Pheochromocytoma is a rare tumor that develops in the adrenal glands in the body.
A pheochromocytoma can cause distinct episodes of symptoms in the body. Diagnosis often requires multiple steps, and there are different factors to be considered for treatment.
Contents of this article:
What is the adrenal gland?
The adrenal glands create hormones and steroids for the body that help to balance blood pressure, metabolism, and heart rate.
The adrenal glands are two small endocrine glands that lie above the kidneys. They help create and distribute important hormones and steroids for the body to use, including adrenaline and cortisol.
Adrenal hormones help balance bodily functions, such as:
- heart rate
- blood pressure
- blood sugar
- responses to stress
- immune system responses
Dysfunction of the adrenal glands can cause a range of health concerns in these areas.
What is pheochromocytoma?
Pheochromocytoma (PCC) is the term used for a rare tumor that has grown in the adrenal glands. These tumors are usually benign, meaning they are not cancerous. Most cases involve only one adrenal gland, but it is possible for both to be involved.
In rare cases, PCC can be cancerous. These malignant tumors will spread like other cancers and can cause serious complications.
The condition causes the adrenal glands to pump out too much adrenaline and noradrenaline. These two hormones help keep the heart rate, blood pressure, and stress response in balance. They are also responsible for the body’s “fight or flight” response.
When the body contains too much of these compounds at one time, it goes into a reactive state as if it were in a constant state of high stress.
PCC will always form on the inside of the adrenal glands. They are often grouped together with similar tumors called paragangliomas, which are tumors that grow on the outside of the glands. The tumors are different, but both alter the production of the adrenal glands.
Symptoms of PCC can vary greatly from person to person. For instance, some people experience consistent high blood pressure while others only have random rises in blood pressure.
Some people never experience symptoms and only find out about their PCC when a doctor is looking for something else.
Symptoms usually show up in episodes with a distinct combination of symptoms. People will often experience headaches, irregular heartbeats, and unusual sweating. They will also have high blood pressure if it is measured when they are in this state.
These episodes may last for seconds or hours. Episodes tend to get worse over time and become more frequent as the tumor grows in size.
Other common symptoms of PCC can occur with these episodes or independently. These symptoms include:
A headache, unusual sweating, and an irregular heartbeat may be combined symptoms of PCC.
- stomach pain
- weight loss
- rapid heart rate
- sudden headaches
- anxiety or a sense of doom
- being shaky
- being extremely pale
- mood swings and irritability
Having PCC can disturb the normal function of the adrenal glands. This can make people more at risk for an adrenergic crisis, which causes bursts of adrenal hormones to enter the bloodstream.
People experiencing an adrenergic crisis may have extremely high blood pressure and a rapid heart rate. This can cause serious health problems, such as heart attack, stroke, or death.
The symptoms of PCC can be due to a range of conditions, and if a person notices any of the symptoms, it is essential that they see a doctor quickly to get a proper diagnosis.
Episodes of symptoms may occur at any time, or they may be brought on by certain life events.
Straining the body during hard physical activity or childbirth may be enough to trigger an attack. Surgeries or processes involving anesthesia may trigger events in some people as well. Having a lot of physical or emotional stress may also cause an episode.
A compound called tyramine may also be responsible for triggering symptoms of PCC. Tyramine is found in fermented or aged foods, such as red wine, chocolate, and some cheese.
Certain drugs may also trigger symptoms in some people. These can include monoamine oxidase inhibitors (MAOIs) and stimulants, such as cocaine or amphetamines.
Medical science is not exactly sure what causes PCC. It may develop at any age, though it appears most common in adults aged 20 to 50. The condition also appears to be linked to genetics.
People who develop PCC caused by genetics may also be more at risk to inherit other genetic conditions. There are three syndromes classically associated with PCC:
- von Hippel-Lindau syndrome
- multiple endocrine neoplasia type 2
- neurofibromatosis type 1
There are now at least eight different genetic dysfunctions that are considered to increase the risk of PCC.
PCC can be difficult to diagnose and requires a few different steps. Doctors will order a variety of tests to check for key markers of the condition.
Blood or urine tests may tell doctors if a person has abnormally high levels of hormones in their body. A CT scan or MRI may then be used to take an image of the adrenal glands if a tumor is suspected.
If doctors find a tumor, they may order tests to see if it is caused by a genetic disorder. This may help determine if the person is at risk for other genetic disorders or if their family members will have a higher risk of the disorder.
Removing the tumor via non-invasive laparoscopic surgery is one of the best ways to treat PCC.
The best way to treat PCC is to remove the tumor. This is most often done by non-invasive laparoscopic methods, which require only small incisions. The benefits of these methods are a shorter recovery time and less of a chance for complications from surgery.
In the days leading up to the surgery, the person with PCC may have to take medication to balance out their heart rate and lower their blood pressure.
In most cases of PCC, a tumor grows in a single adrenal gland. In these situations, doctors may remove the entire gland, as the remaining gland is capable of producing the hormones the body needs to survive.
When a person has tumors in both adrenal glands, doctors will try to remove the tumor tissue only, leaving a part of the glands intact. If this is not possible, they may have to remove both adrenal glands. With benign tumors, removing the tumor will usually cause the symptoms to go away.
People who have both adrenal glands removed will need to take steroids to help replace the hormones the body can no longer make. People who have cancerous tumors may require radiation or chemotherapy to keep the tumor from coming back or spreading.
All people with PCC are likely to experience complications. People with PCC that remains untreated, however, are at a higher risk of developing heart problems, such as high blood pressure, irregular heartbeats, and heart attack. They are also more likely to experience organ failure and damage to the optic nerve.
Surgery comes with its own complications. Some conditions can develop during surgery, including high blood pressure crisis, low blood pressure crisis, and irregular heartbeat.
Surgery also involves altering the adrenal glands, which can cause an imbalance in the hormones. Most people will be given steroids during recovery to help balance out these hormones.