Paragangliomas

Paragangliomas are uncommon neuroendocrine tumors that form in certain nerve cells (chromaffin cells) along major blood vessels and nerve pathways. When stimulated by the sympathetic nervous system in response to stress, chromaffin cells release “flight-or-fight” hormones (catecholamines) into the bloodstream.

Because paragangliomas are relatively rare, most physicians see few (if any) patients with this complex diagnosis. The highly specialized expertise required for diagnosing and treating head and neck paragangliomas can be found in Tampa General Hospital’s Neuroscience Institute, where some of the world’s foremost neurosurgeons practice in our Skull Base Surgery Center. For 2023-24, U.S. News & World Report has designated TGH as a “high performer” in Neurology and Neurosurgery.

What Can Cause a Paraganglioma?

The majority of paragangliomas have no known cause. A small percentage of tumors are linked to an inherited gene mutation or an underlying neuroendocrine condition, such as:

• Neurofibromatosis type 1 (NF1)
• Multiple endocrine neoplasia 2 syndrome, types A and B (MEN2A and MEN2B)
• von Hippel-Lindau disease

What Are the Symptoms of a Paraganglioma?

Some paragangliomas do not release hormones or cause symptoms. Tumors that form in the carotid body or vagus nerve may appear as a painless mass in the neck. Depending on its precise location, a paraganglioma may also cause bouts of:

• Ear pain
• Hearing loss
• Tinnitus
• Weakness in the face, tongue or shoulder
• Vocal hoarseness
• Difficulty swallowing
• A drooping eyelid

If a paraganglioma releases catecholamines, it can also cause:

• High blood pressure (hypertension)
• A rapid or irregular heartbeat
• Shaking
• Sweating
• Headaches
• Anxiety

How Is a Paraganglioma Diagnosed?

Some paragangliomas are detected in imaging scans performed for an unrelated reason. A paraganglioma may also be suspected if hypertension does not respond to standard treatment. To confirm the diagnosis, a physician will typically:

• Perform a physical examination, medical history review and blood pressure check
• Order a 24-hour urine test to measure the levels of hormones in the urine
• Order a blood catecholamine test to measure the levels of hormones in the blood
• Order a positron emission tomography (PET) scan to check for tumors and areas of metabolically active cells
• Order computed tomography (CT) scans with and without contrast to determine the location and size of a tumor in the neck
• Order magnetic resonance imaging (MRI) scans with and without contrast to further evaluate a tumor

What Are the Treatment Options for a Paraganglioma?

Paraganglioma treatment can vary based on the size and location of the tumor, whether it is cancerous and whether it is causing symptoms. Some options include:

• Monitoring the tumor with periodic exams and imaging scans
• Medications to regulate blood pressure and heart rate
• Skull base neurosurgery to remove the tumor
• Stereotactic radiation therapy to slow or stop tumor growth

Choose TGH for World-Class Care  

The neurosurgeons and otolaryngologists at TGH are internationally recognized for their skill in performing skull base surgery. We diagnose and treat all types of brain conditions, including benign and cancerous paragangliomas. If you would like to discuss your symptoms or explore your treatment options with a specialist at TGH, contact us at (800) 822-3627 to request an appointment.