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Pheochromocytoma

What Is Pheochromocytoma?

Highlights

  1. Pheochromocytoma (PCC) is a rare tumor that forms in the cells in the middle of an adrenal gland. PPCs are usually noncancerous (benign).
  2. PCCs cause the affected adrenal gland to release hormones that cause high blood pressure. If left untreated, a PCC can harm the body, especially the cardiovascular system.
  3. Surgical removal of a PCC is usually effective in returning blood pressure to a normal level.

Your body has two adrenal glands, one located above each of your kidneys. The adrenals produce hormones that give the organs and tissues in your body instructions on how they should function. These hormones regulate vital bodily functions such as:

  • blood pressure
  • stress response
  • blood sugar
  • immune response
  • metabolism
  • heart rate

Pheochromocytoma (PCC) is a rare tumor that can form in cells in the middle of the adrenal glands. In the case of PCC, a tumor can cause the adrenal glands to make too much of the hormones norepinephrine (noradrenaline) and epinephrine (adrenaline). Together these hormones control heart rate, metabolism, blood pressure, and the body’s stress response.

Increased levels of these hormones can put the body into a stress-response state, causing blood pressure to increase.

Tumors that form on the outside of the adrenal glands are called paragangliomas. Both PCCs and paragangliomas can also impact the adrenal glands’ production of adrenal hormones called catecholamines.

What Are the Causes and Risk Factors of PCC?

Risk Factors

It is believed that the development of a PCC is linked to a reduced oxygen supply (hypoxia). Conditions that may cause hypoxia include severe hypertension, high blood pressure, and congenital heart disease.

A PCC can develop at any age, but is most common in early to middle adulthood, and is believed that it the condition is often genetically inherited.

People who inherit PCC from their parents may also develop associated genetic conditions. These conditions include:

  • Von Hippel-Lindau disease, a condition where cysts and tumors grow in the central nervous system, kidneys, adrenal glands, or other areas of the body
  • Neurofibromatosis type 1, the development of tumors on the skin and optic nerves
  • Multiple endocrine neoplasia type 2 (MEN2), a form of thyroid cancer that develops in conjunction with PCC

What Are the Symptoms of PCC?

Symptoms

A sudden large increase in adrenal hormones is called an adrenergic crisis (AC). AC causes severe high blood pressure (hypertension) and a rapid heart rate (tachycardia).

Common symptoms of PCC are:

  • episodic and sudden onset of severe headaches
  • sweating
  • abdominal pain
  • high blood pressure that may be resistant to conventional medications
  • rapid heart rate
  • irritability and anxiety

Common causes of AC are:

  • drugs, such as corticosteroids, chemotherapy agents, etc.
  • surgical anesthesia
  • emotional stress

Diagnosis of PCC

Diagnosis

Diagnosis of PCC has improved with modern technology. However, PCC is still difficult to diagnose. There are several different tests your doctor may use to diagnose PSS. These include:

  • MRI
  • PET imaging
  • laboratory tests to assess hormone levels
  • blood plasma test for catecholamine and metanephrine levels
  • urine metanephrines test for catecholamine and metanephrine levels

Treatment of PCC

Treatment

Surgical removal of the tumor is usually the main method of treatment. However, because of the importance of the adrenal glands, this surgery can be very difficult.

If a PCC is found to be cancerous, cancer treatments such as chemotherapy and radiation therapy may be required after surgery.

After surgery you may have short-term problems with adrenal hormone regulation. Your doctor may prescribe steroids to substitute your natural hormones until the adrenal glands restart normal function.

Complications of PCC

Complications

Without treatment, those with a PCC are at a higher risk for the following conditions:

  • high blood pressure crisis
  • irregular heart beat
  • heart attack
  • multiple organs of the body begin to fail

However, as with any surgery, treating PCC surgical may involve complications. The surgery affects powerful hormones in the body. During an operation, some of the conditions that may develop include:

  • high blood pressure crisis
  • low blood pressure crisis
  • irregular heart beat

In rare cases, PCC may be cancerous. In these cases surgery is followed with radiation therapy or chemotherapy.

What Is the Long-Term Outlook?

Outlook

The outlook for a person with a PCC depends on if the tumor is cancerous. People who had a PCC that wasn’t cancerous had a five-year survival rate of 96 percent. The survival rate was 44 percent for people who had a cancerous tumor.

Early diagnosis is not always enough to ensure successful treatment. Because of the difficulty of the surgery, seek out a surgeon who is highly skilled and able to handle possible complications.

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