Pheochromocytoma and Paraganglioma

Pheochromocytoma and paraganglioma are rare neuroendocrine tumors that can produce excess hormones that affect blood pressure, heart rate, and other vital functions.

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Pheochromocytoma and paraganglioma are rare neuroendocrine tumors.

Pheochromocytoma refers to a tumor that forms in the adrenal glands, which are at the top of the kidneys. Paraganglioma is a tumor that often forms near the carotid artery in the head and neck. It can also form on either side of the spine.

Paragangliomas and pheochromocytomas can make hormones called catecholamines, which are related to adrenaline. These hormones help control heart rate, blood pressure, blood sugar, and stress. When an excess of these hormones is released through the bloodstream, it can cause a variety of symptoms.

Because of their similarities, pheochromocytoma and paraganglioma are sometimes referred to together as pheo para.

If you have any of these symptoms, that doesn’t mean you have pheochromocytoma or paraganglioma. But if you’re concerned about the frequency of these kinds of symptoms, you should discuss them with your doctor.

Some pheochromocytoma and paraganglioma tumors don’t cause any noticeable symptoms.

Some inherited disorders and changes in genes increase your risk of pheochromocytoma or paraganglioma.

The cause of pheochromocytoma and paraganglioma is not known. But some things are known to increase your chances of getting them. These are called risk factors.

Leaders in advanced, comprehensive pheo para care

Pheochromocytoma and paraganglioma are complex conditions that demand expert, coordinated care. At University of Iowa Health Care Holden Comprehensive Cancer Center, our dedicated multidisciplinary pheo para team meets regularly to collaborate on patient care plans, treatment, research, and more. Our team includes adult and pediatric specialists in gastroenterology, cardiology, neurology, endocrinology, oncology, radiation oncology, nuclear medicine, and genetic counseling.

We're also nationally and internationally recognized for our leadership in pheo para care and research. Our distinctions include:

Fifth center in the U.S. to be named a Center of Excellence by the Pheo Para Alliance

First U.S. cancer center to earn Center of Excellence designation from the European Neuroendocrine Tumor Society

First in the nation to receive a Specialized Program of Research Excellence grant from the National Cancer Institute for neuroendocrine tumor research

Holden is one of only five centers in the United States that use MR-Linac, a device that combines state-of-the-art MRI imaging and radiation targeting. This technology is especially useful for patients with paragangliomas and pheochromocytomas near the brain or spinal cord, or in the liver, pancreas, or adrenal glands. MR-Linac more accurately targets the tumor and saves more healthy tissue.

We are also one of 13 centers in the U.S. performing AZEDRA MIBG, radioactive therapy that involves injecting a drug that can kill tumor cells. This is a good option for those with tumors that can’t be removed by surgery.

Risk factors for pheochromocytoma and paraganglioma

The cause of pheochromocytoma and paraganglioma is not known. But some things are known to increase your chances of getting these diseases, such as some inherited disorders and genetic abnormalities. This includes:

Multiple endocrine neoplasia 2 syndrome, types A and B (MEN2A and MEN2B)
von Hippel-Lindau (VHL) syndrome
Neurofibromatosis type 1 (NF1)
Hereditary paraganglioma syndrome
Carney-Stratakis dyad (paraganglioma and gastrointestinal stromal tumor, also called GIST)
Carney triad (paraganglioma, GIST, and pulmonary chondroma)

Genetic testing and counseling is available for adult and pediatric patients. Our genetic counselors provide diagnosis and work closely with our specialized care teams to help develop screening and care plans for patients who are affected by, or at risk for, hereditary paraganglioma syndrome.

Signs and symptoms of pheochromocytoma and paraganglioma

Not all pheochromocytoma and paraganglioma tumors have noticeable symptoms.

However, those with pheo para often report symptoms that include:

High blood pressure that is difficult to control

Headaches

Anxiety attacks

Fast or irregular heartbeat

Excessive sweating

Shakiness

A pale appearance in the face

Sometimes the symptoms of pheochromocytoma and paraganglioma can be triggered by events such as:

Physical activity

Stress

Childbirth

Eating certain foods that contain tyramine, such as red wine or chocolate

Having any of these symptoms doesn’t mean you have pheochromocytoma or paraganglioma. If you’re concerned about the frequency of these symptoms, talk to your provider.

How pheochromocytoma and paraganglioma is diagnosed

Twenty-four-hour urine test. This involves collecting samples of your urine for a 24-hour period to measure the amount of adrenal hormones (catecholamines) in your urine. Higher amounts of adrenal hormones could indicate pheochromocytoma.

Blood catecholamine tests. Much like the urine test, your provider may take blood samples to measure the amount of adrenal hormones in your blood. Higher amounts of adrenal hormones could indicate pheochromocytoma.

Computed tomography (CT). A CT scan helps your providers see where the tumor is located.

Magnetic resonance imaging (MRI). An MRI scan can produce more detailed images of the inside of your body, allowing your provider to locate tumors.

Positron emission tomography (PET). A PET scan can also help produce detailed images of your organ and tissues to locate tumors and see if the cancer has spread to other parts of the body.

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That’s why getting a second opinion matters. Our cancer experts can confirm your diagnosis and help you understand all your treatment options.

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Treatments for pheochromocytoma and paraganglioma

Your pheo para care team will work together to create a personalized treatment plan to best treat your cancer. Your treatment plan could include:

Medication. Your provider may recommend medications to regulate blood pressure (alpha-blockers) or heart rate (beta-blockers), or medications to block the effects of excess hormones released by your adrenal glands.

Surgery. It may be necessary to remove one or both adrenal glands as well as any affected tissue or lymph nodes.

Radiation. May be paired with surgery to help kill cancer cells and shrink tumors using radiation. Both external radiation and radiation targeting are used to treat pheo para, including MR Linac, AZEDRA MIBG, and peptide receptor radionuclide therapy.

Theranostics. Linking radiation to molecular targets, this therapy allows specialists to selectively target cancer cells with radiation without damaging the healthy tissue that surrounds it.

Chemotherapy. May be paired with surgery to help kill cancer cells and shrink tumors using anticancer drugs.

Ablation therapy. Destroys cancer cells using either heat caused by radio waves or by freezing the tissue.

Clinical trials. Clinical trials give you the opportunity to receive the latest, most advanced treatment options, such as drugs that are not yet on the market and other promising treatments that could increase your chances for recovery and improve your quality of life.