Pheochromocytoma is a rare, usually noncancerous (benign) tumor that develops in the adrenal gland. 10% of patients have cancerous tumors. You have two adrenal glands, one above each kidney. Usually, a pheochromocytoma develops in only one adrenal gland. However in both cases, tumors can develop from nerve tissue over the adrenal gland.
- What are the Symptoms of Pheochromocytoma?
- What are the Causes of Pheochromocytoma?
- What is the Role of Hormones?
- What are Tumors Related to Pheochromocytoma?
- What are the Risk Factors?
- What are the Complications of Pheochromocytoma?
- Why is the Diagnosis of Pheochromocytoma Important?
- What is the Treatment of Pheochromocytoma?
If you have a pheochromocytoma, the tumor releases hormones that can cause high blood pressure, headaches, sweating, and panic attack symptoms. If pheochromocytoma is not treated, serious or life-threatening damage to other body systems can occur.
Most pheochromocytomas are discovered in people between the ages of 20 and 50. However, the tumor can develop at any age. Pheochromocytoma surgery usually returns blood pressure to normal.
What are the Symptoms of Pheochromocytoma?
Signs and symptoms of pheochromocytomas usually include:
- high blood pressure,
- heavy sweating,
- rapid heartbeat,
- paleness of the face,
- shortness of breath,
- panic attack type symptoms.
Less common signs or symptoms may include:
- anxiety or a sense of doom,
- oweight loss.
The symptoms listed above may be continuous or occasionally appear or intensify. Certain activities or conditions can make symptoms worse, such as:
- physical effort,
- anxiety or stress,
- changes in body position,
- labor and delivery,
- surgery and anesthesia.
Foods high in tyramine, a substance that affects blood pressure, can also worsen symptoms. Tyramine is common in fermented, soaked, pickled, cured, ripened foods. These foods include:
- beer and wine,
- dried or smoked meats.
Some medications that can make symptoms worse include:
Monoamine oxidase inhibitors (MAOIs) such as phenelzine (Nardil), tranylcypromine (Parnate) and isocarboxazid (Marplan), some rhythm regulators, blood pressure drugs (beta-blocking drugs such as metoprolol, propronolol) may increase the complaints.
Stimulants such as amphetamine or cocaine.
What are the Causes of Pheochromocytoma?
Researchers do not know exactly what causes pheochromocytoma. The tumor develops in special cells called chromaffin cells located in the center of the adrenal gland. These cells secrete certain hormones, primarily adrenaline (epinephrine) and noradrenaline (norepinephrine), which help control many body functions such as heart rate, blood pressure, and blood sugar.
What is the Role of Hormones?
Adrenaline and noradrenaline trigger your body's fight-or-flight response to a perceived threat. Hormones cause your blood pressure to rise and your heart to beat faster. They prepare other body systems that allow you to react quickly. A pheochromocytoma causes more of these hormones to be released, even when you are not in a threatening situation.
What are Tumors Related to Pheochromocytoma?
While most chromaffin cells are found in the adrenal glands, small clusters of these cells are also found throughout the heart, head, neck, bladder, posterior abdominal wall, and spine. Pheochromocytomas that develop in these areas are called Paraganglioma, this is a chromaffin cell tumor in the tumor, it can cause the same effects on the body.
What are the Risk Factors?
People with certain rare inherited disorders have an increased risk of pheochromocytoma or paraganglioma. Tumors associated with these disorders are more likely to be cancerous. These genetic conditions include:
Multiple endocrine neoplasia, type 2 (MEN 2), is a disease that causes tumors in more than one part of the body's hormone-producing endocrine system. Other tumors associated with this condition can be seen in the thyroid, parathyroid, lips, tongue and gastrointestinal tract.
Von Hippel-Lindau diseasecan cause tumors in various locations including the central nervous system, endocrine system, pancreas, and kidneys.
Neurofibromatosis 1(NF1) can cause multiple tumors of the skin (neurofibromas), pigmented skin spots and optic nerve tumors.
Hereditary paraganglioma syndromes are inherited disorders that result in pheochromocytomas or paragangliomas.
What are the Complications of Pheochromocytoma?
High blood pressure can damage multiple organs, especially the tissues of the cardiovascular system, the brain, and the kidneys. This damage can cause a number of critical conditions, including:
- heart diseases (rhythm disorder, palpitations, heart failure),
- stroke (brain hemorrhage),
- renal insufficiency,
- problems with the nerves of the eye (intraocular bleeding),
- cancerous tumors,
- rupture of major vessels.
Rarely, pheochromocytoma is cancerous (malignant) and cancerous cells spread to other parts of the body. The cancerous cells in pheochromocytoma or paraganglioma most often travel to the lymph system, bones, liver, or lungs.
When to see a doctor?
Although high blood pressure is the primary manifestation of pheochromocytoma, most people with high blood pressure do not have an adrenal tumor. Talk to your doctor if any of the following factors apply to you:
- difficulty controlling high blood pressure with current treatment,
- intermittent worsening of high blood pressure,
- family history of pheochromocytoma,
- family history of a related genetic disorder such as multiple endocrine neoplasiaType 2; von Hippel-Lindau disease; familial paraganglioma or neurofibromatosis 1,
- those with signs and symptoms of pheochromocytoma (hyperadrenergic attacks),
- resistant hypertension,
- very high blood pressure during anesthesia, intubation, surgery, pregnancy, angiography,
- hypertension at a young age,
Why is the Diagnosis of Pheochromocytoma Important?
Diagnosis of pheochromocytoma is important because:
•on average, 10% of it is cancerous,
•it may lead to a potentially fatal hypertensive crisis,
•cure can be achieved with surgical resection of the tumor.
What is the Treatment of Pheochromocytoma?
The main treatment in pheochromocytoma is surgical removal of the tumor. Most tumors are benign and can be removed.