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A pheochromocytoma is an infrequent tumor that typically has a non-malignant nature, and it arises within one of the adrenal glands situated on top of each kidney. The adrenal glands are a crucial component of the body’s endocrine system, responsible for producing various hormones.
A pheochromocytoma often only affects one adrenal gland. However, it can form in both glands.
The inner layer of the adrenal gland, known as the adrenal medulla, is where 80–85% most pheochromocytomas develop. The term extra-adrenal pheochromocytomas or paragangliomas refers to the 15-20% of pheochromocytomas that develop outside of this region.
If an individual has a pheochromocytoma, the tumor can discharge hormones that may cause panic attack-like symptoms, elevated blood pressure, headaches, and sweating. If left untreated, the pheochromocytoma can lead to severe or even life-threatening damage to other bodily systems.
Blood pressure typically returns to normal after a pheochromocytoma has been surgically removed.
Symptoms
Pheochromocytoma symptoms and signs frequently include:
- Elevated blood pressure
- Headache
- Profused sweating
- Tremor
- Increased heart rate
- Being pale
- Difficulty of breathing
- Symptoms similar to a panic attack
Less frequent signs or symptoms could be:
- Losing weight
- Anxiousness or fear of something terrible is going to happen
- Constipation
- Blurred vision
Symptomatic spells
Pheochromocytoma symptoms can be persistent, sporadic, or both, and they can also worsen. Activities, environmental factors, meals heavy in tyramine, a compound that influences blood pressure, and drugs can all trigger these episodes.
The following situations or behaviors can exacerbate symptoms:
- Strenuous physical activity
- Anxiety
- Stress
- Changing one’s physical position, such as standing up after lying down or sitting
- Labor and giving birth
- Having surgery and using anesthesia, a medicine that puts you to sleep during the procedure
Tyramine, a chemical that influences blood pressure, is present in foods and can exacerbate symptoms. Foods that have been fermented, aged, pickled, cured, overripe, or spoilt frequently contain tyramine. Examples of these foods are beers, wines, chocolates, smoked or dried meats, and certain types of cheese.
There are some drugs that can elevate the symptoms, including Monoamine Oxidase Inhibitors (MAOIs) (e.g., phenelzine, tranylcypromine and isocarboxazid), and stimulants (e.g., amphetamines and cocaine).
Despite the fact that a pheochromocytoma’s major symptom is elevated blood pressure, most persons with this condition do not also have an adrenal tumor. If any of the following apply to you, speak with your health care provider:
- A history of pheochromocytoma in the family
- A history of a related genetic ailment in the family, such as neurofibromatosis type 1, von Hippel-Lindau disease, hereditary paraganglioma syndromes, or multiple endocrine neoplasia, type 2 (MEN 2).
- High blood pressure that is difficult to manage with existing therapies
- Periods in which high blood pressure worsens
Causes
The specific causes of pheochromocytomas remain unknown, as per researchers. These tumors develop in the chromaffin cells, specialized cells present in the center of the adrenal gland. The primary hormones released by these cells are adrenaline (epinephrine) and noradrenaline (norepinephrine), which regulate several bodily processes like heart rate, blood pressure, and blood sugar levels.
In response to a perceived threat, adrenaline and noradrenaline trigger the body’s fight-or-flight response, causing an increased heart rate and elevated blood pressure, preparing the body for a quick reaction. However, a pheochromocytoma releases excessive amounts of these hormones, even when there is no danger, leading to symptoms.
Adrenal glands contain the majority of the chromaffin cells. However, there are also minor groups of these cells in the heart, head, neck, bladder, back wall of the stomach, and throughout the spine. Paragangliomas are chromaffin cell tumors that develop outside of the adrenal glands. They may have the same impact on the body to a pheochromocytoma.
Risk factors
The majority of pheochromocytomas are found in patients between the ages of 20 and 50. However, a tumor can appear at any age.
Pheochromocytoma or paraganglioma risk is higher in people with some rare hereditary diseases. These conditions increase the likelihood that a tumor will be malignant. Both of the adrenal glands are also more prone to developing tumors. These inherited conditions consist of:
- Multiple endocrine neoplasia, type 2 (MEN 2) is a condition that causes tumors in multiple endocrine system organs. Both type 2A and type 2B are categories of multiple endocrine neoplasia (MEN) disorders that are associated with the development of pheochromocytomas. The thyroid, parathyroid, lips, tongue, digestive system, and other bodily regions may also develop tumors related with this illness.
- Von Hippel-Lindau disease may lead to tumors at a variety of locations, including the kidneys, pancreas, endocrine system, and central nervous system.
- Neurofibromatosis 1 generates a lot of skin tumors (neurofibromas), pigmented skin spots, and tumors of the optic nerve.
- Hereditary paraganglioma syndromes are hereditary diseases that can cause paragangliomas or pheochromocytomas.
Diagnosis
To diagnose pheochromocytoma, your doctor may order a series of tests.
Lab tests
These exams check your body’s levels of adrenaline, noradrenaline, or chemicals that are produced as a result of those hormones:
- 24-hour urine test. Every time you urinate over the course of a 24-hour period, you must collect a urine sample for this test. Request written directions on how to label, store, and send back the samples.
- Blood test. Blood will be drawn for laboratory testing.
It is important to consult with your doctor regarding any necessary preparations for both types of tests. For example, you may be advised to fast (abstain from food) or skip a medication dose before the test. However, it is crucial to never miss a prescription dose without your doctor’s permission.
Imaging tests
If the lab test results indicate the presence of a paraganglioma or pheochromocytoma, your doctor will likely request one or more imaging tests to search for a possible tumor. These tests may include:
- Computed tomography (CT) scan, an advanced form of X-ray technology
- Magnetic Resonance Imaging (MRI) combines a magnetic field and radio waves to create detailed images.
- M-iodobenzylguanidine (MIBG) imaging, a scanning technique that can find minute amounts of injected radioactive material absorbed by paragangliomas or pheochromocytomas
- Positron emission tomography (PET), a scanning technique that is also capable of identifying radioactive materials absorbed by a tumor
Imaging tests performed for other purposes may detect a tumor in an adrenal gland. If that occurs, your doctor will typically request additional testing to learn more about the tumor.
Genetic testing
To ascertain whether a pheochromocytoma is connected to an inherited condition, your doctor may advise genetic testing. Information on potential genetic influences may be crucial for a variety of reasons, including:
- Since some genetic abnormalities can result in a number of conditions, test results may indicate the need for additional medical screenings.
- Because some illnesses are more likely to recur or be malignant, your test results may have an impact on the course of your therapy or the long-term plans you make to monitor your health.
- Test results may indicate that other family members should undergo screenings for pheochromocytoma or disorders that are similar.
You can better comprehend the outcomes of your genetic testing with the aid of genetic counseling.
Treatment
The primary treatment for pheochromocytoma is surgery to remove the tumor. Prior to the procedure, your doctor may recommend certain blood pressure medications. These medications suppress the high-adrenaline hormones and help reduce the risk of dangerously high blood pressure during surgery.
Preparations before surgery
Before surgery, you’ll probably need to take drugs for 7 to 10 days to decrease your blood pressure. Other blood pressure medications you now use will either be replaced by these medications or added. A high-salt diet may also be suggested for you.
Smaller veins and arteries are kept open and relaxed by medications including alpha blockers, beta blockers, and calcium channel blockers. Blood flow is enhanced, and blood pressure is reduced. Your heart may beat more slowly and with less force as a result of certain of these drugs. The blood pressure may drop even lower as a result.
These drugs reduce blood vessel fluid because they enlarge the blood vessels, which results in reduced blood vessel fluid. When standing, this might result in potentially fatal reductions in blood pressure. In order to prevent low blood pressure during and after surgery, a high-salt diet will draw more fluid into the blood vessels.
Surgery
Laparoscopic surgery is typically used to execute the procedure to remove an adrenal tumor. To complete the procedure, your surgeon will make a few minor cuts (incisions) in your abdomen. These wounds will be used to introduce wand-like instruments that are fitted with cameras and small tools.
With a pheochromocytoma, your surgeon often removes the entire adrenal gland. Your surgeon, however, might merely remove the tumor and leave some healthy adrenal gland tissue. When the other adrenal gland has also been removed or when both adrenal glands have tumors, this may be done.
The tumor and any surrounding malignant tissue will be removed if a tumor is cancerous. Even if all of the malignant tissue is not removed, medical treatment and surgery may lessen hormone production and help to regulate blood pressure.
After surgery
The remaining healthy adrenal gland is capable of performing the tasks typically handled by two glands. Normal blood pressure levels usually recover. For the rest of your life, you’ll require routine checkups with your doctor to monitor your health, spot issues, or determine whether the tumor has returned.
Cancer treatments
Malignant pheochromocytomas that are cancerous are rare, and as a result, the most effective treatments have received limited attention in research. However, treatment options for malignant tumors related to pheochromocytoma and cancer that has spread throughout the body may include:
- Targeted therapies combining a radioactive substance that kills cancer cells with a medicine or substance that targets cancer cells for destruction. There may potentially be drug therapies that target certain aberrations in cancer cells that enable their survival.
- Chemotherapy employing strong medications that kill cancer cells that develop quickly.
- Radiation therapy may ease the pain-causing symptoms of malignancies that have progressed to the bone.
