What’s to know about pheochromocytoma?

A rare tumor that develops in the adrenal glands is pheochromocytoma, or “pheo.” It affects adrenaline production and can contribute to high blood pressure and other health issues. It is usually benign.

However, pheochromocytoma can be cancerous in around 3–13 percent of cases. Cancer cells can spread to other parts of the body in such cases.

Most mild cases include one adrenal gland only, although both may be affected by the disease. Two small endocrine glands that reside above the kidneys are the adrenal glands. They assist in the development and distribution of significant hormones, including adrenaline and cortisol.

Adrenal hormones assist to regulate functions like:

As a result, pheochromocytoma can affect a wide range of bodily functions.

What is pheochromocytoma?

A person with pheochromocytoma
A person with pheochromocytoma may experience headaches, heart palpitations, and sweating.

The adrenal glands in pheochromocytoma contain too much adrenaline, noradrenaline, or both.

These hormones, like the fight-or-flight response, help regulate the heart rate, blood pressure, and stress response.

The body will reach a condition resembling chronically high stress when levels of these hormones are too high. The body will react as though it were constantly under stress.

Pheochromocytomas are linked to paragangliomas, another form of tumor. These develop on the outside of the adrenal glands but in the same types of tissue. Both can result in adrenal hormone abnormalities.

Symptoms

Symptoms of pheochromocytoma differ from individual to individual. About 60 percent of individuals with this disorder experience high blood pressure, either chronic or intermittent.

Some individuals experience consistent high blood pressure, while others may have high blood pressure episodes. This can occur from several times a day to twice a month.

In episodes, other symptoms typically occur, with a distinct combination.

The most common pheochromocytoma symptoms are:

Other symptoms that can occur during an episode or independently include:

  • nausea
  • stomach pain
  • weight loss
  • rapid heart rate
  • anxiety or a sense of doom
  • feeling shaky
  • being extremely pale
  • mood swings and irritability
  • high blood sugar

Symptoms can be seconds or hours long. Over time, they begin to intensify and become more common as the tumour develops.

Some persons, however, experience no symptoms. Only after they undergo an imaging examination for another disease will these individuals discover that they have pheochromocytoma.

Adrenergic crisis

The regular activity of the adrenal glands may be disrupted by pheochromocytoma. This can raise the likelihood of an adrenergic crisis that happens when the bloodstream enters bursts of adrenal hormones.

An adrenergic crisis, both of which can be life threatening, can lead to a heart attack or stroke.

Extremely high blood pressure and rapid heart rate are signs of an adrenergic crisis.

Getting these symptoms does not mean a person certainly has pheochromocytoma, as they can occur with other conditions. However for diagnosis, anyone who notices these signs should see a physician.

What causes hypertension? Find out more here.

Causes

Happy family
People with a family history of pheochromocytoma could consider genetic testing.

Doctors do not know what causes pheochromocytoma exactly. Many occur spontaneously, but in certain cases, genetic factors appear to play a role.

According to the National Organization for Rare Diseases, a person inherits the disorder in about 25–35 percent of cases.

At the age of 20-50 years, it is most likely to occur, but at any age it can appear.

An individual with pheochromocytoma-related genetic features may also have a higher risk of developing a number of other conditions, including:

  • von Hippel–Lindau syndrome
  • multiple endocrine neoplasia type 2
  • neurofibromatosis type 1
  • Sturge–Weber syndrome

For this reason, in order to assess the risk of other complications, a person diagnosed with pheochromocytoma should consider undergoing genetic testing.

Triggers

Episodes can occur at any moment, but they can also be caused by certain life events.

Possible triggers include:

  • straining the body during hard physical activity or childbirth
  • undergoing a surgery or procedure that involves anesthesia
  • high levels of emotional or physical stress

Symptoms can also trigger a compound called tyramine. Fermented or aged foods, such as red wine, chocolate, and some cheeses, contain tyramine.

Some medications can also cause symptoms in some individuals. Monoamine oxidase inhibitors, for instance, have been known to cause symptoms as a treatment alternative for depression and other mental health problems.

Diagnosis

It can be hard to diagnose pheochromocytoma, since it is rare and affects multiple individuals in various ways.

To rule out or confirm pheochromocytoma, a person with either of the following should undergo testing:

  • episodes of high blood pressure or constant high blood pressure
  • heart palpitations, sweating, headaches, or other symptoms
  • an incidental adrenal mass

Tests

Checking for key markers of the condition can help with a number of tests. For instance:

Blood or urine tests: These can show whether a person’s body contains unusually high levels of adrenaline or noradrenaline.

Imaging tests: A CT or MRI scan of the adrenal glands can confirm that a tumor is present. In imaging tests, pheochromocytomas often have a distinctive appearance.

Genetic testing: If investigations confirm a tumor, genetic testing may be recommended by a doctor. This can show whether the individual has a mutation in the gene that increases the risk of this and other tumors. They may also recommend screening for family members if a gene mutation is present.

Treatment

Surgery for pheochromocytoma aims to remove the tumor.
Surgery for pheochromocytoma aims to remove the tumor.

Usually, a doctor would recommend surgically removing the tumor. Laparoscopic surgery, which requires only small incisions, is mostly opted for by surgeons.

Just one adrenal gland is normally affected by pheochromocytoma. Therefore a surgeon will remove the entire gland because the remaining gland will produce enough hormones for the survival of the body.

The surgeon can attempt to remove the tumor tissue only if tumors arise in both glands, leaving a portion of the glands intact. They will have to remove both adrenal glands if this is not feasible.

If both adrenal glands are removed by the surgeon, the body will no longer be able to produce adrenal hormones. To help replace these hormones, the person will need to take steroids.

Changes in blood pressure or an irregular heartbeat during surgery can lead to complications. The person may need to take medicine for several days before the surgery to balance their heart rate and reduce their blood pressure.

Pheochromocytomas are mostly benign. The symptoms will normally go away after surgery.

Malignant tumors

However in some situations, the person may have a malignant tumor that may recur at distant sites, typically the lungs, bones, or liver.

If the tumor is malignant, options for therapy include:

Addison’s disease also affects the adrenal glands. Learn more here.

Complications

Pheochromocytomas are mostly benign. However they may contribute to complications without treatment.

Possible complications include:

  • heart muscle disease (cardiomyopathy)
  • a heart attack (myocardial infarction)
  • bleeding in the brain (cerebral hemorrhage)
  • a buildup of fluid in the lungs (pulmonary edema)

An individual may also have high, hard-to-control blood pressure.

Outlook

Pheochromocytoma is unusual and is typically not cancerous. It can however, lead to severe complications without treatment.

Typically, removing the tumor would cause the symptoms to go away. However since certain pheochromocytomas may recur, a physician can prescribe lifelong follow-up.

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