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Pheochromocytoma

A pheochromocytoma (fee-o-kroe-moe-sy-TOE-muh) is a rare, usually noncancerous (benign) tumor that develops in cells in the center of an adrenal gland. You have two adrenal glands, one above each kidney. Your adrenal glands produce hormones that give instructions to virtually every organ and tissue in your body.

If you have a pheochromocytoma, an adrenal gland releases hormones that cause persistent or episodic high blood pressure. If left untreated, a pheochromocytoma can result in severe or life-threatening damage to other body systems, especially the cardiovascular system.

Most people with a pheochromocytoma are between the ages of 20 and 50, but the tumor can develop at any age. Surgical treatment to remove a pheochromocytoma usually returns blood pressure to normal.

Symptoms Causes Risk factors Complications

Signs and symptoms of pheochromocytomas often include:

  • High blood pressure
  • Rapid or forceful heartbeat
  • Profound sweating
  • Severe headache
  • Tremors
  • Paleness in the face
  • Shortness of breath

Less common signs or symptoms may include:

  • Anxiety or sense of doom
  • Abdominal pain
  • Constipation
  • Weight loss

These signs and symptoms often occur in brief spells of 15 to 20 minutes. Spells can happen several times a day or less often. Your blood pressure may be within the normal range or remain elevated between spells.

Triggers of symptomatic spells

Spells may occur spontaneously or may be triggered by such factors as:

  • Physical exertion
  • Anxiety or stress
  • Changes in body position
  • Bowel movement
  • Labor and delivery

Foods high in tyramine, a substance that affects blood pressure, also can trigger a spell. Tyramine is common in foods that are fermented, aged, pickled, cured, overripe or spoiled. These foods may include:

  • Some cheeses
  • Some beers and wines
  • Dried or smoked meats
  • Avocados, bananas and fava beans
  • Pickled fish
  • Sauerkraut or kimchi

Certain medications that can trigger a symptomatic spell include:

  • Decongestants
  • Monoamine oxidase inhibitors (MAOIs), such as phenelzine (Nardil), tranylcypromine (Parnate) and isocarboxazid (Marplan)
  • Stimulants, such as amphetamines or cocaine

When to see a doctor

The signs and symptoms of pheochromocytoma can be caused by a number of different conditions. Therefore, it's important to get a prompt diagnosis.

Although high blood pressure is a primary sign of a pheochromocytoma, most people with high blood pressure don't have an adrenal tumor. Talk to your doctor if any of the following factors are relevant to you:

  • Difficulty controlling high blood pressure with current treatment plan
  • A family history of pheochromocytoma
  • A family history of a related genetic disorder: multiple endocrine neoplasia, type II (MEN II); von Hippel-Lindau disease; familial paraganglioma or neurofibromatosis 1 (NF1)

Researchers don't know exactly what causes the development of a pheochromocytoma. They do understand that the tumor develops in specialized cells, called chromaffin cells, located in the center of an adrenal gland. These cells produce and release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine).

The role of hormones

Adrenaline and noradrenaline are hormones that normally function as the trigger for your body's fight-or-flight response to a perceived threat. The hormones prompt higher blood pressure, a faster heart rate and a boost in other body systems that enable you to react quickly with a burst of energy. A pheochromocytoma results in the irregular and excessive release of these hormones.

Related tumors

While most chromaffin cells reside in the adrenal glands, small clusters of these cells are also located in the heart, head, neck, bladder, back wall of the abdomen and along the spine. Tumors in these chromaffin cells, called paragangliomas, may result in the same effects on the body.

People with rare inherited disorders have an increased risk of developing a pheochromocytoma or paraganglioma, and tumors associated with these disorders are more likely to be cancerous. These genetic conditions include the following:

  • Multiple endocrine neoplasia, type II (MEN II) is a disorder resulting in tumors in more than one part of the body's hormone-producing (endocrine) system. The locations of other tumors associated with MEN II include the thyroid, parathyroid, lips, tongue and gastrointestinal tract.
  • Von Hippel-Lindau disease can result in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
  • Neurofibromatosis 1 (NF1) results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
  • Hereditary paraganglioma syndromes are inherited disorders that result in either pheochromocytomas or paragangliomas.

High blood pressure can damage multiple organs, particularly tissues of the cardiovascular system, brain and kidneys. If left untreated, high blood pressure associated with pheochromocytomas may result in a number of critical conditions, including:

  • Heart disease
  • Stroke
  • Kidney failure
  • Acute respiratory distress
  • Damage to nerves of the eye

Cancerous (malignant) tumors

Rarely, a pheochromocytoma is cancerous (malignant), and the cancerous cells spread to other parts of the body (metastasize). Cancerous cells from a pheochromocytoma or paraganglioma most often migrate to the lymph system, bones, liver or lungs.

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