Difficulty swallowing
• Hearing loss
• Paralysis of the face

Calling your health care provider

Call your health care provider if you:

• Are having difficulty with hearing or swallowing
• Develop pulsations in your ear
• Notice a lump in your neck
• Notice any problems with the muscles in your face

References

Rucker JC. Cranial neuropathies. In: Bradley WG, Daroff RB, Fenichel GM, Jankovic J. Neurology in Clinical Practice. 5th ed. Philadelphia, Pa: Saunders Elsevier; 2007:chap 74.

Marsh M, Jenkins H. Temporal bone neoplasms and lateral cranial base surgery. In: Cummings CW, Flint PW, Haughey BH, et al. Otolaryngology: Head & Neck Surgery. 4th ed. St Louis, Mo: Mosby; 2005:chap 162.

Reviewed By

Review Date: 02/01/2010

David C. Dugdale, III, MD, Professor of Medicine, Division of General Medicine, Department of Medicine, University of Washington School of Medicine; and Seth Schwartz MD, MPH, Otolaryngologist, Virginia Mason Medical Center, Seattle, Washington. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.