Paragangliomas (glomus tumors)

Head and Neck Cancer Introduction Page
oral cavity
oropharynx
(soft palate, tonsil, base of tongue)
larynx (voice box)
hypopharynx (pyriform sinus, posterior phanryngeal wall, post-cricoid region)
nasal cavity/paranasal sinuses
nasopharynx
salivary glands
paragangliomas (glomus tumors)
thyroid


Paragangliomas (glomus tumors)

Background
Signs and Symptoms
Clinical Evaluation
Treatment
Follow-Up

Background


Illustration courtesy of Lippincott Williams & Wilkins © 2004

Paragangliomas, sometimes called glomus tumors, are uncommon growths that are closely associated with certain nerves and blood vessels of the head and neck. They typically grow very slowly and have a large amount of blood flowing through them. The most common paraganglioma develops on the main artery of the neck, the carotid artery. They may also develop on vessels and nerves that are high in the neck, in the area of the undersurface of the skull. They may even extend into the skull, adjacent to the brain.

A family history can be a risk factor for developing these tumors, and there may occasionally be more than one of these tumors present in the head and neck. Paragangliomas are usually benign tumors, but they are malignant in approximately 10 percent of cases.

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Signs and Symptoms

Patients most often first see their doctor with a slowly growing, painless but throbbing neck mass. Sometimes there may be decreased hearing ability on one side with an associated throbbing noise (called tinnitus). Hoarseness of the voice, difficulty swallowing or problems with tongue motion may also occur.

Clinical Evaluation

Evaluation starts with a thorough history and physical examination performed by a physician. This will determine the size and location of the tumor and possible weakness of any nerves involved by a paraganglioma. An ear examination is included in this evaluation, because it may show tumor behind the eardrum. Both CT scans (which use X-rays to create a two-dimensional image of the area), and MRI scans (which use a magnetic field and pulses of radio wave energy to visualize the detailed anatomy), help to confirm the diagnosis. These scans also help define the extent of the tumor and identify any other paragangliomas growing in the neck. Frequently, angiography (a study showing the blood vessels of the neck) is performed to reveal the blood supply to these tumors and also to define the circulation to the brain. Most often, a biopsy of the tumor is not performed prior to treatment, because it may be lead to unacceptable bleeding.

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Treatment

The treatment of paragangliomas depends on the size and location of the tumor, the age of the patient, the presence of any nerve weakness, and evaluation of additional medical problems that the patient may have. Any tumors undergoing surgery usually require angiography (see Clinical Evaluation). The blocking of the blood supply to the tumor (known as embolization) is performed at that time. This facilitates the surgical procedure. If possible, tumors of the carotid artery (carotid body tumors) and small tumors of the middle ear (glomus tympanicum) are treated with surgery.

Surgery is also recommended for other paragangliomas (e.g., glomus jugulare, glomus vagale) in which multiple nerve weaknesses exist. However, radiation therapy is used if all the nerves are functioning well. Larger tumors with extensive involvement of the skull base and/or brain are treated with radiation therapy, which is also suggested for older patients with medical problems. Radiation therapy is also the treatment of choice in patients with more than one paraganglioma.

Click Here for more information about radiation oncology treatments for head and neck cancer.

Follow-Up

Periodic physical examination of the head and neck is necessary to monitor the patient after treatment. CT or MRI scans may also be necessary, particularly if a patient has been treated with radiation therapy.

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