Pheochromocytoma · Paraganglioma · Adrenaline-producing tumors
Spells of pounding heart and sweats? Rule this out.
A pheochromocytoma is a rare tumor of the adrenal medulla that releases adrenaline and noradrenaline in surges. It causes episodes of headache, sweating and palpitations with blood pressure spikes, and it can be dangerous if missed before surgery or pregnancy. It is also highly curable once found. Dr. Hasan is experienced in testing for, diagnosing and coordinating treatment of pheochromocytoma and paraganglioma.
Recognizing it
Pheochromocytomas arise in the adrenal glands; paragangliomas are the same tumor type outside the adrenals, along the sympathetic chain or in the head and neck. Together they are found in about 0.5 percent of people with hypertension and roughly 5 percent of adrenal nodules found by chance. Up to 40 percent are hereditary, so genetic testing is now recommended for everyone diagnosed.
The classic spell, and what else to look for
- 1Pounding headache; anxiety or a sense of doom during spells
- 2Palpitations, racing heart, blood pressure surges (sustained or episodic)
- 3Drenching sweats; pallor during an attack, flushing afterward
- 4Tremor
- 5Weight loss, constipation, high blood sugar
- 6Adrenal mass, often bright on T2 MRI
- 7Chest pain; in severe cases stress cardiomyopathy
Spells typically last 15 to 60 minutes and may be triggered by exertion, bending, certain medications or anesthesia. Between spells many people feel normal.
Diagnosis and treatment
Testing is biochemical first. Plasma free metanephrines drawn after 30 minutes lying down, or a 24-hour urine collection for fractionated metanephrines, detect nearly all tumors. Several medications and situations raise metanephrines falsely, including tricyclic antidepressants, some decongestants, acute illness and sleep apnea, so interpretation matters. Imaging follows a clearly positive result; functional imaging (Ga-68 DOTATATE PET) is added for large, extra-adrenal or hereditary tumors.
Starting a beta-blocker before an alpha-blocker can provoke a hypertensive crisis, which is why preparation is done by an endocrinologist.
Dr. Hasan is experienced in evaluating suspected pheochromocytoma and paraganglioma, interpreting borderline metanephrine results, preoperative alpha-blockade, and lifelong surveillance including hereditary syndromes such as MEN2, VHL and SDH mutations.
Common questions
My metanephrines are mildly elevated. Do I have a tumor?
Usually not. Levels under twice the upper limit are most often from medications, stress or how the sample was drawn. A repeat under standardized conditions, or a clonidine suppression test, usually settles it.
Is pheochromocytoma cancer?
Most are benign, but about 10 percent (more in paragangliomas and certain gene mutations) can spread. Because no pathology feature reliably predicts this, everyone is followed with yearly testing after surgery.
Why is surgery urgent?
Unrecognized pheochromocytoma can cause a dangerous blood pressure crisis during anesthesia, labor, or with certain medications. Once prepared with alpha-blockade, surgery is safe and usually curative.
Should my family be tested?
If a hereditary mutation is found, first-degree relatives are offered genetic testing and screening. Many hereditary tumors are caught early this way.
Related conditions
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