Pheochromocytoma – Symptoms and Causes
Overview
Pheochromocytoma is a rare tumor that develops in an adrenal gland. These tumors are typically benign (non-cancerous). The adrenal glands sit on top of each kidney and produce important hormones that regulate bodily functions like blood pressure.
Most often, pheochromocytoma affects just one adrenal gland, though it can sometimes occur in both. The tumor causes problems by releasing excess hormones into the bloodstream, which can trigger various symptoms.
Common symptoms include:
- High blood pressure
- Headaches
- Excessive sweating
- Panic attack-like symptoms
Without proper treatment, pheochromocytoma can lead to serious complications affecting multiple body systems. The primary treatment approach is surgical removal of the tumor, which usually normalizes blood pressure and resolves other symptoms.
Signs and Symptoms
People with pheochromocytoma often experience symptoms that come and go in episodes or attacks. These episodes can happen suddenly and may repeat over time, sometimes without any clear cause.
Several things can trigger these symptom episodes:
- Physical Activity: Heavy exercise or exertion.
- Emotional States: Feeling anxious or stressed.
- Position Changes: Standing up after sitting or lying down, or bending over.
- Medical Situations: During childbirth or surgery with anesthesia.
Foods with high tyramine content may also set off symptoms. Tyramine affects blood pressure and is found in:
- Aged or strong cheeses
- Some alcoholic beverages
- Soy products
- Chocolate
- Cured meats
Certain medications can also trigger symptom episodes:
- Tricyclic antidepressants
- Monoamine oxidase inhibitors (MAOIs)
- Stimulants, including caffeine, amphetamines, and cocaine
During these episodes, you may experience:
- High blood pressure
- Headaches
- Heavy sweating
- Rapid heartbeat
- Trembling
- Pale skin
- Breathing difficulties
- Panic-like feelings
- Anxiety
- Vision problems
- Digestive issues, including constipation
- Weight loss
Some people with this tumor have no symptoms at all. In these cases, the tumor might only be discovered during imaging tests done for other reasons.
When To Contact a Doctor
You should speak with a healthcare provider if you experience:
- Recurring symptom episodes that include headaches, excessive sweating, and a pounding heartbeat
- Difficult-to-control high blood pressure despite treatment
- Early-onset high blood pressure (before age 20)
- Sudden, significant blood pressure spikes
It’s especially important to seek medical advice if you have:
- A family history of pheochromocytoma
- A family history of related genetic conditions like multiple endocrine neoplasia type 2, von Hippel-Lindau disease, inherited paraganglioma syndromes, or neurofibromatosis 1
While high blood pressure is a main symptom of pheochromocytoma, most people with high blood pressure don’t have this tumor. However, these additional factors warrant medical attention to determine the cause of your symptoms.
Causes
A pheochromocytoma develops in the chromaffin cells found at the center of an adrenal gland. While experts haven’t identified the exact cause, they understand how these tumors affect the body.
Chromaffin cells normally produce important hormones—mainly adrenaline and noradrenaline. These chemicals control critical body functions like heart rate, blood pressure, and blood sugar levels.
These hormones typically trigger the body’s “fight-or-flight” response during threatening situations. When this happens:
- Blood pressure increases
- Heart rate speeds up
- Other body systems prepare for quick reaction
With a pheochromocytoma, these hormones are released in excessive amounts and at inappropriate times—when no threat exists.
Most chromaffin cells are located in the adrenal glands. However, small clusters can also be found in other body parts, including:
- Heart
- Head and neck
- Bladder
- Stomach area
- Along the spine
When chromaffin cell tumors develop outside the adrenal glands, they’re called paragangliomas. These tumors can produce the same effects on the body as pheochromocytomas, causing similar symptoms and hormone disruptions.
Risk Factors
Several factors may increase a person’s chance of developing a pheochromocytoma.
Age
Most cases of pheochromocytoma occur in adults between 20 and 50 years old. However, these tumors can develop at any age.
Genetic Conditions
People with certain inherited disorders have a higher risk of developing these tumors. In these cases, the tumors may be benign (non-cancerous) or malignant (cancerous). Genetic conditions linked to pheochromocytomas include:
Multiple Endocrine Neoplasia Type 2 (MEN 2): This disorder causes tumors in hormone-producing glands. Both subtypes (2A and 2B) can involve pheochromocytomas. Related tumors may also appear in the thyroid, parathyroid glands, and digestive system.
Von Hippel-Lindau Disease: This condition leads to tumor formation in multiple body parts, including the brain, spinal cord, pancreas, kidneys, and endocrine system.
Neurofibromatosis 1: People with this condition develop skin tumors called neurofibromas. They may also have tumors on the optic nerve, which connects the eye to the brain.
Hereditary Paraganglioma Syndromes: These family-inherited conditions can cause pheochromocytomas or related tumors called paragangliomas.
When pheochromocytomas develop due to genetic conditions, they often form in both adrenal glands rather than just one.
Cancer Tumors
In rare cases, tumors may become cancerous. The cancer cells can then spread to other parts of the body. When cancer cells travel from these tumors, they most commonly move to:
- Lymph system
- Bones
- Liver
- Lungs
The high blood pressure associated with these tumors can damage important organs throughout the body. This damage affects the heart, blood vessels, brain, and kidneys, potentially leading to serious health issues.
Potential complications include:
- Heart disease
- Stroke
- Kidney failure
- Vision loss
