About Pheochromocytoma
Pheochromocytoma is a neuroendocrine tumor arising from chromaffin cells, most commonly in the adrenal medulla (90%), though extra-adrenal tumors (paragangliomas) occur in 10%. The tumor produces catecholamines (epinephrine and norepinephrine) that cause the characteristic symptoms. Episodic catecholamine release causes sudden hypertensive attacks with severe headache, sweating, palpitations, and anxiety.
Attacks can be triggered by abdominal pressure, exercise, micturition, foods containing tyramine, or occur spontaneously. The rule of 10s classically applied: 10% bilateral, 10% familial, 10% malignant, 10% extra-adrenal, 10% in children. However, newer genetic and imaging data suggest higher rates of hereditary disease. Sustained hypertension or episodic hypertension with normal blood pressure between attacks both occur.
Untreated tumors increase risk of myocardial infarction, stroke, cardiomyopathy, and sudden cardiac death. Long-term catecholamine excess can cause irreversible complications. About 10% are malignant with metastatic potential, particularly SDH mutation-associated tumors. Genetic testing is recommended for all patients to identify hereditary syndromes.
Common Symptoms of Pheochromocytoma
Recognizing the signs of Pheochromocytoma early can lead to faster diagnosis and better outcomes. Symptoms may vary in severity from person to person. If you or a loved one are experiencing any of the following, consider speaking with a specialist.
- Severe hypertension, often episodic or paroxysmal
- Intense headaches during attacks
- Profuse sweating
- Palpitations and chest or abdominal pain
- Tremor, anxiety, and sense of impending doom
- Syncope (fainting) or hypertensive crisis with stroke risk
Who Pheochromocytoma Affects
Pheochromocytoma can develop at any age but is most common in the fourth and fifth decades of life. It affects males and females roughly equally. The disease occurs across all racial and ethnic groups. About 30-40% of cases are hereditary, associated with MEN2, NF1, VHL, or SDHA/B/C/D syndromes. Hereditary cases often present at younger ages and may be bilateral or extra-adrenal.
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FDA-Approved Treatments for Pheochromocytoma
There is currently 1 FDA-approved medication for Pheochromocytoma. These therapies represent the current standard of care and may be used alongside or compared against investigational treatments in active clinical trials.
Source: openFDA drug labeling data. This list may not include all treatments. Always consult your doctor.
Help Paying for Pheochromocytoma Treatment
Charity funds and drugmaker programs for Pheochromocytoma, checked at the source. Pick your insurance to see what fits.
No charity fund for this condition is open right now. Funds reopen when they receive new donations; the foundations let you sign up to be told when one opens.
Side Effect Explorer
Real-world side effect reports from the FDA Adverse Event Reporting System (FAERS). Includes both FDA-approved drugs and investigational therapies from active clinical trials. Click any drug to see what patients reported.
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Genetic Testing
Genetic testing can confirm a diagnosis, guide treatment decisions, and identify family members who may be at risk.
The gene pages for RET and NF1 list every condition Orphanet links to the gene and the open trials that name it.
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Trusted Pheochromocytoma Resources
Reputable organizations and medical references for learning more about Pheochromocytoma, including disease registries, foundation resources, and clinical guidelines.