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Health condition

Paraganglioma, a rare tumour family with a genetic core.

Paragangliomas arise from paraganglia anywhere from the neck to the pelvis. Some secrete hormones, many are hereditary, and all deserve specialist care.

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A radiographer guides a patient onto the bed of an advanced 3 Tesla MRI scanner in a London imaging suite

Why trust this guide

  • 01

    Editorial team

    Written by our editorial team from the UK sources listed on each page.

  • 02

    Sourced from guidance

    Checked against ESE, ESMO and NHS specialist endocrine and head and neck standards.

  • 03

    Current for 2026

    Reflects modern practice including 68Ga-DOTATATE PET-CT, SDHx genetic panels and PRRT for metastatic disease.

Key facts

Paraganglioma at a glance.

The essentials, in plain English - what it is, where it occurs, and how it is assessed and treated in the UK.

  • What it is

    A rare neuroendocrine tumour arising from paraganglia, clusters of chromaffin-related cells linked to the autonomic nervous system.

  • Where they occur

    Head and neck (carotid body, jugular, vagal, laryngeal), the sympathetic chain in the chest, abdomen and pelvis, and the adrenal medulla - where they are called phaeochromocytoma.

  • Functioning or not

    Some secrete catecholamines and cause hypertension, palpitations and sweating; many, especially head and neck sites, are non-functioning and present as a mass.

  • Genetic component

    Up to 30 to 40 per cent are hereditary, most often from SDHx mutations, alongside VHL, NF1, MEN2, MAX and TMEM127.

  • Assessment

    Plasma or urine metanephrines, cross-sectional imaging, 68Ga-DOTATATE PET-CT and germline genetic testing, coordinated through a specialist endocrine MDT.

  • Management

    Surgery where feasible, alpha and beta blockade first if functioning, radiotherapy or embolisation for unresectable disease, and lifelong surveillance.

Looking for the carotid body specifically?

If your paraganglioma is at the carotid bifurcation in the neck, read our dedicated guide to carotid body tumour and paraganglioma, which covers the Shamblin classification, Fontaine sign and site-specific surgical planning in detail.

Why this guide matters

One tumour family, many locations.

Paraganglioma can mean very different things depending on where it sits and whether it secretes hormones. The three points below shape everything else here.

  • Location changes the presentation

    Head and neck paragangliomas usually present as a mass; sympathetic and adrenal ones more often present with catecholamine excess.

  • Genetics changes the plan

    Up to 30 to 40 per cent are hereditary, and SDHx status changes both surveillance and metastatic risk for you and your family.

  • Biochemistry comes before imaging

    Excluding a functioning tumour with metanephrines protects against a hypertensive crisis during scanning, biopsy or surgery.

How the diagnosis is made

From symptoms to a specialist plan.

The steps a UK endocrine team will normally follow, in order - so you know what to expect and why.

  1. 01

    Assessing

    History and examination

    A review of symptoms of catecholamine excess, any mass, and family history of paraganglioma or phaeochromocytoma.

  2. 02

    Assessing

    Biochemical screening

    Plasma free metanephrines or 24 hour urinary fractionated metanephrines and catecholamines, done before any imaging or intervention.

  3. 03

    Assessing

    Blood pressure profiling

    Ambulatory or home readings to characterise sustained or paroxysmal hypertension linked to a functioning tumour.

  4. 04

    Confirming

    Cross-sectional imaging

    CT or MRI of the neck, chest, abdomen and pelvis to localise the tumour and assess local invasion.

  5. 05

    Confirming

    68Ga-DOTATATE PET-CT

    The preferred functional scan for detecting somatostatin receptor expressing paragangliomas, multifocal disease and metastases.

  6. 06

    Confirming

    MIBG scintigraphy where relevant

    An alternative functional scan, particularly useful in some catecholamine-secreting tumours being considered for MIBG-based therapy.

  7. 07

    Preparing

    Genetic testing and family screening

    A germline panel for SDHx, VHL, NF1, MEN2, MAX, TMEM127 and FH, with cascade testing and surveillance for relatives who test positive.

Typical timeline: from first suspicion to an MDT plan in a few weeks in a specialist centre.

Symptoms

What paraganglioma actually feels like.

Presentation depends heavily on location and whether the tumour secretes hormones. Below are the patterns we see most often.

  • Sustained or episodic hypertension

    The commonest sign of a functioning paraganglioma, sometimes resistant to standard blood pressure medication.

  • Palpitations and tremor

    Sudden episodes of a racing heart, shaking and anxiety, often triggered by exertion, straining or abdominal pressure.

  • Sweating and flushing

    Profuse sweating, facial flushing or pallor during a catecholamine surge, frequently paired with headache.

  • A painless mass

    Non-functioning tumours, especially in the head and neck, often present simply as a slow-growing, painless lump.

  • Cranial nerve or local symptoms

    Larger head and neck paragangliomas may cause hoarseness, tongue weakness, dysphagia or facial nerve palsy.

  • Headache and anxiety

    A classic triad with palpitations and sweating, frequently misattributed to anxiety or panic disorder for years.

  • Multifocal or bilateral disease

    More than one paraganglioma, or bilateral tumours, strongly suggests an inherited SDHx or other hereditary syndrome.

  • Red flag - hypertensive crisis

    Severe, sudden hypertension, especially around anaesthesia, surgery or childbirth, can signal an undiagnosed functioning tumour.

A note on location

A pulsatile, painless neck mass mobile from side to side but not up and down (the Fontaine sign) is classic for a carotid body tumour. Sympathetic paragangliomas of the chest, abdomen or pelvis more often present with hormone-driven symptoms rather than a palpable lump.

Treatment

How paraganglioma is treated in the UK.

  • Specialist endocrine MDT

    Every case is discussed by endocrinology, surgery, oncology, radiology and clinical genetics before a plan is agreed.

  • Preoperative alpha blockade

    Phenoxybenzamine or doxazosin for one to two weeks before surgery in functioning tumours, to prevent a hypertensive crisis.

  • Beta blockade if needed

    Added only after adequate alpha blockade, to control reflex tachycardia without unopposed alpha stimulation.

  • Surgical resection

    The definitive treatment for accessible tumours, ideally at a centre experienced in paraganglioma and phaeochromocytoma surgery.

  • Preoperative embolisation

    Used for highly vascular head and neck paragangliomas to reduce intraoperative blood loss before resection.

  • External beam radiotherapy

    A well-tolerated option for unresectable, recurrent or skull-base tumours, with good long-term local control.

  • Stereotactic radiosurgery

    Gamma Knife or CyberKnife for inoperable, bilateral or multi-site paragangliomas, sparing nearby cranial nerves.

  • PRRT and systemic therapy

    Metastatic disease may be treated with 177Lu-DOTATATE, sunitinib or temozolomide within a specialist neuroendocrine tumour service.

Why alpha blockade comes first

If a paraganglioma secretes catecholamines, manipulating it during surgery, biopsy or even some imaging can trigger a sudden surge in hormones and a hypertensive crisis. Alpha blockade, usually with phenoxybenzamine or doxazosin, is started one to two weeks beforehand, with beta blockade added afterwards only if needed. This preparation is essential and non-negotiable for functioning tumours, whether the site is the carotid body, sympathetic chain or adrenal gland.

What this guide is based on

The sources behind every claim on this page.

UK national guidance and international specialist society standards, current at the time of last review.

Key references

Guidelines and standards we relied on.

A quiet reminder

This guide is for information, not medical advice.

Your endocrinologist, surgeon or clinical geneticist knows your case and can tell you which parts apply to you.

  • European Society of Endocrinology. Clinical practice guideline on phaeochromocytoma and paraganglioma.

  • ESMO Clinical Practice Guidelines. Neuroendocrine and paraganglioma tumours.

  • ENT UK and BAHNO. Head and neck paraganglioma guidance.

  • NHS England service specification for complex endocrine and head and neck tumours.

Red flags

When paraganglioma needs urgent attention.

Some features change the pace and the plan. These are the ones that shouldn't wait.

  • Suspected functioning tumour

    Episodic hypertension, palpitations, sweating or headache means biochemistry and alpha blockade before any procedure or scan with contrast.

  • Hypertensive crisis

    A sudden severe rise in blood pressure, particularly around surgery, anaesthesia or childbirth, needs urgent specialist assessment.

  • Multiple or bilateral tumours

    More than one paraganglioma strongly suggests an inherited syndrome and warrants urgent genetics referral.

  • Never biopsy blindly

    Percutaneous biopsy of a suspected paraganglioma is avoided because of the risk of catastrophic bleeding and hypertensive crisis.

  • Family history of PGL or phaeo

    Any first degree relative with paraganglioma, phaeochromocytoma or related tumours warrants genetic counselling.

  • Pregnancy with a known tumour

    Functioning paragangliomas in pregnancy carry high maternal and fetal risk and need joint obstetric and endocrine care.

  • New cranial nerve deficit

    Hoarseness, tongue weakness, facial droop or dysphagia in a head and neck paraganglioma needs urgent imaging and MDT review.

  • Rapid growth or metastatic symptoms

    Fast growth, bone pain or weight loss should trigger staging with DOTATATE PET-CT, particularly with SDHB mutations.

Living with it

A rare tumour, with a lifelong plan.

Four things that make the biggest difference over the years - the right team, genetic clarity, steady surveillance and considered treatment choices.

A quiet reminder

Small tumours are much easier to treat.

Regular biochemistry, sensible imaging follow-up and cascade family testing turn a rare diagnosis into a manageable long-term picture.

  1. 01 Team

    Stay under a specialist MDT

    Care belongs in a UK endocrine and, where relevant, head and neck or skull base centre with genetics on hand.

  2. 02 Genetics

    Take genetic testing seriously

    A germline panel guides your own surveillance and allows relatives to be screened early, when tumours are smallest.

  3. 03 Surveillance

    Commit to lifelong follow-up

    Regular biochemistry and imaging catches new or recurrent tumours before they cause harm.

  4. 04 Choices

    Weigh surgery against other options

    For some patients, radiotherapy, radiosurgery or surveillance offers a reasonable alternative to immediate surgery.

Frequently asked

Everything we get asked about paraganglioma.

Quick answers on genetics, function, alpha blockade, surgery and metastatic disease.

  • What is a paraganglioma?

    A paraganglioma is a rare neuroendocrine tumour arising from paraganglia, small clusters of cells associated with the autonomic nervous system. They can occur in the head and neck, along the sympathetic chain in the chest, abdomen and pelvis, or in the adrenal gland, where they are called phaeochromocytoma.

  • What is the difference between paraganglioma and phaeochromocytoma?

    They are closely related tumours from the same cell family. Phaeochromocytoma specifically refers to a paraganglioma arising within the adrenal medulla, while paraganglioma usually describes tumours outside the adrenal gland, such as at the carotid body, along the sympathetic chain, or in the head and neck.

  • Are all paragangliomas hormone-secreting?

    No. Sympathetic paragangliomas, including most in the abdomen, chest and adrenal gland, are often functioning and secrete catecholamines. Head and neck paragangliomas, such as carotid body tumours, are usually non-functioning and present as a mass instead.

  • Do I need genetic testing?

    Yes. Guidelines recommend germline testing for SDHx, VHL, NF1, MEN2, MAX, TMEM127 and FH in anyone with a paraganglioma or phaeochromocytoma. Up to 30 to 40 per cent of cases are hereditary, and a positive result changes surveillance for you and your family.

  • Why is preoperative alpha blockade so important?

    In a catecholamine-secreting tumour, manipulation during surgery or biopsy can trigger a sudden, dangerous release of hormones causing a hypertensive crisis. Alpha blockade, sometimes followed by beta blockade, stabilises blood pressure and heart rate beforehand and is considered essential for safe surgery.

  • What happens if a paraganglioma spreads?

    Metastatic paraganglioma is managed by a specialist neuroendocrine tumour service. Options include peptide receptor radionuclide therapy with 177Lu-DOTATATE, tyrosine kinase inhibitors such as sunitinib, chemotherapy with temozolomide, and ongoing biochemical and imaging surveillance.

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