Pheochromocytoma

What is pheochromocytoma?

A pheochromocytoma (pronounced fee-oh-kroh-moh-sy-TOH-mah) is a rare tumor that grows in the adrenal glands. The adrenal glands are two small, triangle-shaped organs that sit on top of the kidneys. Their inner core, called the adrenal medulla, produces hormones known as catecholamines — mainly adrenaline (epinephrine) and noradrenaline (norepinephrine). These are the “fight or flight” hormones that raise your heart rate and blood pressure when your body senses stress or danger.

When people ask what is pheochromocytoma, the simplest answer is this: it is a tumor of the adrenal medulla that releases too much adrenaline and noradrenaline, often in sudden bursts. Because these hormones directly control blood pressure and heart rate, the tumor can cause dramatic spikes in blood pressure and episodes of pounding heartbeat, sweating, and headache.

Most pheochromocytomas are benign, meaning they are not cancer and do not spread to other parts of the body. A smaller number are malignant (cancerous) and can spread. Even a benign pheochromocytoma, however, can be dangerous if it is not treated, because uncontrolled hormone surges can strain the heart, blood vessels, and brain over time.

Pheochromocytoma can occur at any age, but it is most often diagnosed in adults between roughly 30 and 50 years old. It affects men and women in similar numbers. A closely related tumor called a paraganglioma arises from the same type of hormone-producing tissue but grows outside the adrenal glands, for example along nerves in the chest, abdomen, or neck. Doctors often discuss the two conditions together because they behave similarly and share many causes.

Symptoms of pheochromocytoma

Pheochromocytoma symptoms come mainly from the excess adrenaline and noradrenaline the tumor releases. A hallmark of the condition is that symptoms often occur in sudden episodes, sometimes called “spells” or attacks, which may last from a few minutes to an hour and then fade. Between episodes, many people feel relatively normal.

Common symptoms include:

• High blood pressure (hypertension) — this may be constant, or it may spike sharply during episodes and be more difficult to control with standard medication.
• Severe headaches — often described as pounding and sudden in onset.
• Heavy sweating — sometimes drenching, without an obvious cause such as heat or exercise.
• Rapid or pounding heartbeat (palpitations) — a racing or fluttering feeling in the chest.
• Tremor — shakiness of the hands or body.
• Pale skin during an episode.
• Anxiety or a sense of dread — some people describe a panic-attack-like feeling.
• Nausea, abdominal pain, or constipation.
• Unintended weight loss in some cases.

The classic combination doctors watch for is episodes of headache, sweating, and rapid heartbeat together with high blood pressure. However, the picture varies widely. Some people have persistent high blood pressure without dramatic spells. Others have entirely normal blood pressure between episodes. A growing number of pheochromocytomas are found by chance, when a scan done for an unrelated reason shows a mass on the adrenal gland; some of these people have few or no symptoms at all.

Symptoms can also differ depending on which hormone the tumor mainly produces. Tumors that release mostly noradrenaline tend to cause sustained high blood pressure, while tumors that release mostly adrenaline may cause episodic spells with palpitations, anxiety, and sometimes even low blood pressure on standing. Tumors linked to certain inherited conditions can behave differently again, and some produce very little hormone.

Episodes may start without warning, or they may be triggered by physical pressure on the tumor, exercise, certain foods, some medications, anesthesia, surgery, or even emotional stress. Because the symptoms overlap with far more common problems — such as anxiety disorders, panic attacks, overactive thyroid, or ordinary hypertension — pheochromocytoma is often not the first diagnosis doctors consider, and delays in recognition are common.

Causes and risk factors

In many cases, the exact pheochromocytoma causes are not known. The tumor develops from chromaffin cells — the specialized cells in the adrenal medulla that make catecholamine hormones — but what pushes a particular cell to become a tumor is often unclear. These are called sporadic cases, meaning they occur without an obvious inherited cause.

A substantial proportion of pheochromocytomas, however, are linked to inherited genetic changes. This is one reason doctors frequently recommend genetic counseling and testing after a diagnosis. Inherited conditions associated with pheochromocytoma include:

• Multiple endocrine neoplasia type 2 (MEN2) — an inherited syndrome that also raises the risk of medullary thyroid cancer and parathyroid problems.
• Von Hippel-Lindau disease (VHL) — a condition that causes tumors and cysts in several organs, including the adrenal glands, kidneys, brain, and eyes.
• Neurofibromatosis type 1 (NF1) — a disorder that causes growths on nerve tissue and characteristic skin changes.
• Hereditary paraganglioma syndromes — caused by changes in a family of genes known as SDH (succinate dehydrogenase) genes, which increase the risk of both pheochromocytomas and paragangliomas.

Risk factors to be aware of include:

• A family history of pheochromocytoma, paraganglioma, or one of the inherited syndromes above.
• A personal history of one of these genetic conditions.
• Younger age at diagnosis, tumors in both adrenal glands, or tumors outside the adrenal glands — all of which make an inherited cause more likely.

It is important to understand that pheochromocytoma is not caused by lifestyle choices. Diet, stress, smoking, or exercise habits do not cause the tumor to form, although stress and certain foods or medications can trigger symptom episodes once a tumor is present.

Diagnosis

Pheochromocytoma diagnosis rests on two steps: first proving that the body is producing too much catecholamine hormone, and then finding where the tumor is located.

Biochemical testing

The most reliable initial tests measure metanephrines — the breakdown products of adrenaline and noradrenaline. Because tumors release hormones in bursts but break them down continuously, metanephrine levels are more consistently elevated than the hormones themselves. Doctors typically use one or both of the following:

• Plasma free metanephrines — a blood test, often drawn after you have rested lying down.
• 24-hour urine metanephrines and catecholamines — you collect all urine over a full day so the lab can measure hormone output over time.

Certain medications, foods, caffeine, and physical stress can affect these results, so your doctor may ask you to adjust medications or follow specific instructions before testing. Mildly abnormal results are sometimes repeated or followed by additional confirmatory tests.

Imaging

Once blood or urine tests suggest excess hormone production, imaging is used to locate the tumor:

• CT scan (computed tomography) — a detailed X-ray-based scan of the abdomen, usually the first imaging test.
• MRI (magnetic resonance imaging) — a scan using magnetic fields, often preferred in pregnancy, in children, or when tumors outside the adrenal gland are suspected.
• Functional (nuclear medicine) imaging — specialized scans, such as MIBG scintigraphy or certain PET scans, that use small amounts of radioactive tracer taken up by tumor tissue. These help when doctors need to check for multiple tumors, tumors outside the adrenal glands, or spread of disease.

Genetic testing

Because a meaningful share of these tumors is inherited, current practice is to offer genetic counseling and testing to most people diagnosed with pheochromocytoma. Identifying a genetic cause guides long-term follow-up for the patient and allows relatives to be screened if appropriate.

One important caution: doctors generally avoid needle biopsy of a suspected pheochromocytoma, because puncturing the tumor can trigger a dangerous surge of hormones. The diagnosis is made through hormone tests and imaging instead.

Treatment options

The mainstay of pheochromocytoma treatment is surgery to remove the tumor, but medication comes first and plays an essential safety role. Care is usually coordinated by a team that includes endocrinologists (hormone specialists), surgeons, anesthesiologists, and sometimes cancer specialists. At Acibadem, this condition is evaluated and managed through the Endocrinology & Metabolism department together with the surgical team; an overview of the hospital group’s approach is available on its pheochromocytoma treatment page.

Medication before surgery

Operating on a pheochromocytoma without preparation can be dangerous, because handling the tumor releases a flood of hormones that can cause a blood pressure crisis. To prevent this, patients typically take medications for one to two weeks or more before surgery:

• Alpha-blockers — medicines that block the effect of noradrenaline on blood vessels, lowering blood pressure and protecting against surges. These are usually started first.
• Beta-blockers — medicines that slow the heart rate, added only after alpha-blockade is established, because starting them first can paradoxically worsen blood pressure.
• Other blood pressure medicines and increased salt and fluid intake — often used to stabilize blood pressure and blood volume before the operation.

Surgery

Surgical removal of the tumor, called adrenalectomy, is the standard and usually curative treatment for benign pheochromocytoma. In many cases the operation can be done laparoscopically — through small incisions using a camera and thin instruments — which generally means less pain and a shorter recovery than open surgery. Larger tumors, tumors suspicious for cancer, or complex anatomy may require open surgery. When tumors affect both adrenal glands, surgeons may try to preserve part of the adrenal tissue so the body can still make essential steroid hormones, though this is decided case by case.

If the entire adrenal gland is removed on one side, the remaining gland usually takes over hormone production. If both glands must be removed, lifelong replacement of adrenal steroid hormones is required.

Watchful waiting and special situations

In selected situations — for example, very small tumors in older or frail patients for whom surgery poses high risk — doctors may recommend careful monitoring with medication to control blood pressure, rather than immediate surgery. This decision weighs the risks of the tumor against the risks of the operation and is made individually.

Treatment for malignant pheochromocytoma

When a pheochromocytoma is cancerous and has spread, treatment becomes more complex. Options that doctors may consider, depending on the individual case, include surgery to remove as much tumor as possible, targeted radioactive therapies (such as radiolabeled MIBG), chemotherapy, radiation therapy to specific sites, and long-term medication to control hormone effects. There is no single standard path for metastatic disease, and care is usually managed by a multidisciplinary team.

Living with pheochromocytoma and outlook

For most people with a benign pheochromocytoma that is completely removed, the outlook is generally good. Blood pressure often improves substantially after surgery, and many people are able to reduce or stop blood pressure medications, although some — particularly those who had long-standing hypertension — may continue to need treatment. No outcome can be guaranteed, and your own doctors are the best source of information about your individual situation.

Long-term follow-up is important for everyone treated for pheochromocytoma, because these tumors can recur, sometimes many years after surgery, and a small number turn out to be malignant. Follow-up usually includes periodic blood or urine metanephrine testing and, when needed, imaging. People with an inherited genetic cause typically need lifelong surveillance, and their close relatives may be offered screening.

Day to day, people living with or recovering from pheochromocytoma are often advised to:

• Monitor blood pressure at home as recommended by their care team.
• Take medications exactly as prescribed, especially in the period before surgery.
• Tell every doctor, dentist, and anesthesiologist about the diagnosis, since some medications and anesthesia require special precautions.
• Attend all scheduled follow-up visits and lab tests, even when feeling well.

Living with uncertainty — waiting for surgery, or attending years of follow-up — can be stressful. Support from family, patient organizations, and mental health professionals can help, and it is reasonable to discuss anxiety openly with your care team.

Frequently asked questions

What is pheochromocytoma in simple terms?

It is a rare tumor of the adrenal gland — a small hormone-producing organ above the kidney — that releases too much adrenaline and related hormones. This excess causes high blood pressure and episodes of headache, sweating, and a racing heartbeat. Most of these tumors are benign, but they still need treatment because the hormone surges can harm the heart and blood vessels.

Is pheochromocytoma cancer?

In the majority of cases, no — the tumor is benign, meaning it does not spread to other organs. A minority of pheochromocytomas are malignant and can spread, most often to bones, liver, lungs, or lymph nodes. Doctors cannot always tell benign from malignant tumors under the microscope, which is one reason long-term follow-up is recommended even after successful surgery.

Can pheochromocytoma be cured?

In many cases, yes. When a benign pheochromocytoma is completely removed by surgery, the condition is often cured, and hormone levels return to normal. However, tumors can occasionally come back years later, and people with inherited genetic causes have a higher chance of developing new tumors, so ongoing monitoring is generally advised rather than a one-time check.

How serious is pheochromocytoma if left untreated?

Untreated pheochromocytoma can be dangerous. Repeated or sustained surges of adrenaline and noradrenaline can lead to severe high blood pressure, heart rhythm problems, heart muscle damage, stroke, and life-threatening blood pressure crises — sometimes triggered by surgery, anesthesia, or certain medications given for other reasons. This is why doctors treat the condition even when day-to-day symptoms seem manageable.

What does a pheochromocytoma attack feel like?

People often describe a sudden episode of pounding headache, drenching sweat, a racing or hammering heartbeat, trembling, pale skin, and an intense feeling of anxiety or dread. Episodes typically last minutes to an hour and may occur unpredictably or after triggers such as physical exertion, pressure on the abdomen, or certain foods and medications. Because these spells resemble panic attacks, the condition can go unrecognized for some time.

How long is recovery after pheochromocytoma surgery?

Recovery depends on the type of operation and your overall health. After laparoscopic (keyhole) adrenalectomy, many people leave the hospital within a few days and return to normal activities over several weeks; open surgery generally takes longer. Blood pressure and hormone levels are checked after surgery, and medications are adjusted as needed. Your surgical team can give you a realistic timeline for your specific case.

Should my family members be tested?

Possibly. A significant share of pheochromocytomas is linked to inherited gene changes, so genetic counseling is usually offered after diagnosis. If testing identifies an inherited cause in you, your close relatives may be offered testing and, if they carry the same gene change, periodic screening so any tumor can be found early. A genetic counselor can explain what testing would mean for your family.

When to see a doctor

Talk to a doctor if you have repeated episodes of headache, sweating, and rapid heartbeat, blood pressure that is high or hard to control despite treatment, unexplained spells that feel like panic attacks, or a family history of pheochromocytoma or related genetic conditions. If a scan done for another reason has shown a mass on your adrenal gland, follow up with an endocrinologist even if you feel well.

Seek emergency medical care immediately if you experience any of the following red-flag signs:

• A sudden, severe headache unlike any you have had before.
• An extreme spike in blood pressure, especially with headache, blurred vision, or confusion.
• Chest pain, pressure, or severe shortness of breath.
• A very fast, irregular, or pounding heartbeat that does not settle.
• Fainting or near-fainting.
• Sudden weakness, numbness, trouble speaking, or vision loss — possible signs of stroke.

These symptoms can signal a hypertensive crisis or other serious complication and require urgent evaluation. Do not wait to see whether an episode passes on its own; emergency assessment is the safest course.