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​Glomus Tumors

Glomus tumors, or paragangliomas, are slow-growing, benign tumors of the neuroendocrine tissues in the body. They are rare but most commonly occur in the head, neck, and adrenal gland. In very rare situations, they are malignant.

Glomus tumors occur in three places in the head and neck:

  • Middle ear
  • Jugular bulb
  • Carotid artery

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What Is a Glomus Tumor?

Glomus tumors — also known as paragangliomas — of the head and neck are benign (noncancerous) tumors that form from glomus cells.

Glomus tumors are highly vascular and may exist alone or, in around 10% of cases, can occur with multiple glomus tumors in the same patient. Rarely, they are associated with other tumors in the adrenal gland, thyroid, or parathyroid glands.

In the head and neck, glomus tumors can be in the:

  • Jugular bulb (glomus jugulare) — Glomus tumors are most common in this region of the jugular vein just below the middle ear. Tumors in this area may grow into the middle ear, skull base, and cranial cavity.
  • Middle ear (glomus tympanicum) — Tumors that start in glomus cells of the middle ear are glomus tympanicum tumors. These are the most common vascular tumors of the middle ear.
  • Carotid artery (glomus vagale) — Glomus tumors may also form in the deep neck (parapharyngeal) area along the course of the vagus nerve. These are glomus vagale tumors.

Because they tend to be benign and grow slowly, mortality rates are very low. But glomus tumors can damage the tissue and nerves around them as they grow. In rare cases they can be malignant. They may also produce hormones that can affect your blood pressure, heart rate, and can sometimes make anesthesia dangerous.

What causes a glomus tumor?

The exact cause of a glomus tumor is unknown, and there are no known risk factors.

Are glomus tumors hereditary?

Glomus tumors are associated with a family of hereditary genetic mutations that can be passed on to your children. It may be important to be tested for these genetic conditions so you make appropriate family planning decisions.

But glomus tumors also may occur without any family history — meaning they can form from gene mutations that happen over time throughout your life.

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What Are the Symptoms of Glomus Tumors?

The symptoms of glomus tumors depend on where the tumors are in your body.

In rare cases, these tumors may produce hormones that may cause:

  • Anxiety.
  • Headaches.
  • High blood pressure.
  • Increased heart rate.

The symptoms of glomus tumors depend on where the tumors are in your body. 

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How Do You Diagnose Glomus Tumors?

To diagnose a glomus tumor, your doctor will:

  • Ask about symptoms you're having. In the middle ear, most glomus tympanicum or jugulare tumors cause hearing loss and pulsatile tinnitus.
  • Do an exam. Doctors do a physical exam to find these tumors, which appear as reddish-blue masses behind the eardrum. They may inspect the vocal cords and back of the throat or neck for other tumors.
  • Order imaging and other tests.

Tests for diagnosing glomus tumors

Your doctor my order tests to diagnose your glomus tumors:
  • Angiogram.
  • Audiogram.
  • CT scan.
  • MRI scan.
  • Nuclear medicine scan (such as a DOTA scan).
  • Urine or blood tests to screen for tumor-produced hormones.
  • Genetic testing.

Because of the association of these tumors with other tumors and because these tumors can produce hormones, it is important to have a full workup before proceeding with management of a glomus tumor.

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How Do You Treat Glomus Tumors?

At UPMC, the approach to managing glomus tumors is comprehensive and multidisciplinary. We aim to manage both the tumor itself and the symptoms it can produce.

The multidisciplinary team may include a:

  • Neurotologist.
  • Neurosurgeon.
  • Head and neck surgeon.
  • Audiologist.
  • Physical therapist.
  • Interventional radiologist.
  • Radiation oncologist.
  • Medical oncologist.

Sometimes if the tumor is small and not growing, observation may be recommended. If the tumor is growing or large, the team will recommend treatment. The options are microsurgery or stereotactic radiosurgery.

The aims of microsurgery are to reduce tumor growth, minimize symptoms, and preserve long-term function. The surgical approach may be:

  • Through the ear canal.
  • Through the mastoid (bony lump behind the ear), called the transmastoid.
  • Through a skin crease in the neck.

A larger surgery may be needed when the tumor spreads inside the cranial cavity or into the neck.

Stereotactic radiosurgery is an excellent option for some tumors. UPMC offers both LINAC radiotherapy and Gamma Knife® radiosurgery. Stereotactic radiosurgery is painless and uses hundreds of highly focused radiation beams to target tumors and lesions in the brain, with no incisions.

Transmastoid surgical approach

At UPMC, the preferred surgical approach for glomus tumors that grow outside of the middle ear is the transmastoid approach (behind the ear). Your surgical team of experts will include a neurotologist and a neurosurgeon if the tumor spreads into the cranial cavity.

The approach can be tailored to remove part or all of the tumor, depending on nerve function and goals. An angiogram with embolization (blockage of tumor blood flow) often is performed before surgery to reduce bleeding during surgery. UPMC's approach is a surgical technique that minimizes risk to the facial nerve and other nerves that provide voice and swallowing.

Transcervical surgical approach

When glomus tumors are based in the neck, a head and neck surgeon or neurosurgeon will access the tumor via a neck dissection. The goal will be to preserve the nerve function that assists with voice and swallowing.

Stereotactic radiosurgery for glomus tumors

Radiosurgery is a painless technique that requires no incision. Hundreds of highly focused radiation beams target tumors and lesions in the brain.

Radiosurgery aims to prevent further growth of the glomus tumor and may maintain current neurological and hearing function.

Radiosurgery may be the first treatment option in cases where the tumor is not causing mass effect. It may also be a treatment after surgeons remove part of a glomus tumor.

UPMC leads the nation in Gamma Knife techniques with experts using them to treat more than 18,000 people.

Glomus Tumor Research

UPMC is a world leader in glomus tumor research. Researchers are looking at the mechanisms for why they occur, potential novel treatments in the future, and developing advanced surgical techniques.

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