What is pheochromocytoma?
Pheochromocytoma is a rare tumor of the adrenal gland tissue. It causes the announcement of too much epinephrine and norepinephrine, hormones that control heart rate, metabolism, and blood pressure.
Causes of pheochromocytoma
Doctors don’t know exactly what causes pheochromocytoma. Many appear randomly, but genetic factors seem to play a role in some cases.
In about 25 to 35% of cases, a person inherits the condition, according to the National Organization for Rare Diseases. It is most likely to appear between the ages of 20 and 50, but it can appear at any age.
A person with genetic characteristics related to pheochromocytoma may also be at increased risk of developing a variety of other conditions, including:
- Von Hippel-Lindau syndrome
- Multiple endocrine neoplasia type 2
- Neurofibromatosis type 1
- Sturge-Weber syndrome
For this reason, a person with a diagnosis of pheochromocytoma should consider genetic testing to assess the risk of other complications.
Episodes can happen at any time, but certain life events can also trigger them.
Possible triggers include:
- Straining the body during strenuous physical activity or childbirth
- Undergo surgery or a procedure that involves anesthesia
- High levels of emotional or physical stress
A compound called tyramine can also trigger symptoms. Tyramine is present in fermented or aged foods, such as red wine, chocolate, and some cheeses.
In some people, certain medications can also trigger symptoms. For example, monoamine oxidase inhibitors, a treatment option for depression and other mental health conditions, are known to trigger symptoms.
Multiple endocrine neoplasias, type IIB (MEN IIB)
Multiple Endocrine Neoplasia, Type IIB (MEN IIB) Open Popup Dialog. People who have certain rare inherited disorders are at increased risk for pheochromocytoma or paraganglioma. The tumors associated with these disorders are more likely to be cancerous. These genetic conditions include:
- Multiple endocrine neoplasia type 2 (MEN 2) is a disorder that causes tumors in more than one part of the hormone-producing (endocrine) system of the body. Other tumors associated with this condition can look on the thyroid, parathyroid, lips, tongue, and gastrointestinal tract.
- Von Hippel-Lindau disease can cause tumors at multiple sites, including the central nervous system, endocrine system, pancreas, and kidneys.
- Neurofibromatosis 1 (NF1) causes multiple skin tumors (neurofibromas), pigmented skin spots, and optic nerve tumors.
- Hereditary paraganglioma syndromes are inherited disorders that cause pheochromocytomas or paragangliomas.
Symptoms of pheochromocytoma
A sudden large increase in adrenal hormones is called an adrenergic crisis (AC). CA causes severe high blood pressure (hypertension) and a fast heart rate (tachycardia).
Common symptoms of CCP are:
- High blood pressure that may be resistant to conventional medications
- Fast heart rate or palpitations
- Abdominal pain
- Irritability and anxiety
Common risk factors for AC are:
- Medications, such as corticosteroids, chemotherapy agents, etc.
- Surgical anesthesia
- Emotional stress
Diagnosis of pheochromocytoma
A diagnosis of pheochromocytoma can be suspected based on a detailed history of the patient (including previous cases of pheochromocytoma in the family), a thorough clinical evaluation, and identification of characteristic findings (paroxysmal seizures, hypertension unresponsive to normal treatment, etc.). Blood and urine tests can confirm a diagnosis of pheochromocytoma by detecting elevated levels of catecholamines or their metabolites in urine and blood (plasma).
Metabolites are the by-products of catecholamines that are produced when the body breaks down (metabolizes) catecholamines. A specialized test, the clonidine suppression test, can be done to rule out other causes of elevated catecholamines. Clonidine reduces catecholamine production to normal ranges in individuals without pheochromocytoma, but not in those with pheochromocytoma.
Imaging techniques such as computed tomography (CT) and magnetic resonance imaging (MRI) are often done to determine the specific location and size of a pheochromocytoma. During the CT scan, a computer and X-rays are used to create a film that shows cross-sectional images of internal structures. MRI uses a magnetic field and radio waves to create full cross-sectional images of certain organs and tissues. Magnetic resonance imaging is the imaging method of choice for pregnant women who are suspected of having a pheochromocytoma.
Treatment for pheochromocytoma
You will most likely need surgery to remove the tumor. Your doctor may be able to do this by using small cuts instead of a large opening. This is called laparoscopic or minimally invasive surgery. You generally recover faster from these procedures than from traditional surgery.
Before surgery, you may need to take medicine to lower your blood pressure and control an occasionally fast heart rate. If you have a tumor in only one adrenal gland, your doctor will probably remove that entire gland. The other gland will make the hormones your body needs. If you have tumors in both glands, your surgeon may remove only the tumors and leave part of the glands.
If your tumor is cancerous, you may also receive radiation, chemotherapy, or targeted therapy (which uses drugs to attack specific cancer cells) to help keep it from growing.
High blood pressure can damage multiple organs, particularly the tissues of the cardiovascular system, the brain, and the kidneys. This damage can cause several critical situations, including:
- Heart disease
- Renal insufficiency
- Problems with the nerves of the eye.
In rare cases, a pheochromocytoma is cancerous (malignant), and the cancer cells spread to other parts of the body. Cancer cells from a pheochromocytoma or paraganglioma most often travel to the lymphatic system, bones, liver, or lungs.