A pheochromocytoma is a rare, usually noncancerous (benign) tumor of the adrenal gland tissue. Every individual has two adrenal glands, one above each kidney. The adrenal glands produce hormones that give instructions to virtually every organ and tissue in the body. Pheochromocytoma is what occurs when the glands release too much epinephrine and norepinephrine, hormones that control heart rate, metabolism and blood pressure. Most individuals with pheochromocytoma are between the ages of 20 and 50; however, the tumor can develop at any age.
If left untreated, a pheochromocytoma can result in severe or life-threatening damage to other body systems, especially the cardiovascular system. Surgical treatment is recommended in order to remove a pheochromocytoma and return blood pressure levels back to normal.
Signs and symptoms of pheochromocytomas often include:
· High blood pressure
· Chest pain
· Rapid heart rate
· Throbbing headache
· Paleness in the face
· Shortness of breath
Less common signs or symptoms may include the following:
- Abdominal pain
- 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. Factors that can trigger symptomatic spells include:
- Overuse of the muscles during extreme physical workout/activity
- Anxiety or stress
- Changes in body position
- Bowel movement
- Foods high in tyramine (Some cheeses, dried or smoked meat, avocados, bananas, pickled fish, fava beans, others)
- Certain medications (Decongestants, Monoamine oxidase inhibitors (MAOIs), Stimulants, such as amphetamines or cocaine)
Although the exact cause of the development of a pheochromocytoma remains unclear, it is known that a single tumor usually develops in specialized cells (chromaffin cells) located in the center of one or both the adrenal glands. These cells produce and release certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine) that are used to trigger the body’s response to a perceived threat. However, when irregular or excessive release of these hormones takes place, high blood pressure levels and a faster heart rate are caused. This is known as pheochromocytoma.
Factors that increase the risk of developing pheochromocytoma can be seen in individuals with rare inherited disorders and tumors associated with these disorders. These genetic conditions include the following:
· Multiple endocrine neoplasia, type II (MEN II) : a disorder that results in tumors in more than one part of the body's hormone-producing (endocrine) system.
· Von Hippel-Lindau disease: a disease that can results in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
· Neurofibromatosis 1 (NF1): an inherited neurological disorder that results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
· Hereditary paraganglioma syndromes: a group of inherited disorders that result in either pheochromocytomas or paragangliomas.
A pheochromocytoma results in high blood pressure levels in an individual. Having high blood pressure can damage multiple organs, particularly tissues of the cardiovascular system, brain and kidneys. If left untreated, complications that can arise may results in a number of critical medical conditions including:
· Heart disease
· Kidney failure
· Acute respiratory distress
· Nerve damage to the eyes
The primary treatment for a pheochromocytoma is surgery to remove the tumor. Before undergoing surgery, a doctor will prescribe medications to control blood pressure levels and lower the risk of complications during surgery. These medications include:
- Alpha blockers: pharmacological agents that act to prevent noradrenaline from stimulating the muscles in the walls of smaller arteries and veins. Because these blood vessels remain open and relaxed, blood flow improves and blood pressure lessens.
- Beta blockers: a class of drugs that are used to manage and control cardiac arrhythmias, protecting the heart from a second heart attack or hypertension.
- Other medications to lower blood pressure levels
Additional treatment options may include:
- Surgery: Surgical removal of the entire adrenal gland with a pheochromocytoma. The remaining healthy adrenal gland carries out the functions normally performed by two, and blood pressure usually returns to a normal level
Treatments for malignant tumors and metastasized cancer related to a pheochromocytoma may include the following:
· Radionuclide treatment: The use of a radioactive drug called radiopharmaceutical to target and kill cancerous cells.
· Chemotherapy: The use of powerful drugs to kill fast growing cancer cells.
· Targeted cancer therapies: The use of drugs to interfere with specific molecules necessary for tumor growth and progression of cancerous cells.