Pheochromocytoma
What is Pheochromocytoma?
Pheochromocytoma is a rare tumor that grows in the adrenal glands. These small glands sit on top of your kidneys. They make hormones that help control your heart rate, blood pressure, and stress response.
This tumor causes your adrenal glands to release too much of certain hormones called catecholamines. These include adrenaline and noradrenaline. When your body has too much of these hormones, you may experience sudden spikes in blood pressure and heart rate. Most pheochromocytomas are not cancerous, but they still need treatment because the hormone surges can be dangerous.
Pheochromocytoma affects about 2 to 8 people per million each year. It can happen at any age but most commonly appears between ages 30 and 50. Some people inherit genes that increase their risk. Others develop it without any family history.
Symptoms
- Severe headaches that come on suddenly
- Heavy sweating without physical activity or heat
- Rapid or pounding heartbeat
- High blood pressure that may spike suddenly
- Pale or flushed skin
- Tremors or shaking
- Anxiety or sense of doom
- Chest pain or abdominal pain
- Nausea or vomiting
- Weight loss despite normal eating
Symptoms often come in episodes called spells or attacks that last minutes to hours. These spells may happen several times a day or just a few times per month. Some people have persistent high blood pressure between episodes. Others may have no symptoms at all until the tumor is discovered during testing for another reason.
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Causes and risk factors
Pheochromocytoma develops when cells in the adrenal gland grow abnormally and form a tumor. Doctors do not always know why this happens. About 30 to 40 percent of cases are linked to inherited genetic conditions. These include multiple endocrine neoplasia type 2, von Hippel-Lindau disease, and neurofibromatosis type 1. People with these conditions have a higher chance of developing this tumor.
Most cases happen without a clear family history or genetic link. The tumor triggers the release of excess catecholamines, which causes the symptoms. Certain activities or foods can trigger symptom episodes. These include physical activity, bowel movements, eating foods high in tyramine, and certain medications. Stress and anxiety may also trigger episodes in some people.
How it's diagnosed
Diagnosing pheochromocytoma starts with blood and urine tests that measure catecholamines and related substances. Your doctor may order plasma metanephrine tests or 24-hour urine collection tests. These tests check if your body is producing too much of these hormones. Blood tests for Chromogranin A can also help identify neuroendocrine tumors like pheochromocytoma.
If blood or urine tests suggest a tumor, imaging tests help locate it. CT scans and MRI can show tumors in the adrenal glands. Specialized nuclear medicine scans may also be used. Talk to a doctor if you experience symptoms like severe headaches, unexplained sweating, or sudden blood pressure spikes. They can order the appropriate specialized tests and refer you to an endocrinologist if needed.
Treatment options
- Surgery to remove the tumor is the main treatment for most cases
- Medications to block the effects of excess hormones before surgery, including alpha blockers and beta blockers
- Blood pressure medications to control hypertension during treatment
- High-salt diet and increased fluid intake before surgery to expand blood volume
- Genetic counseling and testing if you have a family history of related conditions
- Regular monitoring after surgery to watch for tumor recurrence
- Radiation therapy or chemotherapy for cancerous tumors that cannot be removed
- Stress management techniques to help reduce symptom triggers
Frequently asked questions
The classic triad includes severe headaches, heavy sweating, and rapid heartbeat. These symptoms often come in sudden episodes along with high blood pressure. Some people also experience anxiety, tremors, and pale skin during these spells.
Diagnosis involves blood and urine tests that measure catecholamines and metanephrines. Chromogranin A blood tests can also help identify the tumor. If tests are positive, imaging scans like CT or MRI locate the tumor in the adrenal glands.
Most pheochromocytomas are benign, meaning they are not cancerous. About 10 percent are malignant and can spread to other parts of the body. Even benign tumors need treatment because the hormone excess can cause serious health problems.
Yes, surgery to remove the tumor can cure most cases. After successful surgery, blood pressure and other symptoms usually return to normal. Some people need ongoing monitoring because tumors can rarely return or appear in other locations.
Physical activity, certain foods, medications, and stress can trigger symptom episodes. Foods high in tyramine, like aged cheese and cured meats, may cause spells. Bowel movements, abdominal pressure, and even certain anesthesia drugs can also trigger symptoms.
Pheochromocytoma affects about 2 to 8 people per million each year. It accounts for less than 1 percent of all high blood pressure cases. The condition is most common in people between ages 30 and 50.
Yes, excess catecholamines can cause severe anxiety and panic attack-like symptoms. Many people report feeling a sense of impending doom during episodes. These feelings are caused by the hormones, not by a mental health condition.
About 30 to 40 percent of cases are linked to inherited genetic conditions. These include multiple endocrine neoplasia type 2 and von Hippel-Lindau disease. If you are diagnosed, your doctor may recommend genetic testing and counseling for family members.
Untreated pheochromocytoma can lead to serious complications including heart attack, stroke, and kidney damage. The repeated spikes in blood pressure put strain on your heart and blood vessels. Early diagnosis and treatment prevent these dangerous outcomes.
Doctors prescribe medications called alpha blockers and beta blockers for 7 to 14 days before surgery. These drugs block the effects of excess hormones and stabilize blood pressure. You may also need a high-salt diet and extra fluids to prepare your body for the procedure.