Pheochromocytoma: Everything You Need to Know

April 2021

In a nutshell, Pheochromocytoma is a tumor in the adrenal gland. It can be present for years before showing any symptoms, but when it does happen, it's always sudden and dramatic to say the least.

This post will outline the history of this condition and talk about what you need to know if you suspect that you might have one too.

Pheochromocytoma is a rare form of cancer that develops in 1-3% of people with high blood pressure and 3-5% of people with headaches. It is more common in women than men, but it could develop at any time of life. Pheochromocytoma can also develop in people that have no history of high blood pressure or headaches.

The rapid and unexpected death that occurs in people with pheochromocytoma is due to a sudden and dramatic release of catecholamines (the body's chemical messengers). These catecholamines cause the heart to beat faster, to increase blood pressure, and to cause the kidneys to work harder. The sudden increase in blood pressure and other effects are what causes the death if they are not treated immediately 

Pheochromocytoma causes excess production of adrenaline (also known as epinephrine or "fight-or-flight" hormone). This can cause people to feel tense, have an increased heart rate and blood pressure, sweat excessively, tremble or shake. While pheochromocytoma does not usually affect a person's normal memory, people who have this tumor do need to go to the doctor more often than usual.

Pheochromocytoma can be diagnosed using a variety of tests, including blood tests and CT scans. People who have been diagnosed with pheochromocytoma must avoid certain activities. They may need to go to the doctor more often and should not drive a car or operate heavy machinery; people with this type of tumor often have episodes where they are so nervous that they cannot stand up or could become dizzy. If someone has an adrenal gland tumor, doctors may recommend treatment. It may vary, depending on the size of the tumor and where it is located.

Imaging: MRI or CT scan of the abdomen and pelvis to detect pheochromocytoma, which is often found as a solid enhancing mass. Synchronous metastases in the lungs are seen in 30% of patients.  Genetic testing can also be performed on these tumors to make a diagnosis. Sometimes, pheochromocytomas can be discovered by operation while removing another tumor.

Diagnosis: Diagnosis can be difficult because the abnormal catecholamine secretions can cause a variety of different physical and behavioral symptoms, which resemble those of other diseases.  These can include headaches, tremor, anxiety, palpitations, or frank hypertension. On occasion the symptoms mimic those of Parkinson's disease.

Primary tumors are usually detected after blood tests reveal abnormally high levels of catecholamines (epinephrine and/or norepinephrine).  To confirm the diagnosis, the patient is typically given a "stress test" or an  ECG (electrocardiogram) to measure heart rate – which may show an unusually elevated heart rate in response to mild stress.

To confirm the diagnosis, a computed tomography (CT) scan of the abdomen and pelvis is sometimes performed. If the test is normal, further testing may be helpful to distinguish malignant from benign pheochromocytomas, to locate other tumors if they are found, and to assess the tumor's size and growth pattern.  About half of all pheochromocytomas are NOT malignant.  Malignant pheochromocytoma can be found by chest X-ray or autopsy.  If a tumor is detected during examination or surgery for another tumor, then it should be removed – if possible – at that time as well with only minimal risk of malignancy.

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