AUA Summit - What is Pheochromocytoma (Adrenal Medulla Tumor)?

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What is Pheochromocytoma (Adrenal Medulla Tumor)?

Pheochromocytoma is a tumor found in the adrenal medulla (the inner part of the adrenal gland). The adrenal medulla makes the hormones adrenaline (epinephrine), noradrenaline (norepinephrine) and dopamine. If a tumor forms in this area, it can cause too much of these hormones to be made. This can be very harmful, as it can cause very high blood pressure.

Pheochromocytoma in the adrenal gland is not often cancerous, but it may be linked to cancer in other glands, like the thyroid or kidney.

It is of great value to diagnose this problem early. Finding and treating it could cure unsafe blood pressure. If it is not treated, it can lead to a heart attack, stroke and cause the arteries to work poorly.

What Happens Under Normal Conditions?

The adrenal glands are found above each kidney. They are triangle-shaped, and measure about half an inch in height and three inches in length. Each adrenal gland has two layers.

  • The adrenal medulla (inner part) makes epinephrine (also called adrenaline) and other similar hormones.
  • The adrenal cortex (outer part) makes steroid hormones (such as cortisol and aldosterone).

The adrenal glands control many processes in the body. Their job is to keep the body in balance by making various hormones that are vital for keeping good health.

These hormones do many important things. For example, they help regulate fluid and salt levels in the body that affect blood volume and blood pressure. They also help the body react to stress and change. They cause a faster heart rate and boost other systems that help you to react quickly with a burst of energy when needed. Problems in the cortex or the medulla can then result in high blood pressure.

Symptoms

Most patients with pheochromocytoma have high blood pressure. Many patients have three main symptoms: headache, excess sweating and episodes of a hard, fast heartbeat (palpitations).

You should talk with your health care team about whether you may have pheochromocytoma if:

  • You have high blood pressure that is hard to control
  • You are taking more than four drugs for blood pressure
  • You have high blood pressure before age 35 or after age 60
  • You have symptoms such as:
    • Bad headaches
    • Excessive sweating
    • Racing heartbeat
    • Anxiety and worry
    • Nervous shaking
    • Pain in the lower chest or upper belly area
    • Feeling overheated
    • Vision problems
    • Seizures

Spikes in blood pressure should be talked about with your health care team. They can be short term or long lasting. Sudden attacks often last less than one hour. They can happen many times a week, or only a few times a year. Women experience these spikes more often than men. They can be brought on by physical, emotional or drug related issues.

Causes

Pheochromocytoma is rare. It occurs as often in men as women, causing less than 1 out of 100 of all high blood pressure cases. It can develop at any age, but is more common in people 40 to 60 years old. It is not known what causes most cases. Very few cases are linked to family history. Hereditary cases are seen more often in young patients. If pheochromocytoma is diagnosed before age 50, genetic screening is helpful to find out whether it is hereditary.

Diagnosis

If your health care provider suspects pheochromocytoma, some standard tests that are done are:

  • A blood test to check for adrenaline hormones (epinephrine, norepinephrine, dopamine) or byproducts of adrenaline called metanephrine and normetanephrine
  • A 24-Hour urine collection test to measure levels of adrenaline and adrenaline byproduct hormones

Next, a CT scan or MRI scan will help your doctor find an adrenal mass and find its location (left or right abdomen), size and presence, or absence of invasive features.

Treatment

Controlling high blood pressure with special types of drugs is the first step. Once your blood pressure is stable, surgery is used to treat the problem.

The diseased adrenal gland could be removed with laparoscopic, robotic or open surgery. Most often, the whole adrenal gland is removed. For a smaller tumor, especially in hereditary cases, only the diseased part of the gland is removed. The healthy part of the gland remains.

Other symptoms may also be controlled for a short time using medications before the tumor is removed.

Laparoscopy and robotic surgery are less invasive procedures. Anesthesia is used. The hospital stay is one to two days if blood pressure is well controlled. Most people can return to normal activity that does not require heavy lifting after about two weeks.

Open surgery is more involved. It requires two to four days in the hospital and at least a four week recovery period.

It is of great value to know that adrenal surgery is complex, and may involve harmful changes in blood pressure. It helps to choose a surgeon who has a large amount of experience with surgery in the area around the kidneys and adrenal glands. Talk with a few surgeons before you choose one to work with.

After Treatment

Most patients feel much better and their blood pressure improves greatly after treatment. A few patients still have high blood pressure after surgery. Medicine may still be used for blood pressure control in those cases.

Long-term follow-up care is of great value for everyone after treatment.  In some cases, but not often, a pheochromocytoma may be cancerous and may come back. This is possible even 15 years, or more, after surgery. This cancer can’t be seen clearly under the microscopic, so long-term follow-up may be suggested. But, for most patients, surgical removal of the pheochromocytoma is curative, meaning it does not come back.

Without the right treatment, the severe high blood pressure tied to pheochromocytoma can lead to heart attack or stroke. After treatment, the outlook is good for most patients.

Updated October 2024. 


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