High blood pressure is the most important symptom, but a fast and pounding pulse, excessive sweating, light-headedness when standing, rapid breathing, severe headaches, and many other symptoms may also occur.
Doctors measure the blood levels of catecholamines or products created when catecholamines are broken down by the body and use imaging tests to try to find the tumor.
Usually, the best treatment is to remove the pheochromocytoma.
(See also Overview of the Adrenal Glands Overview of the Adrenal Glands The body has two adrenal glands, one near the top of each kidney. They are endocrine glands, which secrete hormones into the bloodstream. Each adrenal gland has two parts. Medulla: The inner... read more and the figure .)
Most pheochromocytomas grow within the outer layer (cortex) of the adrenal glands. About 10% grow in chromaffin cells outside the adrenal glands. Less than 10% of pheochromocytomas that grow within the adrenal glands are cancerous, but this percentage is higher for those outside the adrenal glands. Pheochromocytomas may occur in men or women at any age, but they are most common in people between the ages of 20 and 40.
Some people who develop pheochromocytomas have a rare inherited condition called multiple endocrine neoplasia Multiple Endocrine Neoplasia Syndromes (MEN) Multiple endocrine neoplasia syndromes are rare, inherited disorders in which several endocrine glands develop noncancerous (benign) or cancerous (malignant) tumors or grow excessively without... read more that makes them prone to tumors in the thyroid, parathyroid, and adrenal glands. Pheochromocytomas may also develop in people who have von Hippel–Lindau disease Von Hippel–Lindau Disease (VHL) Von Hippel-Lindau disease is a rare hereditary disorder that causes noncancerous and/or cancerous tumors to develop in several organs. Von Hippel-Lindau disease is caused by mutations in a gene... read more and in those who have neurofibromatosis Neurofibromatosis Neurofibromatosis is a group of genetic disorders in which many soft, fleshy growths of nerve tissue (neurofibromas) form under the skin and in other parts of the body, and flat spots that are... read more (von Recklinghausen disease) or a number of other genetic diseases. It is likely that nearly 50% of people who have pheochromocytomas have a genetic or familial disease such as these.
Symptoms of Pheochromocytoma
Pheochromocytomas may be quite small. However, even a small pheochromocytoma can produce large amounts of potent catecholamines. Catecholamines are hormones such as adrenaline (epinephrine), norepinephrine, and dopamine, which tend to greatly increase blood pressure and heart rate and cause other symptoms usually associated with life-threatening situations.
The most prominent sign of a pheochromocytoma is high blood pressure High Blood Pressure High blood pressure (hypertension) is persistently high pressure in the arteries. Often no cause for high blood pressure can be identified, but sometimes it occurs as a result of an underlying... read more , which may be very severe. However, only about 1 in 1,000 people with high blood pressure has a pheochromocytoma. Symptoms include
A fast and pounding heart rate
Light-headedness when standing
Cold and clammy skin
Chest and stomach pain
Nausea and vomiting
An odd sense of impending doom
When these symptoms appear suddenly and forcefully, they can feel like a panic attack Panic Attacks and Panic Disorder A panic attack is a brief period of extreme distress, anxiety, or fear that begins suddenly and is accompanied by physical and/or emotional symptoms. Panic disorder involves recurrent panic... read more .
In half of affected people, symptoms come and go, sometimes triggered by pressure on the tumor, massage, drugs (especially anesthetics and beta-blocking drugs), emotional trauma, and, on rare occasions, the simple act of urination. However, many people may have these symptoms as manifestations of an anxiety state, not a glandular disorder.
Diagnosis of Pheochromocytoma
Blood and urine tests
Computed tomography or magnetic resonance imaging
Doctors may not suspect a pheochromocytoma, because almost half of the people have no symptoms other than persistent high blood pressure. However, when high blood pressure occurs in a young person, comes and goes, or accompanies other symptoms of pheochromocytoma, doctors may request certain laboratory tests. For example, the level of certain catecholamines or products created when these catecholamines are broken down may be measured in blood or urine samples. Because of high blood pressure and other symptoms, doctors may prescribe a beta-blocker before knowing that the cause is a pheochromocytoma. Beta-blockers can make high blood pressure worse in people with pheochromocytoma. This paradoxical reaction often makes the diagnosis of pheochromocytoma clear.
If the level of catecholamines is high, computed tomography (CT), magnetic resonance imaging (MRI), or another imaging test can help locate the pheochromocytoma. A test using injected radioactive chemicals that tend to accumulate in pheochromocytomas can also be useful. A scan is then done to see where the radioactive chemicals are.
Once pheochromocytoma has been confirmed, doctors typically do genetic tests on the person.
Treatment of Pheochromocytoma
Surgery to remove the tumor
Drugs to control blood pressure
Usually the best treatment is to remove the pheochromocytoma. Surgery is often delayed, however, until doctors can bring the tumor’s secretion of catecholamines under control with drugs, because having high levels of catecholamines can be dangerous during surgery. Phenoxybenzamine or a similar drug is generally given to stop hormone action. Once this step is accomplished, a beta-blocker can safely be given to further control symptoms.
If the pheochromocytoma is cancerous and has spread, chemotherapy rarely cures the tumor. Newer chemotherapy drugs such as temozolomide and sunitinib seem to help slow the tumor’s growth. Treatment with radioisotopes such as metaiodobenzylguanidine (MIBG) or radioactive octreotide that target the tumor tissue can also be highly effective. The dangerous effects of the excess catecholamines secreted by the tumor can almost always be blocked by continuing to take phenoxybenzamine or a similar drug, such as doxazosin, and beta-blockers.
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