Pheochromocytoma

Introduction

If you or a family member has recently been diagnosed with a pheochromocytoma or doctors are investigating one — you are likely facing a mix of medical terms and a treatment plan that unfolds in stages. The most common question patients ask is some version of: “If there’s a tumour, why can’t it just be removed straight away?” The answer is that pheochromocytomas produce powerful stress hormones, and the body has to be prepared with medication for several weeks before surgery can be done safely.

This guide explains what pheochromocytoma is, how it is diagnosed, what the full treatment journey looks like from medical preparation through surgery and recovery and what long-term follow-up involves. It also covers paraganglioma, the closely related tumour found outside the adrenal glands, and the hereditary syndromes that can cause both. The aim is to give you a clear, realistic picture of the road ahead so the conversations with your endocrinologist and surgical team feel less unfamiliar.

What Is Pheochromocytoma?

A pheochromocytoma is a tumour that grows in the inner part of the adrenal gland, called the adrenal medulla. There are two adrenal glands, one sitting on top of each kidney. The adrenal medulla normally makes hormones called catecholamines mainly adrenaline (epinephrine) and noradrenaline (norepinephrine). These are the “fight or flight” hormones that raise your heart rate and blood pressure in moments of stress or danger.

A pheochromocytoma produces these hormones in large, uncontrolled amounts. Because the body is being flooded with stress hormones at times when there is no actual stress, the result is high blood pressure, racing heartbeats, sweating, headaches, and other symptoms that can come in episodes or be constant.

Pheochromocytomas are rare. Most are benign (non-cancerous) and are cured by surgical removal. A smaller proportion behave aggressively and can spread to other parts of the body; these are described as metastatic or malignant pheochromocytomas. Even benign tumours, however, can be dangerous if left untreated, because the hormone surges put the heart and blood vessels under severe strain.

Pheochromocytoma and Paraganglioma

The same kind of hormone-producing cells that form a pheochromocytoma also exist in clusters outside the adrenal glands, alongside the spine and in the head and neck region. When a tumour of these cells grows outside the adrenal gland, it is called a paraganglioma. Pheochromocytomas and paragangliomas are often grouped together by doctors and abbreviated as PPGL because they share biology, genetics, treatment principles, and follow-up needs. Some paragangliomas (especially in the head and neck) produce little or no hormone, while others behave just like a pheochromocytoma.

Causes and Risk Factors

A pheochromocytoma develops when cells in the adrenal medulla grow abnormally and form a tumour. Researchers do not fully understand why this happens in every case, but a great deal is now known about the genetic background of these tumours.

Hereditary (Genetic) Causes

Pheochromocytoma is one of the most genetically influenced tumours in medicine. Current estimates suggest that around one in three patients with a pheochromocytoma or paraganglioma carries an inherited gene change that contributed to the tumour. The Endocrine Society and major international guidelines recommend that all patients with a pheochromocytoma or paraganglioma be offered genetic counselling and testing, regardless of age or family history.

Several inherited syndromes are linked with pheochromocytoma, including:

• Multiple Endocrine Neoplasia type 2 (MEN2) — caused by changes in the RET gene; also linked with medullary thyroid cancer and parathyroid disease.
• Von Hippel-Lindau (VHL) disease — caused by VHL gene changes; linked with tumours of the kidney, brain, and other organs.
• Neurofibromatosis type 1 (NF1) — recognised by skin changes and nerve sheath tumours.
• Hereditary paraganglioma syndromes — caused by changes in the SDH family of genes (SDHB, SDHD, SDHC, SDHA, and SDHAF2), which can run strongly in families and are associated with paragangliomas as well as pheochromocytomas.

Knowing whether a hereditary syndrome is present matters because it changes the follow-up plan, alerts doctors to look for other tumours, and allows close relatives to be tested and monitored.

Sporadic (Non-Inherited) Cases

Many pheochromocytomas occur in people with no family history and no identified inherited gene change. These are called sporadic cases. Even in sporadic tumours, gene changes can be found within the tumour cells themselves, but they are not passed down through families.

What Does Not Cause Pheochromocytoma

Pheochromocytoma is not caused by diet, stress, lifestyle choices, or anything you did or did not do. It is a biological event in a specific group of cells. Stress and certain activities can trigger hormone release from a tumour that already exists, but they do not cause the tumour itself.

Signs and Symptoms

If you are reading this with a diagnosis or active investigation already in hand, you may already recognise many of the symptoms below. They are listed here so you understand which features were likely the clues that led to your evaluation.

The classic combination — sometimes called the “textbook triad” — is:

• Sudden, severe headaches
• Heavy sweating not linked to exercise or hot weather
• A racing or pounding heartbeat (palpitations)

Other symptoms include:

• High blood pressure — either persistent or in sudden episodes called paroxysms
• Pale skin or flushing
• Tremor (shaking)
• Anxiety or a sense of impending doom
• Nausea and abdominal or chest discomfort
• Unexplained weight loss
• High blood sugar levels

Symptoms may come on suddenly, last from a few minutes to an hour, and then settle. Between episodes, a person can feel completely normal. In some patients, symptoms are subtle, and the tumour is discovered by chance during a scan ordered for another reason — these are called “incidentalomas.” In others, the first sign is a hypertensive crisis triggered by anaesthesia, surgery, childbirth, or certain medications.

Diagnosis

Diagnosing pheochromocytoma is a two-step process. First, doctors confirm that hormone levels are too high (biochemical diagnosis). Second, they locate the tumour using imaging (anatomical diagnosis). Doing these steps in the right order matters: imaging without biochemistry can find harmless adrenal lumps that are not pheochromocytomas, and biochemistry without imaging cannot guide surgery.

Biochemical (Hormone) Testing

Major guidelines, including those from the Endocrine Society, recommend measuring metanephrines as the first-line test. Metanephrines are the breakdown products of adrenaline and noradrenaline; because they are produced continuously inside the tumour, they are more reliable than measuring the parent hormones directly.

Two tests are commonly used:

• Plasma free metanephrines — measured in a blood sample, often taken after lying down for at least 30 minutes.
• 24-hour urinary fractionated metanephrines — you collect all your urine for a full day in a container provided by the lab.

Some medications and foods can interfere with these tests, so your doctor will give you specific instructions in advance.

Imaging Studies

Once high hormone levels are confirmed, imaging locates the tumour. Common options include:

• CT (computed tomography) scan of the abdomen, often the first imaging step.
• MRI (magnetic resonance imaging), useful in younger patients, in pregnancy, and for tumours in the head and neck.
• Functional imaging — specialised nuclear medicine scans such as MIBG scintigraphy or PET scans using tracers like ⁶⁸Ga-DOTATATE or ¹⁸F-FDG. These are used when there is suspicion of multiple tumours, paragangliomas, or spread to other parts of the body.

Genetic Testing

Because hereditary syndromes are common in pheochromocytoma, genetic counselling and testing are recommended for essentially all patients with a confirmed tumour. Testing typically involves a blood sample, and the results inform follow-up planning, screening for other tumours, and testing of close family members. A genetic counsellor or endocrinologist usually guides this conversation.

Why Surgery Cannot Be Done Immediately

A natural reaction to learning you have a tumour is to want it out as soon as possible. With pheochromocytoma, that instinct has to be balanced against an important reality: operating on an unprepared patient is dangerous.

During any operation, the body is touched, moved, and exposed to anaesthetic drugs. In a person with a pheochromocytoma, these stimuli can trigger a massive release of adrenaline and noradrenaline from the tumour, sending blood pressure to dangerous heights and putting the heart at risk of severe rhythm problems, heart attack, or stroke. After the tumour is removed, the opposite problem — a sudden drop in blood pressure — can also occur.

To make surgery safer, doctors stabilise the body with medications over a period of typically seven to fourteen days, and sometimes longer, before the operation. This phase is called pre-operative medical preparation or pre-operative blockade. Endocrine Society guidelines and international expert consensus describe this preparation as a standard part of treatment, not an optional extra.

Pre-operative Medical Preparation

The goal of pre-operative preparation is to blunt the effects of the excess hormones on the heart and blood vessels, restore normal blood volume, and stabilise blood pressure and heart rate before the surgical team operates.

Alpha Blockade

The first medication started is almost always an alpha-adrenergic blocker. Common examples include phenoxybenzamine, doxazosin, prazosin, and terazosin. Alpha blockers prevent the hormones from tightening blood vessels and pushing blood pressure up. The dose is usually started low and increased gradually over days to bring blood pressure into a target range while avoiding very low pressures on standing.

Beta Blockade

A beta blocker (such as propranolol, metoprolol, or atenolol) is sometimes added a few days after the alpha blocker has been started, particularly if the heart is racing. The order matters: starting a beta blocker before adequate alpha blockade can be dangerous, because it allows blood vessels to constrict unopposed and may push blood pressure higher. Your endocrinologist will sequence these medicines carefully.

Other Medications

If blood pressure is still difficult to control, doctors may add other agents such as calcium channel blockers. In selected severe cases, a medication called metyrosine may be used to reduce hormone production directly within the tumour.

Salt and Fluid Intake

People with pheochromocytoma often have a reduced blood volume because chronically tightened blood vessels have squeezed fluid out of the circulation. After alpha blockers relax the vessels, this can show up as light-headedness on standing. To counter this, doctors typically advise a high-salt diet and generous fluid intake in the days leading up to surgery, unless there is a specific reason (such as heart failure) to avoid it. This is one of the few situations in medicine where patients are actively told to add salt.

How You Will Feel

During preparation, you may notice a stuffy nose, mild dizziness when standing up quickly, fatigue, or a slower heart rate. These are expected effects of the medications and usually settle as your body adjusts. Severe symptoms — fainting, chest pain, or breathlessness — should be reported to your medical team without delay.

Targets Before Surgery

Different teams use slightly different numbers, but the general goals before surgery include blood pressure consistently in or below the high-normal range, no severe paroxysms, a heart rate in a controlled range, and no signs of orthostatic drop that would be dangerous in theatre. Your surgical and anaesthetic team will review these targets together before scheduling the operation.

Surgical Treatment

Surgery to remove the affected adrenal gland (and the tumour with it) is called an adrenalectomy. For most pheochromocytomas, this is the definitive treatment and offers the prospect of cure.

Laparoscopic Adrenalectomy

For most adrenal tumours, doctors typically prefer a laparoscopic (keyhole) approach. The surgeon makes several small cuts in the abdomen, inserts a camera and instruments, and removes the gland through one of the openings. Compared with open surgery, the keyhole approach is usually associated with less pain, shorter hospital stay, and faster recovery. Laparoscopic adrenalectomy can be performed through the front of the abdomen (transabdominal) or through the back (posterior retroperitoneal), depending on tumour location and surgeon experience.

Robotic Adrenalectomy

Some centres offer robotic-assisted laparoscopic adrenalectomy, in which the surgeon controls the instruments through a robotic console. For appropriately selected tumours, outcomes are broadly similar to standard laparoscopy.

Open Adrenalectomy

Open surgery — a larger incision in the abdomen or flank — is still used for very large tumours, tumours that look invasive on imaging, suspected malignant tumours, or when there is involvement of nearby structures. The trade-off is a longer recovery in exchange for the wider field of view the surgeon needs in complex cases.

Partial Adrenalectomy

In some patients, especially those with hereditary syndromes and tumours in both adrenal glands, surgeons may attempt to remove only the tumour while preserving a portion of healthy adrenal tissue. This is called cortical-sparing or partial adrenalectomy. The aim is to keep enough adrenal cortex to make cortisol and aldosterone, reducing the need for lifelong steroid replacement. This is a specialist decision and is not appropriate for every patient.

Surgery for Paraganglioma

Paragangliomas are removed by surgical teams with the appropriate expertise for the location — an abdominal or vascular surgeon for tumours near the major blood vessels, and an ENT or skull-base surgeon for head-and-neck paragangliomas. The principles of pre-operative blockade still apply when the tumour produces hormones.

During the Operation

The anaesthetic team plays a critical role. They monitor blood pressure and heart rhythm continuously, manage sudden hormone surges that can still occur when the tumour is touched or its blood supply is clamped, and prepare for the drop in blood pressure that often follows tumour removal. An arterial line and central venous line are commonly used to allow second-by-second adjustments.

Recovery After Surgery

The Hospital Stay

For laparoscopic adrenalectomy, hospital stays are typically two to four days, depending on individual recovery and local practice. Open surgery usually requires a longer stay. During this time, the team watches for:

• Low blood pressure, which is common and usually managed with intravenous fluids
• Low blood sugar, which can happen as adrenaline levels fall
• Bleeding or wound issues
• Return of normal bowel function

The First Few Weeks

After discharge, most patients gradually return to normal activities over four to six weeks following laparoscopic surgery; recovery after open surgery is longer. You will usually be advised to avoid heavy lifting for several weeks and to attend a follow-up appointment to check the wound, review pathology, and plan biochemical retesting.

Steroid Replacement

If only one adrenal gland is removed, the other gland normally takes over and steroid replacement is not required long-term. If both adrenal glands have to be removed, or if the remaining cortex is not functioning, lifelong replacement with hydrocortisone (or another glucocorticoid) and sometimes fludrocortisone becomes essential. Your endocrinologist will explain how to manage doses, including the need to increase steroids during illness, fever, or injury (“sick day rules”) to prevent adrenal crisis.

Confirming Cure

Doctors typically retest metanephrines a few weeks after surgery to confirm that hormone levels have normalised. A normal result, combined with resolution of symptoms, suggests the tumour has been successfully removed. This is the start of long-term follow-up rather than the end of medical contact.

Risks and Complications

As with any major operation, adrenalectomy carries risks. With modern pre-operative preparation and experienced surgical and anaesthetic teams, serious complications are uncommon, but they are real.

Potential risks include:

• Severe blood pressure swings during and after surgery
• Bleeding, sometimes requiring transfusion
• Injury to nearby structures such as the kidney, spleen, pancreas, or large blood vessels
• Infection of the wound or chest
• Blood clots in the legs or lungs
• Adrenal insufficiency, particularly if both glands are removed or function is impaired
• Need to convert from a laparoscopic to an open operation if the surgery cannot be safely completed with keyhole technique

Risk levels depend on tumour size, whether it is benign or malignant, the patient’s overall health, and centre experience. Your surgeon will discuss your individual risk profile before consent.

Malignant and Metastatic Pheochromocytoma

Most pheochromocytomas are benign and cured by complete removal. A smaller proportion behave aggressively, with spread to lymph nodes, bones, liver, lungs, or other sites. These are described as malignant or metastatic. Importantly, the diagnosis of malignancy in PPGL is not made by looking at the tumour under the microscope; it is based on whether the tumour spreads to places where chromaffin tissue is not normally found.

Treatment of metastatic disease is individualised and often involves a multidisciplinary team. Options that doctors may consider include:

• Continued alpha and beta blockade to control symptoms
• Surgical removal of accessible deposits
• Targeted radionuclide therapy, such as ¹³¹I-MIBG or ¹⁷⁷Lu-DOTATATE, depending on tumour uptake on functional imaging
• External beam radiotherapy for painful or threatening bone deposits
• Systemic therapies including certain chemotherapy regimens and, in selected patients, targeted agents such as tyrosine kinase inhibitors

Specific gene changes — particularly in the SDHB gene — are associated with a higher risk of metastatic behaviour, which is one reason genetic testing is so valuable.

Pheochromocytoma in Children

Pheochromocytoma and paraganglioma in children are rarer than in adults but have some distinct features.

• Hereditary causes are more common. A larger proportion of paediatric PPGL is linked to inherited gene changes (VHL, SDH genes, MEN2, NF1) than in adults. Genetic counselling and testing are essential.
• Bilateral and multifocal tumours — tumours in both adrenal glands or in more than one site — are more frequent in children.
• Presentation may be subtle. Children may show persistent (rather than episodic) high blood pressure, weight loss, headaches, or poor growth. Diagnosis is sometimes delayed because the symptoms can be mistaken for other conditions.
• Surgical considerations often favour cortical-sparing approaches when both adrenals are involved, to avoid lifelong steroid replacement in a growing child where possible.
• Long-term surveillance is particularly important. Children with hereditary syndromes need lifelong follow-up planned with their paediatric endocrinology and genetics teams.

Families navigating a paediatric diagnosis benefit from care at a centre with experience in childhood endocrine tumours, where paediatric endocrinologists, surgeons, anaesthetists, and geneticists work together.

Pheochromocytoma in Pregnancy

Pheochromocytoma in pregnancy is uncommon but high-risk for both mother and baby, because the hormone surges and unrecognised hypertension can be severe. When it is diagnosed during pregnancy, care is shared between an endocrinologist, an obstetrician with high-risk pregnancy expertise, an anaesthetist, and a surgeon. The principles of alpha blockade still apply, with medication choices adjusted for pregnancy. Decisions about the timing of surgery and the mode of delivery are made on an individual basis. If you are pregnant or planning pregnancy and have a diagnosis or family history of PPGL, raise it early with your obstetric team.

Long-term Follow-up and Surveillance

Even after a successful operation and a normal post-operative hormone test, follow-up does not end. Pheochromocytomas can recur in the same area, appear in the other adrenal gland, or arise as paragangliomas elsewhere — particularly in patients with hereditary syndromes. Recurrence can also happen years or even decades later.

Current Endocrine Society guidance and international expert consensus recommend lifelong follow-up for all patients with PPGL. A typical plan includes:

• Annual measurement of plasma or urinary metanephrines, to detect recurrence early
• Blood pressure monitoring
• Periodic imaging, with frequency depending on the underlying genetics, original tumour features, and clinical picture
• Surveillance for other tumours associated with any identified hereditary syndrome (for example, thyroid in MEN2, kidney in VHL)
• Coordination with a clinical geneticist for family screening

For relatives who carry the same gene change but do not yet have a tumour, surveillance often starts in childhood or adolescence according to syndrome-specific protocols.

Living with and After Pheochromocytoma

Once hormone levels return to normal, many patients are surprised at how much better they feel — they had become used to a baseline of anxiety, palpitations, or headaches. Energy, sleep, and emotional balance often improve substantially.

Blood Pressure After Surgery

In many patients, blood pressure normalises after surgery. In others — particularly those who had long-standing hypertension before diagnosis — some blood pressure medication may still be needed long-term, because the blood vessels themselves have adapted to years of strain. Your doctor will reassess your medications in the months following surgery.

Emotional and Mental Health

Living with episodic catecholamine surges can leave behind a sense of constant alertness, anxiety, or trauma-like symptoms, even after the tumour is gone. If you find that anxiety or low mood persists, talking to your primary care doctor, a psychologist, or a counsellor is a reasonable step. Support groups — including online communities specifically for PPGL patients and families — can also help.

Medications and Foods to Be Aware Of

Before surgery, certain medications can provoke hormone release and should be used with caution. These include some anti-nausea medicines (such as metoclopramide), certain decongestants, some antidepressants, and unopposed beta blockers. Always tell any new doctor — including dentists and emergency clinicians — that you have or have had a pheochromocytoma, particularly before any operation or new prescription. After cure, the list of concerns narrows, but it is wise to keep this history visible in your records.

Family Members

If genetic testing identifies an inherited gene change, first-degree relatives (parents, siblings, children) are usually offered counselling and testing. This can feel daunting but allows tumours to be found at an early, treatable stage in relatives who carry the gene, and reassures those who do not.

When to Seek Urgent Medical Attention

Before surgery, during preparation, and even in long-term follow-up after cure, certain symptoms warrant urgent medical assessment:

• A sudden, very severe headache different from your usual pattern
• Chest pain, severe breathlessness, or fainting
• One-sided weakness, slurred speech, or vision loss (signs of possible stroke)
• A blood pressure reading dramatically higher than your usual range, especially with symptoms
• Severe vomiting, abdominal pain, or extreme weakness in someone on steroid replacement (possible adrenal crisis)

Trust your judgement. If something feels seriously wrong, seek emergency care rather than wait for a scheduled appointment.

Frequently Asked Questions

Is pheochromocytoma cancer?

Most pheochromocytomas are benign and are cured by surgical removal. A smaller proportion behave aggressively and can spread, in which case they are described as malignant or metastatic. The likelihood depends on tumour features and underlying genetics, and your endocrinology team will discuss your individual risk.

Why do I need medications for weeks before surgery?

The hormones produced by the tumour can cause dangerous blood pressure and heart rhythm changes during surgery if not blocked first. Several weeks of alpha blockade, sometimes combined with beta blockade and a high-salt diet, prepare the body so the operation can be performed safely. This is standard practice supported by international guidelines.

Will I be cured after the tumour is removed?

Many patients with a single, benign pheochromocytoma are cured after complete surgical removal. Hormone levels return to normal and most symptoms resolve. However, lifelong follow-up is still recommended because of the risk of recurrence or new tumours, particularly in hereditary cases.

Should my children and siblings be tested?

If genetic testing identifies an inherited gene change, first-degree relatives are usually offered counselling and testing. If no inherited gene change is found, family testing is generally not required, although your clinical geneticist will advise based on the family history.

Can I have children after a pheochromocytoma?

Many people go on to have healthy pregnancies after successful treatment. If you have a hereditary form, your team can discuss the implications for your children, including options such as pre-pregnancy genetic counselling. Pregnancy in someone with an active or unresected tumour is high-risk and needs specialist planning.

Will my blood pressure return to normal after surgery?

In many patients, blood pressure normalises within weeks to months after a successful operation. In those who had long-standing high blood pressure before diagnosis, some antihypertensive medication may still be needed. Your endocrinologist will gradually adjust medications based on follow-up readings.

How long will I need follow-up?

Current guidance from the Endocrine Society and other major societies is that follow-up — usually including annual hormone testing and periodic imaging — should continue for life. The exact schedule is individualised based on tumour features, genetics, and any other tumours found.

Can stress cause a pheochromocytoma?

No. Stress does not cause a pheochromocytoma to form. However, stress, vigorous exercise, certain medications, anaesthesia, and even abdominal pressure can trigger hormone release from an existing tumour, which is why it is important to be diagnosed and prepared before any planned procedure.

Conclusion

Pheochromocytoma is a rare but serious condition, and the treatment journey unfolds in clear stages: confirming the diagnosis with hormone and imaging tests, preparing the body with several weeks of carefully managed medication, removing the tumour surgically, and then committing to long-term follow-up because the same biology that produced one tumour can sometimes produce another.

The pre-operative wait can feel frustrating, but it is one of the most important parts of safe care — it is what allows surgery to be performed with confidence rather than with risk. With current diagnostic tools, well-established medical preparation protocols, experienced surgical and anaesthetic teams, and modern genetic testing, the outlook for most patients is good. Understanding each phase of the plan, asking your endocrinology and surgical teams the questions that matter to you, and keeping up with long-term follow-up are the steps that turn a frightening diagnosis into a manageable medical journey.