Pheochromocytoma: What It Is, How It Causes High Blood Pressure, and Why Surgery Is Often the Cure

• by Melinda Hawthorne
• 0 Comments

A sudden spike in blood pressure so high it feels like your head might explode. Sweating through your shirt for no reason. Your heart pounding like you just ran a marathon-even when you’re sitting still. These aren’t signs of stress or anxiety. They could be symptoms of something far more specific: a rare adrenal tumor called pheochromocytoma.

Most people with high blood pressure have what’s called essential hypertension. It’s gradual, steady, and managed with lifelong medication. But pheochromocytoma is different. It’s not just high blood pressure. It’s explosive, unpredictable, and often misdiagnosed. And here’s the good news: if caught in time, it can be cured with surgery.

What Exactly Is a Pheochromocytoma?

Pheochromocytoma is a tumor that grows in the adrenal medulla-the inner part of the adrenal glands, which sit on top of each kidney. These glands normally produce hormones like adrenaline (epinephrine) and noradrenaline (norepinephrine) to help you respond to stress. But when a pheochromocytoma forms, it starts pumping out these hormones nonstop, even when there’s no danger around.

This isn’t a normal tumor. It’s a neuroendocrine tumor, meaning it comes from nerve-related cells that make hormones. About 90% of these tumors are benign, but even benign ones can be dangerous because of the hormone floods they cause. The rest-around 10%-can spread, though confirming malignancy is tricky. Some tumors stay in the adrenal gland; others grow outside it, called paragangliomas, often near the spine or bladder.

It’s rare. Only 0.1% to 0.6% of people with high blood pressure have one. But because it’s so uncommon, most doctors see maybe one or two cases in their entire career. That’s why it’s often missed.

The Classic Triad: Headaches, Sweating, and Heart Palpitations

If you’ve ever had a panic attack, you know what it feels like to suddenly feel like you’re dying. But pheochromocytoma doesn’t feel like anxiety-it feels like your body’s alarm system is stuck on full blast.

The three most common symptoms-called the classic triad-are:

• Severe headaches (85-90% of cases)
• Profuse sweating (75-80%)
• Heart palpitations or rapid heartbeat (70-75%)

These don’t happen all the time. They come in attacks-called “spells”-that last minutes to hours. They can be triggered by:

• Physical exertion
• Emotional stress
• Being put under anesthesia
• Urinating (if the tumor is in the bladder)
• Even bending over or lifting something heavy

Other signs include high blood pressure that spikes above 180 mmHg systolic, pale skin, nausea, abdominal pain, unexplained weight loss, and panic-like anxiety. Some patients even get orthostatic hypotension-where their blood pressure crashes when they stand up-because their body’s normal blood pressure controls get messed up.

These symptoms are so dramatic, they’re often mistaken for panic disorder. One patient told her story after four years of being told she had anxiety: “My blood pressure hit 240/130 in the ER-and they still thought it was a panic attack.”

Why This Tumor Causes Such Extreme High Blood Pressure

Adrenaline and noradrenaline are the body’s natural fight-or-flight chemicals. They make your heart race, your blood vessels tighten, and your blood pressure shoot up-so you can run from danger or fight a threat.

With pheochromocytoma, your body thinks it’s under constant attack. Even when you’re asleep, the tumor releases bursts of these hormones. That’s why your blood pressure doesn’t drop at night like it should. It stays high or spikes unpredictably.

This is very different from essential hypertension, where blood pressure is consistently elevated but doesn’t spike wildly. It’s also different from other secondary causes like kidney artery blockages or excess aldosterone-those don’t cause sweating or palpitations.

The key difference? Pheochromocytoma is the only common cause of high blood pressure that can be cured by removing a tumor. Once it’s gone, 85-90% of patients no longer need blood pressure meds.

How Is It Diagnosed?

Because symptoms mimic so many other conditions, diagnosis starts with the right test-not imaging, not a scan, but a blood or urine test.

The gold standard is measuring fractionated metanephrines-the breakdown products of adrenaline and noradrenaline. Two tests are used:

• 24-hour urinary metanephrines: 96-99% sensitive
• Plasma-free metanephrines: 97% sensitive, 89-94% specific

Doctors look for levels 3 times above the normal upper limit to confirm the diagnosis. Anything less could be a false positive. In fact, about 15-20% of borderline results lead to unnecessary scans and anxiety.

Once the hormone test is positive, imaging follows. MRI or CT scans show where the tumor is. Newer scans like 68Ga-DOTATATE PET/CT are more accurate, spotting tumors that older scans miss.

But here’s the critical point: never do imaging before biochemical testing. If you scan first, you might miss the diagnosis entirely. Biochemical testing comes first-always.

Genetic Testing Is Not Optional

Up to 40% of pheochromocytoma cases are linked to inherited gene mutations. That means if you have one, your siblings, children, or parents might also be at risk.

Key genes involved include:

• SDHB
• SDHD
• VHL
• RET
• NF1

SDHB mutations are especially concerning. They’re linked to higher risk of malignant tumors and tumors outside the adrenal gland. People with this mutation need lifelong monitoring with yearly whole-body MRIs.

Even if you have no family history of the disease, you still need genetic testing. Recent studies show 25% of patients with no known family history carry a mutation. That’s why guidelines now say: test everyone.

Surgery: The Only Cure

If the diagnosis is confirmed, surgery is the next-and often only-step. The goal is to remove the tumor completely. In most cases, that’s done with a minimally invasive laparoscopic adrenalectomy.

But surgery isn’t as simple as just cutting out the tumor. If you go into the operating room without preparation, the tumor can release a flood of adrenaline during handling. That can cause a hypertensive crisis-blood pressure soaring to dangerous levels, leading to stroke, heart attack, or even death.

That’s why preoperative preparation is non-negotiable.

For 7-14 days before surgery, patients take alpha-blockers like phenoxybenzamine. This relaxes blood vessels and prevents dangerous spikes. Patients also need to drink 2-3 liters of water a day and eat a high-sodium diet (over 200 mEq/day) to expand their blood volume. These tumors cause chronic blood vessel tightening, which shrinks blood volume by 20-30%. Without volume expansion, you risk low blood pressure and shock after removal.

After surgery, most patients see their blood pressure normalize within 48 hours. Many stop all blood pressure meds within weeks.

Recovery is usually quick. One patient wrote: “My blood pressure normalized within 48 hours of surgery-off all meds after 3 weeks.”

What Happens After Surgery?

If only one adrenal gland is removed, the other one usually takes over hormone production. You won’t need lifelong steroids.

But if both glands are removed-either because of two tumors or because one was too big to leave-you’ll need lifelong hormone replacement:

• Hydrocortisone: 15-25 mg daily
• Fludrocortisone: 0.05-0.2 mg daily

Without these, you can develop adrenal insufficiency-a life-threatening condition called Addisonian crisis. One patient shared: “I developed Addisonian crisis after bilateral adrenalectomy. Now I need these meds every day.”

Even with successful surgery, some people feel fatigued for months. About 12% report chronic tiredness lasting over six months. That’s not fully understood, but it’s common enough to be expected.

What If the Tumor Is Cancerous?

Metastatic pheochromocytoma is rare but serious. Survival drops to about 50% at five years. Treatment options are limited but improving.

New therapies like 177Lu-DOTATATE (a type of peptide receptor radionuclide therapy) are showing promise. In early trials, about 65% of patients had tumor shrinkage or stabilization. This treatment targets hormone receptors on the tumor cells and delivers radiation directly to them.

Other experimental drugs like Belzutifan, originally developed for kidney cancer in VHL patients, are now being tested for pheochromocytoma. Early results in VHL-related cases look encouraging.

Why So Many People Are Misdiagnosed

It’s not that doctors aren’t smart. It’s that this tumor is rare, and its symptoms look like more common conditions.

Patients are often sent to psychiatrists for anxiety. Others get treated for migraines, heart arrhythmias, or even menopause. The average delay in diagnosis is over three years. One in four are initially told they have a mental health disorder.

That’s why if you have unexplained spells of high blood pressure, sweating, and palpitations-especially if they’re episodic-ask your doctor about metanephrines. Don’t accept anxiety as the answer unless the hormone tests are normal.

What Comes Next?

If you’ve been diagnosed with pheochromocytoma, your path is clear: biochemical testing, genetic counseling, preoperative preparation, and surgery. Most people go on to live normal, healthy lives after removal.

But even after surgery, you need follow-up. Annual blood tests for metanephrines, and for those with SDHB mutations, yearly whole-body MRIs. Recurrence is rare but possible.

And if you’re a doctor reading this: don’t dismiss unexplained hypertensive spells. Test for metanephrines before ordering expensive scans. You might just save someone’s life.

Pheochromocytoma isn’t common. But when it’s found, it’s one of the most treatable causes of high blood pressure. It’s not just a tumor-it’s a hidden trigger. And once you turn it off, your body can finally rest.