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Pheochromocytoma

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In one line

·A tumor of chromaffin cells (usually in the adrenal gland) dumps bursts of adrenaline and noradrenaline into the blood.

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Normal physiology

·Chromaffin cells live inside the adrenal medulla (the inner core of each adrenal gland, which sits on top of each kidney) and in a few spots along the spine called paraganglia. Their job is to make and release adrenaline (epinephrine) and noradrenaline (norepinephrine) when the sympathetic nervous system tells them to—like when you are scared, hurt, or need a burst of energy.

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What goes wrong

·A tumor grows from chromaffin cells and starts making adrenaline and noradrenaline on its own, ignoring the brain's normal on-off switch. The tumor dumps these chemicals in huge surges (or a constant leak), flooding the body with a fake emergency signal even when the person is resting.

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Hallmark signs

·Sudden episodes of pounding headache
·Heavy sweating that comes in waves
·Fast, pounding heartbeat (palpitations)
·Sudden spikes in blood pressure (hypertensive crisis)
·Pale skin during an attack
·Shaking or tremor
·A family history of pheochromocytoma or related tumors
·Finding a lump (mass) on the adrenal gland by accident on a scan (adrenal incidentaloma)

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Red flags · escalate now

·Blood pressure spiking suddenly to dangerously high levels (hypertensive crisis), which can cause stroke or heart attack
·Chest pain or trouble breathing during an attack, suggesting the heart is being overworked
·Severe headache with confusion, vision changes, or weakness, which can mean bleeding in the brain from very high pressure
·Episodes triggered by pressing on the belly, surgery, or certain medicines (beta blockers alone), which can provoke a life-threatening surge

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Workup

·Plasma free metanephrines (blood test drawn after the patient has rested lying down for 30 minutes)
·24-hour urine collection for fractionated metanephrines and catecholamines
·CT scan of the abdomen and pelvis with contrast, or MRI if CT contrast is risky
·Genetic testing panel for MEN2, von Hippel-Lindau, NF1, and SDH mutations
·Functional imaging with ¹²³I-MIBG scan or ⁶⁸Ga-DOTATATE PET scan

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Treatment

·Alpha-blocker (phenoxybenzamine 10 mg twice daily, increased every 2–3 days to 20–40 mg twice daily, or doxazosin 2–8 mg daily) started at least 7–14 days before surgery
·Beta-blocker (propranolol 10–40 mg three times daily or metoprolol 25–100 mg twice daily) started only AFTER alpha-blockade is working well
·Surgical removal of the tumor (laparoscopic adrenalectomy for small single tumors under 6 cm; open surgery for larger, bilateral, or malignant tumors)
·Generous IV normal saline (1–2 liters before surgery and continued after tumor removal) to expand blood volume
·Calcium channel blocker (nifedipine extended-release 30–90 mg daily or amlodipine 5–10 mg daily) added if blood pressure stays high despite alpha- and beta-blockade
·Lifelong annual biochemical screening (plasma or urine metanephrines) and imaging every 1–2 years after surgery

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NCLEX trap

·Always block alpha receptors first with phenoxybenzamine or doxazosin. If you block only beta receptors, the alpha receptors stay wide open — blood vessels clamp down even harder, and pressure can skyrocket dangerously. Alpha first, beta second.
·When headache, sweating, pounding heart, and high blood pressure all happen together in spells, think pheochromocytoma — a tumor pouring out adrenaline-like chemicals — not a mental health problem. Always test urine or blood for metanephrines (breakdown products of those chemicals) before calling it anxiety.
·Any adrenal mass found by surprise (an incidentaloma) needs biochemical testing for pheochromocytoma. Even a small tumor can dump enough adrenaline to cause a stroke or heart attack. Size does not predict danger here — test every one.
·You must block alpha and beta receptors for 7 to 14 days before surgery. An unblocked patient can have a catecholamine storm (a huge surge of adrenaline-like chemicals) when the surgeon touches the tumor. That storm can kill. Blockade is not optional — it saves lives.
·High blood pressure that will not go down with usual drugs, plus spells of headache and sweating, plus a family history of certain tumors should make you think pheochromocytoma immediately. Get metanephrine testing before you start routine blood pressure medicines.
·Order genetic testing on every pheochromocytoma patient. At least 30 out of 100 carry a gene mutation that runs in families (MEN2, von Hippel-Lindau, neurofibromatosis type 1, SDH mutations). Family members need screening too. Catch it early.

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