---
title: Phaeochromocytoma and paraganglioma
description: What phaeochromocytoma and paraganglioma are, why too much adrenaline and noradrenaline causes attacks of high blood pressure, headache, sweating and palpitations, why this is dangerous for the heart, how the tumour is found and treated, and when to call an ambulance.
type: disease
aliases:
  - phaeochromocytoma
  - pheochromocytoma
  - paraganglioma
  - adrenal phaeochromocytoma
  - catecholamine-secreting tumour
sources:
  - tier: guideline
    org: minzdrav-rf
    title: Артериальная гипертензия у взрослых
    year: 2024
    doi: 10.15829/1560-4071-2024-6117
    url: https://cr.minzdrav.gov.ru/view-cr/62_3
  - tier: guideline
    org: minzdrav-rf
    title: Нейроэндокринные опухоли
    year: 2025
    url: https://cr.minzdrav.gov.ru/view-cr/610_2
  - tier: guideline
    org: esc
    title: 2024 ESC Guidelines for the management of elevated blood pressure and hypertension
    year: 2024
    doi: 10.1093/eurheartj/ehae178
    url: https://doi.org/10.1093/eurheartj/ehae178
  - tier: guideline
    org: nice
    title: 'Hypertension in adults: diagnosis and management (NG136)'
    year: 2026
    url: https://www.nice.org.uk/guidance/ng136
  - tier: reference
    org: msd-manuals
    title: Pheochromocytoma (MSD Manual Professional Version)
    year: 2026
    url: https://www.msdmanuals.com/professional/endocrine-and-metabolic-disorders/adrenal-disorders/pheochromocytoma
  - tier: reference
    org: msd-manuals
    title: Pheochromocytoma (MSD Manual Consumer Version)
    year: 2026
    url: https://www.msdmanuals.com/home/hormonal-and-metabolic-disorders/adrenal-gland-disorders/pheochromocytoma
  - tier: reference
    org: statpearls
    title: Pheochromocytoma
    year: 2024
    url: https://pubmed.ncbi.nlm.nih.gov/36944004/
  - tier: reference
    org: statpearls
    title: Paraganglioma
    year: 2024
    url: https://pubmed.ncbi.nlm.nih.gov/31751024/
sources_checked: 2026-10-14
review: null
---

Phaeochromocytoma is a tumour of chromaffin cells that usually sits in the adrenal gland and releases catecholamines: adrenaline, noradrenaline and dopamine.<Cite n={[5, 7]} /> The same kind of tumour outside the adrenal gland is called a paraganglioma.<Cite n={[7, 8]} /> These hormones sharply raise blood pressure and speed up the pulse, so phaeochromocytoma is one of the causes of secondary hypertension, that is, high blood pressure with a specific cause.<Cite n={[1, 3, 6, 7]} /> For high blood pressure in general, see the article on [hypertension](../hypertension/); for another hormonal cause, see the article on [primary aldosteronism](../primary-aldosteronism/).

The tumour is rare and often overlooked, yet it is important to find: removing it completely and in time often means a cure.<Cite n={[7, 8]} />

<Diagram name="pheochromocytoma">
  <p slot="normal">
    The diagram shows a kidney with the adrenal gland on top of it, the heart and an artery. The
    adrenal gland and nerve tissue make adrenaline and noradrenaline: these hormones help keep the
    body's internal balance and respond to stress.<Cite n={7} />
  </p>
  <p slot="disease">
    In phaeochromocytoma a tumour of the adrenal gland releases too much adrenaline and
    noradrenaline, often in surges.<Cite n={[5, 6]} /> Even a small tumour can release large amounts
    of them.<Cite n={6} /> As a result, blood pressure is either raised all the time or shoots up in
    attacks.<Cite n={5} />
  </p>
</Diagram>

## Why it happens

<Patient>

Phaeochromocytoma grows from chromaffin cells, most often in the inner part of the adrenal gland, the medulla.<Cite n={[5, 6, 7]} /> Paragangliomas arise outside the adrenal glands, along the chain of nerve ganglia from the base of the skull to the bladder; tumours in the neck and at the base of the skull usually do not release hormones.<Cite n={8} />

Many cases are linked to inherited conditions: multiple endocrine neoplasia type 2, von Hippel–Lindau disease, neurofibromatosis type 1 and others.<Cite n={[2, 6, 7]} />

Usually the tumour does not spread, but it can form metastases; this is more common with paragangliomas and large tumours.<Cite n={[2, 5, 7]} />

</Patient>

<Student>

Phaeochromocytoma (PCC) is a catecholamine-secreting tumour of chromaffin cells, usually of the adrenal medulla; tumours arising from extra-adrenal chromaffin cells are called paragangliomas (PGL).<Cite n={[5, 7]} /> Sympathetic paragangliomas most commonly arise at the junction of the left renal vein and the inferior vena cava or at the aortic bifurcation (the organ of Zuckerkandl); parasympathetic ones are located mainly in the neck and skull base and are usually hormonally inactive.<Cite n={8} />

According to MSD, phaeochromocytoma occurs at any age with a peak at 20–40 years, and adrenal tumours occur equally in men and women.<Cite n={5} /> Associated hereditary syndromes include MEN2 (Sipple syndrome), von Hippel–Lindau disease, NF1 and mutations in the succinate dehydrogenase (SDH) genes.<Cite n={[2, 5, 8]} /> According to the Russian Ministry of Health guideline on neuroendocrine tumours, every PCC, like any neuroendocrine tumour, has metastatic potential: the former division into benign and malignant has been revised in favour of risk stratification.<Cite n={2} />

</Student>

## Symptoms

<Patient>

The main sign is high blood pressure, sometimes very high.<Cite n={[5, 6]} /> In some people it is raised all the time, in others it shoots up in attacks.<Cite n={5} /> During an attack there is usually a severe headache, heavy sweating and palpitations; blood pressure swings, and on standing up it may drop, causing light-headedness.<Cite n={[1, 6]} />

Other possible symptoms are pallor, cold and clammy skin, rapid breathing, chest and stomach pain, nausea and vomiting, vision problems, tingling fingers, constipation, anxiety or an odd sense of impending doom.<Cite n={[1, 6]} /> A sudden attack can feel like a panic attack.<Cite n={6} /> It can be triggered by pressure on the abdomen or massage, a change in body position, anaesthesia, emotional shock, some medicines and, rarely, passing urine.<Cite n={[5, 6]} />

Many people have similar symptoms because of anxiety, with no tumour at all.<Cite n={6} /> Conversely, almost half of people with phaeochromocytoma have nothing but persistently high blood pressure.<Cite n={6} />

Too much of these hormones is dangerous for the heart: a fast pulse, heart rhythm disturbances, angina and sometimes a specific disease of the heart muscle, catecholamine cardiomyopathy, can occur.<Cite n={[2, 5]} /> Complications affecting the heart and the blood vessels of the brain are more frequent in phaeochromocytoma than in people with the same blood pressure without a tumour.<Cite n={5} />

</Patient>

<Student>

According to MSD, hypertension occurs in most patients and is paroxysmal in 45%; common signs are tachycardia, diaphoresis, postural hypotension, tachypnoea, severe headache, angina, palpitations, epigastric pain, dyspnoea and paraesthesias.<Cite n={5} /> The Russian Ministry of Health hypertension guideline lists drugs that can provoke rises in blood pressure, including beta-blockers, sympathomimetics, opioids and non-selective monoamine reuptake inhibitors.<Cite n={1} />

Retinopathy and cardiomegaly are often less severe than would be expected for the degree of hypertension, but a specific catecholamine cardiomyopathy can occur.<Cite n={5} /> Catecholamines act directly on insulin release, so hyperglycaemia, glycosuria or overt diabetes mellitus may be present; in older patients, severe weight loss with persistent hypertension suggests phaeochromocytoma.<Cite n={5} />

</Student>

## How it is diagnosed

<Patient>

A doctor will suspect phaeochromocytoma with repeated attacks of sweating, headache or palpitations, with high blood pressure that starts suddenly, is severe or responds poorly to treatment, especially in young people, if relatives have had such a tumour, or if a CT scan has found, by chance, a growth in the adrenal gland with certain features.<Cite n={[1, 2, 5]} />

The main test measures metanephrines, the breakdown products of adrenaline and noradrenaline, in the blood or urine.<Cite n={[1, 2, 3, 5]} /> The result of a urine test can be affected by severe stress, sleep apnoea and some medicines, including some antidepressants, so the doctor interprets it in the light of treatment.<Cite n={5} /> Do not stop your medicines on your own before the test.

If the test shows too many hormones, the tumour is located with CT or MRI; sometimes a scan with a radioactive substance that builds up in the tumour is added.<Cite n={[2, 5, 6]} /> Genetic testing is recommended for everyone with this tumour: it helps plan follow-up, find other tumours and check relatives in time.<Cite n={[1, 5, 6]} />

</Patient>

<Student>

Among the reasons to look for secondary hypertension, the Russian Ministry of Health hypertension guideline names sudden onset of hypertension after documented normal blood pressure, resistant hypertension, hypertensive crisis, and symptoms suggesting phaeochromocytoma or a family history of it.<Cite n={1} /> The 2024 ESC guidelines recommend comprehensive screening for the main causes of secondary hypertension in adults diagnosed with hypertension before the age of 40, except for obese young adults, in whom an obstructive sleep apnoea evaluation comes first (I B).<Cite n={3} /> According to the Ministry's neuroendocrine tumour guideline, PCC should also be suspected in an adrenal incidentaloma with high unenhanced CT attenuation, in a personal or family history of PCC/PGL and in features of associated hereditary syndromes.<Cite n={2} />

The screening test is plasma or 24-hour urinary metanephrine and normetanephrine.<Cite n={[1, 2, 3]} /> Plasma free metanephrines are more sensitive than measuring circulating adrenaline and noradrenaline because metanephrines are elevated continuously, whereas adrenaline and noradrenaline are secreted intermittently.<Cite n={5} /> False-positive urine results occur with extreme stress, sleep apnoea and some antidepressants and monoamine oxidase inhibitors.<Cite n={5} />

To assess the extent of the tumour, the Ministry's neuroendocrine tumour guideline recommends contrast-enhanced CT of the chest, abdomen and pelvis; to localise the tumour, MSD recommends CT and MRI of the chest and abdomen, as well as functional imaging with radiopharmaceuticals (PET, scintigraphy).<Cite n={[2, 5]} /> The Ministry's hypertension guideline recommends routine genetic testing in all phaeochromocytomas and paragangliomas; the ESC advises that genetic testing should be considered in specialist centres (IIa B).<Cite n={[1, 3]} />

</Student>

## How it is treated

<Patient>

The main treatment is surgery to remove the tumour; most often it is done through small incisions, endoscopically or laparoscopically.<Cite n={[2, 5, 6]} /> Large tumours and paragangliomas may need open surgery.<Cite n={2} />

Before surgery, preparation with medicines is almost always needed: high hormone levels are dangerous during the operation.<Cite n={[2, 6]} /> Alpha-blockers are started first, and only then, if the pulse is fast, beta-blockers are added.<Cite n={[2, 5]} /> In phaeochromocytoma, beta-blockers without alpha-blockers are dangerous: they can raise blood pressure even further.<Cite n={[5, 6]} />

After surgery, metanephrines are checked and follow-up with a doctor continues for a long time: the tumour can come back, or a new one can appear.<Cite n={[1, 2]} /> If the tumour has spread or cannot be removed, blood pressure and symptoms are controlled with alpha- and beta-blockers, and the disease often progresses slowly.<Cite n={[2, 5]} /> Medicines are chosen and prescribed by a doctor; do not start, stop or change them on your own.

</Patient>

<Student>

Russian Ministry of Health, neuroendocrine tumour guideline: preoperative preparation with alpha-blockers is recommended for all patients with PCC/PGL, with beta-blockers added for tachycardia once alpha-blockade is effective; the exceptions are hormonally inactive head and neck tumours and normotensive patients without tachycardia or hypovolaemia.<Cite n={2} /> The criteria of adequate preparation are normal blood pressure, target heart rate and correction of catecholamine-induced hypovolaemia.<Cite n={2} /> The Ministry's hypertension guideline names alpha-1-blockers as first-choice drugs to prevent life-threatening perioperative cardiovascular complications.<Cite n={1} />

For PCC, endoscopic adrenalectomy is recommended, with an open approach preferred for large and invasive tumours; for PGL an open approach is recommended, and laparoscopy for small PGL is possible in leading specialist centres.<Cite n={2} /> After surgery, plasma or urinary metanephrines are measured and the patient is followed up long term to detect recurrence, metastases or a new tumour.<Cite n={[1, 2]} /> Metastatic PCC is often indolent, and active surveillance can be considered in asymptomatic patients with limited disease.<Cite n={2} />

</Student>

## When to call an ambulance

In phaeochromocytoma, a sudden rise in blood pressure with organ damage counts as a hypertensive crisis, and a hypertensive crisis in phaeochromocytoma is an indication for emergency hospital admission.<Cite n={1} /> The signs of a crisis are described in the article on [hypertensive crisis](../hypertensive-crisis/); if they appear, call your local emergency number (112 in the EU, 911 in the US and Canada, 999 in the UK).

If attacks of headache, sweating and palpitations with a rise in blood pressure keep coming back but there are no signs of a crisis, do not put off seeing a doctor: the UK NICE guideline advises same-day specialist assessment for suspected phaeochromocytoma.<Cite n={4} />

As with any heart or blood vessel condition, call an ambulance for fainting, chest pain or severe breathlessness: this is the site's general safety rule, repeated at the bottom of every page.

<Quiz />