[Durgical](https://durgical.com/en/) › [DurgiPedia](https://durgical.com/en/durgipedia/) › [Conditions](https://durgical.com/en/conditions/) › Head and neck paraganglioma (carotid body tumour, glomus jugulare, glomus tympanicum) # Head and neck paraganglioma (carotid body tumour, glomus jugulare, glomus tympanicum) A usually benign, slow-growing, very vascular tumour of the neck or ear, felt as a lump at the angle of the jaw or heard as a pulsing sound in the ear. Updated Nov 12, 2026 On this page - The basics What it is - Who gets it - Causes - Risk factors - Pathophysiology - Classification - In ENT - Clinical Symptoms and signs - Red flags - Diagnosis - Differential diagnosis - Complications - Prognosis - Treatment Goal of treatment - Treatment - Study Test yourself - Connections Connections ## What it is A neuroendocrine tumour of the parasympathetic paraganglia of the head and neck, most often at the [carotid bifurcation](https://durgical.com/en/anatomy/carotid-bifurcation/) (carotid body tumour), the jugular foramen (glomus jugulare), the middle ear (glomus tympanicum) or along the [vagus nerve](https://durgical.com/en/anatomy/vagus-nerve/) (glomus vagale). Most are benign and slow growing but very vascular, and they can invade the skull base. See it in 3D - [Carotid bifurcation](https://durgical.com/en/anatomy/carotid-bifurcation/) - [Jugular foramen](https://durgical.com/en/anatomy/jugular-foramen/) - [Internal carotid artery](https://durgical.com/en/anatomy/internal-carotid-artery/) - [External carotid artery](https://durgical.com/en/anatomy/external-carotid-artery/) ## +12 - [Common carotid artery](https://durgical.com/en/anatomy/right-common-carotid-artery/) - [Carotid sheath](https://durgical.com/en/anatomy/carotid-sheath/) - [Internal jugular vein](https://durgical.com/en/anatomy/internal-jugular-vein/) - [Sigmoid sinus](https://durgical.com/en/anatomy/sigmoid-sinus/) - [Glossopharyngeal nerve (IX)](https://durgical.com/en/anatomy/glossopharyngeal-nerve/) - [Vagus nerve](https://durgical.com/en/anatomy/vagus-nerve/) - [Accessory nerve (XI)](https://durgical.com/en/anatomy/accessory-nerve/) - [Hypoglossal nerve (XII)](https://durgical.com/en/anatomy/hypoglossal-nerve/) - [Facial nerve (VII)](https://durgical.com/en/anatomy/facial-nerve/) - [Eardrum](https://durgical.com/en/anatomy/tympanic-membrane/) - [Temporal bones](https://durgical.com/en/anatomy/temporal-bone/) - [Cochlea](https://durgical.com/en/anatomy/cochlea/) ## Who gets it Rare (about 0.6% of head and neck tumours), most often between 40 and 60 years and more in women. Carotid body tumours are the most common; glomus jugulare is the most common tumour of the jugular foramen and glomus tympanicum the most common primary tumour of the middle ear. Chronic hypoxia favours carotid body tumours, which are more frequent at high altitude, as in Andean cities such as Bogotá. About a third are hereditary. ## Causes - Tumour of the chief (type I) cells of the paraganglia, derived from the neural crest - Germline variants of the succinate dehydrogenase genes (SDHD, SDHB, SDHC, SDHAF2) and other genes - Syndromes: hereditary paraganglioma-pheochromocytoma; less often von Hippel-Lindau disease, multiple endocrine neoplasia type 2 and neurofibromatosis type 1 - Chronic hypoxia: living at high altitude, chronic lung disease, cyanotic heart disease ## Risk factors - Family history of paraganglioma or pheochromocytoma - Pathogenic variant in an SDH gene - Living at high altitude - Chronic hypoxaemia (for example COPD) - Female sex (sporadic tumours) ## Pathophysiology The carotid body is a chemoreceptor at the carotid bifurcation that senses oxygen, carbon dioxide and pH and signals through the [glossopharyngeal nerve](https://durgical.com/en/anatomy/glossopharyngeal-nerve/). Its chief cells lie in nests (Zellballen) surrounded by S-100-positive sustentacular cells. Loss of succinate dehydrogenase makes succinate accumulate, which stabilises hypoxia-inducible factor and drives a pseudohypoxic growth programme, the same signal that chronic hypoxia produces. Because head and neck paragangliomas arise from parasympathetic paraganglia, only a few percent secrete catecholamines, unlike adrenal and abdominal tumours. They grow slowly and spread along vessels, nerves and through the skull base. ## Classification - By site: carotid body tumour (carotid bifurcation), glomus jugulare (jugular bulb), glomus tympanicum (cochlear promontory, along the tympanic branch of the glossopharyngeal nerve), glomus vagale (along the vagus nerve) - Shamblin classification of carotid body tumours: I small and easily separated from the arteries, II partly surrounding them, III encasing the carotid arteries - Fisch (A to D) and Glasscock-Jackson classifications of jugulotympanic tumours by spread into the temporal bone and skull base - Hereditary or sporadic; single or multiple; secreting or non-secreting; malignant only when metastases are present ## Symptoms and signs - Carotid body tumour: painless, slowly growing lump at the angle of the jaw that moves side to side but not up and down (Fontaine sign), sometimes pulsatile or with a bruit - Jugulotympanic tumour: pulsatile tinnitus (the most common symptom), conductive hearing loss and aural fullness - Red mass behind the [eardrum](https://durgical.com/en/anatomy/tympanic-membrane/) (rising sun sign) that blanches with positive pressure on pneumatic otoscopy (Brown sign) - Lower cranial nerve palsies (IX to XII): dysphagia, hoarseness, shoulder weakness, [tongue](https://durgical.com/en/anatomy/tongue/) deviation; sometimes facial palsy - Rare catecholamine excess: hypertension, palpitations, headache and sweating ## Red flags - A pulsatile neck mass or a red mass behind the eardrum must not be biopsied in clinic: risk of severe bleeding - New cranial nerve palsies (hoarseness, dysphagia, tongue deviation) - Hypertension, palpitations or sweating (secreting tumour or an associated pheochromocytoma: must be checked before any surgery) - Rapid growth, pain or enlarged [neck lymph nodes](https://durgical.com/en/anatomy/lymph-nodes-of-neck/) (possible metastatic disease) - Multiple tumours or young age (hereditary syndrome) ## Diagnosis **Clinical** Carotid body tumour: lateral neck mass at the bifurcation with the Fontaine sign. Jugulotympanic tumour: pulsatile tinnitus with a red retrotympanic mass and the Brown sign. Cranial nerves VII and IX to XII are examined. **Tests** Plasma free metanephrines (or urinary fractionated metanephrines) and methoxytyramine in every patient before treatment; genetic testing for SDH and related genes is offered to all patients; audiometry for ear tumours. **Imaging** Doppler ultrasound is often the first test for a neck lump. Contrast CT shows the [carotid arteries](https://durgical.com/en/anatomy/common-carotid-artery/) splayed apart (lyre sign) or erosion of the [jugular foramen](https://durgical.com/en/anatomy/jugular-foramen/) and the spread in the [temporal bone](https://durgical.com/en/anatomy/temporal-bone/); MRI shows a salt-and-pepper pattern from flow voids; CT or MR angiography maps the blood supply; functional imaging such as DOTATATE PET finds multiple tumours and metastases. ## Differential diagnosis - Vagal or other cranial nerve schwannoma - Metastatic or reactive cervical lymph node - Branchial cleft cyst - Carotid artery aneurysm - Aberrant [internal carotid artery](https://durgical.com/en/anatomy/internal-carotid-artery/) or high jugular bulb (ear) - Cholesteatoma - Meningioma of the jugular foramen ## Goal of treatment Control the tumour while preserving the carotid arteries and the lower cranial nerves; for many slow-growing tumours, especially in older patients, observation is the safest choice. ## Treatment **Follow-up** Wait-and-scan with serial MRI for small, asymptomatic or slow-growing tumours, older patients and those with multiple tumours, since many grow very slowly and treatment can injure cranial nerves. **Surgical** Surgical excision is the main curative treatment for carotid body tumours (best when small, Shamblin I or II) and for glomus tympanicum, through the [ear canal](https://durgical.com/en/anatomy/external-acoustic-meatus/) or mastoid; preoperative embolisation for selected large tumours and vascular surgery support for Shamblin III tumours. **Procedure** Stereotactic radiosurgery or fractionated radiotherapy controls growth in most jugular and vagal tumours with less cranial nerve risk than skull base surgery; also used in older or high-risk patients and for residual tumour. **Medical** For a secreting tumour, alpha-adrenergic blockade before surgery, adding a beta-blocker only after alpha-blockade; peptide receptor radionuclide therapy or chemotherapy for metastatic disease. **Follow-up** Every suspected paraganglioma is referred to a [head and neck](https://durgical.com/en/anatomy/region/head-and-neck/) or skull base surgery team; relatives of carriers of a pathogenic variant are offered genetic counselling and surveillance. ## Complications - Lower cranial nerve palsies with dysphagia, aspiration and hoarseness (from the tumour or its treatment) - Carotid injury or stroke after surgery - Baroreflex failure with labile blood pressure after bilateral carotid body surgery - First bite syndrome (pain in the parotid area with the first bites of a meal) after surgery - Conductive or sensorineural hearing loss and facial palsy - Hypertensive crisis in an unrecognised secreting tumour - Metastases (uncommon, more often with SDHB variants) ## Prognosis Most are benign with excellent long-term survival; morbidity comes from local growth and from treatment near the cranial nerves. Metastases are uncommon and more likely with SDHB variants. Hereditary cases need lifelong follow-up. ## Test yourself Think of the answer, then tap the question to see it. ## What is the classic examination finding of a carotid body tumour? A painless lump at the angle of the jaw that moves side to side but not up and down (Fontaine sign), sometimes with a bruit. ## What does imaging show at the carotid bifurcation with a carotid body tumour? A very vascular mass splaying the internal and external carotid arteries apart (lyre sign). ## A woman has pulsatile tinnitus and a red mass behind an intact eardrum that blanches with positive pressure. What is the diagnosis and what must be avoided? Glomus tympanicum (Brown sign); it must not be biopsied in clinic because it bleeds heavily. ## Which test is done before treating any head and neck paraganglioma, and why? Plasma free (or urinary) metanephrines, to detect catecholamine secretion; a secreting tumour needs alpha-blockade before beta-blockade and surgery. ## Which paraganglioma gene shows paternal (parent-of-origin) inheritance? SDHD (also SDHAF2): tumours usually appear only when the variant is inherited from the father. [Add to my cards (5)](https://durgical.app/estudiar/?add=/en/conditions/head-and-neck-paraganglioma/) ## In ENT - [Tumours of the ear: external ear, middle ear and paraganglioma](https://durgical.com/en/ent/tumours-of-the-ear-external-ear-middle-ear-and-paraganglioma/) ## Connections ## Related conditions 11 - [Hereditary paraganglioma and pheochromocytoma (SDH)](https://durgical.com/en/conditions/hereditary-paraganglioma-and-pheochromocytoma/) - [Phaeochromocytoma](https://durgical.com/en/conditions/phaeochromocytoma/) - [Multiple endocrine neoplasia (MEN 1, 2A, 2B)](https://durgical.com/en/conditions/multiple-endocrine-neoplasia/) - [Neurofibromatosis (types 1 and 2)](https://durgical.com/en/conditions/neurofibromatosis/) - [Vestibular schwannoma (acoustic neuroma)](https://durgical.com/en/conditions/vestibular-schwannoma/) - [Cholesteatoma](https://durgical.com/en/conditions/cholesteatoma/) - [Cervical lymphadenopathy](https://durgical.com/en/conditions/cervical-lymphadenopathy/) - [Thyroglossal duct cyst and branchial cleft cyst](https://durgical.com/en/conditions/thyroglossal-duct-cyst-and-branchial-cleft-cyst/) - [Vocal fold paralysis (recurrent laryngeal nerve injury)](https://durgical.com/en/conditions/vocal-fold-paralysis/) - [Hearing loss (conductive and sensorineural, including sudden hearing loss)](https://durgical.com/en/conditions/hearing-loss/) - [Meningioma](https://durgical.com/en/conditions/meningioma/) Educational information, not medical advice. For any symptom, see a health professional. Sources: [Paraganglioma](https://www.ncbi.nlm.nih.gov/books/NBK549834/) (StatPearls (NCBI Bookshelf), 2024); [Glomus Jugulare](https://www.ncbi.nlm.nih.gov/books/NBK560489/) (StatPearls (NCBI Bookshelf), 2026). [← All conditions](https://durgical.com/en/conditions/) ## **Spotted a mistake?** We are always improving for the community. Tell us what is wrong and our team will review it. Report a mistake Representative model, for educational purposes only. --- Source: https://durgical.com/en/conditions/head-and-neck-paraganglioma/ License: CC BY 4.0 (https://creativecommons.org/licenses/by/4.0/). Cite as: DurgiPedia (durgical.com), https://durgical.com/en/conditions/head-and-neck-paraganglioma/