What is pheochromocytoma?
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.
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
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
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
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