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) and noradrenaline (norepinephrine). If a tumor forms in this area, it can cause too much of these hormones to be made. This can be very dangerous, as it causes very high blood pressure. Pheochromocytoma in the adrenal gland is rarely cancerous, but it may be linked to cancer in other glands, like the thyroid.

It is important 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?

Anatomy of the Adrenal Gland
Anatomy of the Adrenal Gland
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The adrenal glands are found above each kidney. They are triangle-shaped, and measure about half an inch in height and 3 inches in length. Each adrenal gland has 2 layers.

  • The adrenal medulla (inner part) makes epinephrine (also called adrenaline).
  • The adrenal cortex (outer part) makes steroid hormones (such as cortisone 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 critical for maintaining good health.

Adrenal glands

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, then, can result in high blood pressure.



What are the Symptoms of Pheochromocytoma?

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

You should see your health care provider if:

  • You have high blood pressure that is difficult to control
  • You are taking more than 4 drugs for blood pressure
  • You have high blood pressure before age 35 or after age 60
  • You have signs such as:
    • Bad headaches
    • Excessive sweating
    • Racing heartbeat
    • Paleness
    • Anxiety and worry
    • Nervous shaking
    • Pain in the lower chest or upper belly area
    • Upset stomach
    • Weight loss
    • Feeling overheated
    • Fatigue
    • Vision problems
    • Seizures

Spikes in blood pressure should be discussed with your health care provider. They can be short term or long lasting. Sudden attacks often last less than 1 hour. They can happen several 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. Some things that can cause high blood pressure in this condition are:

  • Bending over
  • Abdominal pressure
  • Fear
  • Tobacco use
  • Histamines and glucagons
  • Foods rich in tyramine (like beer, wine and cheese)


What are the Causes of Pheochromocytoma?

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. Researchers don’t know 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.



How is Pheochromocytoma Diagnosed?

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

  • Biochemical analysis to test plasma metanephrines (a byproducts of hormones in the blood)
  • 24-Hour urine tests to measure hormone levels.

Next, CT scans or MRI testing will help your doctor find a mass, its location, and the extent of the problem. Pheochromocytoma happens in less than 1 out of 100 people with high blood pressure and more than 95 out of 100 of those cases are not cancer.



How is Pheochromocytoma Treated?

Controlling high blood pressure with drugs is the first step. Once your blood pressure is stable, surgery may be 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.

Before surgery, drugs are often given to block the effects of hormones and help blood pressure. Other symptoms may also be controlled before the tumor is removed.

Laparoscopy and robotic surgery are less invasive procedures. Anesthesia is used. The hospital stay is 1 to 2 days if blood pressure is well controlled. Most people can return to normal activity after about 2 weeks.

Open surgery, or traditional laparotomy, is more involved. It requires 5 to 7 days in the hospital and a 4-week recovery period.

It’s important to know that adrenal surgery is complex. It helps to choose a surgeon who has significant experience with surgery in the area around the kidneys and adrenal glands. Talk with a few surgeons before you choose one to work with.



What Can Be Expected after Treatment for Pheochromocytoma?

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

Long-term follow-up care is important for everyone after treatment. In very rare cases, pheochromocytomas is cancerous and may come back. This is possible even 15 years, or more, after surgery. This rare cancer can’t be found under the microscopic, so long-term follow-up is required.

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.