This is a tumor that grows in the adrenal gland and it is called pheochromocytoma. Commonly, it is not a cancerous tumor (benign). Normally, people have two adrenal glands located on the top of each kidney. These glands produce hormones that help to control important body functions (such as blood pressure). While pheochromocytoma appears usually on one adrenal gland, other tumor types can happen on both glands.
Along with the adrenal gland the tumor also releases hormones that often cause different symptoms. For example hypertension (high blood pressure), headaches, sweating, and panic attacks. If you are not treating this tumor, it may lead to serious health problems for other body parts. Commonly, surgery that removes the tumor is enough to get rid of symptoms and return to a healthy range.
Symptoms
The following symptoms usually occur in people with this tumor type. Examples include:
- Headaches
- Fast heartbeat
- Excessive sweating
- Hypertension
However, some people may also experience the following symptoms. For example:
- Nervous shaking
- Breathing problems
- Anxiety
- Vision problems
- Constipation
- Weight loss
- Sudden intense fear
However, some people do not experience any symptoms and they do not know they have pheochromocytoma until an imaging test finds it.
Symptom Attacks
Commonly, the symptoms caused by pheochromocytoma come and go. When a person experiences that symptoms suddenly begin and continue to return it is known as spells or attacks. Symptom attacks may have a trigger but may not have but mostly the following conditions and activities trigger these spells. For example:
- Labor and delivery
- Body position changes (including bending over)
- Surgery
- Hard physical work
- Anxiety
- Stressful situations
Another attack’s triggers include food rich in Tyramine. This is a substance that can affect blood pressure. Tyramine is usually found in foods that are aged, cured, overripe, spoiled, pickled, or fermented. Check below some of them:
- Certain cheeses
- Chocolate
- Smoked or dried meats
- Soybeans and any soy products
- Some wines and beers
Furthermore, there are some medications that can trigger spells. For example:
- Tricyclic antidepressants such as Desipramine, Amitriptyline, and others.
- Monoamine Oxidase Inhibitors (MAOIs) include Phenelzine, Tranylcypromine, Isocarboxazid, and others. Moreover, the risk of symptom attacks increases if these medications are administered along with drinks and foods rich in Tyramine.
- Stimulants (including caffeine, amphetamine, cocaine, and others).
The primary symptom of pheochromocytoma is hypertension, but many people have increased blood pressure but do not have this tumor type. However, immediately visit a doctor if you notice any of the following cases. Examples include:
- It is challenging to control blood pressure despite following a treatment
- Hypertension occurs before 20 years old
- Family history of pheochromocytoma
- Symptom attacks are associated with this tumor type (including headaches, sweating, and pounding heartbeat)
- Have a family history of genetic disorders (such as multiple endocrine neoplasia, type 2 (MEN 2), von Hippel-Lindau disease, inherited paraganglioma syndromes, and neurofibromatosis 1
Causes
The exact cause of this tumor is not known. However, pheochromocytomas appear in the cells known as chromaffin cells. These cells are located in the center of the adrenal gland and make hormones mostly Adrenaline and Noradrenaline. Previous hormones play a vital role in the body because they help to control heart rate, blood sugar, blood pressure, and other functions.
Hormones released by chromaffin cells prepare the body for a fight-or-flight response that usually happens when the body thinks a threat appears. Therefore, Adrenaline and Noradrenaline make the body act fast when needed by increasing blood pressure and heart rate. However, a pheochromocytoma provokes the release of the hormones when the body is not in a threatening situation.
While most chromaffin cells are located in the adrenal glands, small amounts of them can be found in the heart, head, neck, bladder, stomach region, and spine. When tumors happen in any of the previous areas, they are called paragangliomas. The effects of the paragangliomas are similar to those of pheochromocytomas.
Risk Factors
The risk of pheochromocytomas elevates according to the person’s age and medical conditions. However, this tumor can happen at any age. Commonly, pheochromocytomas are found in people between 20-50 years old.
A high risk of developing pheochromocytomas have people with rare genetic diseases. Check below some conditions associated with pheochromocytomas:
- Multiple endocrine neoplasia, type 2 (MEN 2) – This is a disease that makes tumors appear in more than one body part that produces hormones (endocrine system). There are two types of this genetic disorder including type 2A and type 2B and both of them can provoke pheochromocytoma. Other body parts in which MEN 2 can provoke tumors include parathyroid glands, thyroid, lips, mouth, and digestive system.
- Von Hippel-Lindau disease – Commonly, this disease causes tumors in different body parts including the brain, spinal cord, endocrine system, pancreas, and kidneys.
- Neurofibromatosis 1 – This is a medical condition that provokes tumors in the skin (neurofibromas) but the tumors can also appear in other body parts such as the optic nerve.
- Hereditary paraganglioma syndromes – This group of health conditions is passed in families. Moreover, they are associated with paragangliomas and pheochromocytomas.
Complications
It is not a good idea to ignore this problem although sometimes it may not cause any symptoms because it may lead to life-threatening complications. Hypertension associated with pheochromocytoma can damage other body organs such as the brain, kidneys, blood vessels, and others. Check below some examples:
Additionally, rarely pheochromocytoma is cancerous and it spreads throughout the body. Pheochromocytoma’s cancerous cells commonly go to the lymphatic system, bones, liver, or lungs.
Diagnosis
To determine whether you have pheochromocytoma or not, doctors perform different tests. Examples include:
Laboratory Tests
The following tests measure hormone levels (Adrenaline and Noradrenaline) and certain substances along with previous hormones (Metanephrines). If tests show you have increased Metanephrines levels it is usually a symptom of pheochromocytoma. However, Metanephrines levels usually are not high when someone does not have this tumor and symptoms can be caused due to other conditions.
- Blood test – A sample of your blood will be sent to the laboratory for testing.
- 24-hour urine test – This test involves collecting urine samples every time you urinate during a whole day.
Before previous tests, discuss with your healthcare professional about precautions. For instance, you may need to not eat or not administer any medicines before these tests.
Imaging Tests
Physicians recommend the following tests if lab tests show pheochromocytoma signs. For example:
- CT (computed tomography) scans
- MRI (magnetic resonance imaging)
- M-iodobenzylguanidine (MIBG) imaging
- PET (positron emission tomography
Sometimes, adrenal gland tumors are found during imaging tests for other health problems. However, if the tumor is found, the treatment is required.
Genetic Test
Doctors usually advise genetic tests if the adrenal gland tumor is linked with a genetic disorder. In any case, genetic factors are important for different reasons. Examples include:
- Those who have a genetic disorder are at higher risk of developing other health problems along with this adrenal gland tumor.
- In some cases, the genetic conditions can return or be cancerous.
- The outcomes from genetic tests may indicate that family members should be tested for pheochromocytoma or other diseases.
Treatment
The primary treatment for this tumor type is surgery that removes it. Doctors often prescribe blood pressure medications before surgery to reduce levels of the hormones (Adrenaline). Otherwise, blood pressure may elevate to dangerous levels during the tumor removal procedure. Check below some treatment options if the tumor is cancerous:
- Targeted Therapies – This treatment involves a combination of medicines with a radioactive substance that helps to find and destroy cancer cells.
- Chemotherapy – In such cases, doctors recommend strong medicines to kill cancer cells. This treatment option is commonly used in people whose cancer spreads.
- Radiation Therapy – It involves beams with powerful energy that help to destroy cancer cells.
- Ablation – This treatment method involves destroying cancerous tumors by freezing them.
Frequently Asked Questions
What is life expectancy for people with pheochromocytoma?
For those who suffer from a cancerous type of pheochromocytoma and it spreads throughout the body, the five-year survival rate is between 34%-60%.
What is the best treatment option for pheochromocytoma?
Commonly, physicians recommend surgery to remove the tumor. However, the surgery can be performed safely after you follow a treatment with alpha-blockers (such as Phenozoybenzamine). The duration of the treatment with medicines may last up to 3 weeks. Consult with your doctor for more details.
What are the primary pheochromocytoma symptoms?
Those who have this adrenal gland tumor experience hypertension, headaches, excessive sweating, and palpitations (fast heart rate). If you experience any of them, contact your doctor right away. Ask your healthcare professional if you have additional questions.
