Paragangliomas of the head and neck

Paragangliomas of the head and neck are rare, representing <0.5% of all head and neck tumours. They arise in a number of locations along the carotid sheath and middle ear including the carotid bifurcation, vagal ganglia, jugular bulb, and tympanic plexus.

For a general discussion of the pathology of these tumours please refer to the paraganglioma article.

Overall there is a 3:1 female predominance. Two-thirds of cases are diagnosed between the ages of 40 and 60. Approximately 25% are multicentric, and these tend to be familial.

Clinical presentation will depend on location.

When involving the middle ear cavity, the tumour may grow large and extend into the external ear: these may present with pulsatile tinnitus, cranial nerve palsies (typically IX-XI, Vernet syndrome), or conductive hearing loss. Direct otoscopic examination may reveal a retrotympanic vascular mass.

In the neck, the patient may present with a local mass. 

Paragangliomas arise from neural crest cells. In the head and neck, paragangliomas tend to be innervated by the parasympathetic system and do not secrete catecholamines and are thus termed nonchromaffin paragangliomas 10

Multiple paragangliomas (both sporadic and familial subtypes) are commonly associated with mutations of the succinate dehydrogenase subunit genes 10

Although often sporadically identified in otherwise normal individuals, paragangaliomas are seen associated with a number of systemic conditions including 10:

They are divided according to location:

  • useful when bone erosion occurs
  • a moth-eaten pattern is typical
  • T1: may show a “salt and pepper” appearance; salt representing blood products from haemorrhage (uncommon) and pepper representing flow voids due to high vascularity (common)
  • T1C+ (Gd): demonstrate rapid wash-in and wash-out (as opposed to the more slow and steady enhancement of a schwannoma) 9

Treatment is usually by excision. Preoperative endovascular embolisation is often used to reduce tumour vascularity and aid excision. Radiotherapy may be used for palliation of unresectable lesions.

Malignant transformation is not terribly uncommon and has been reported in 16-19% of glomus vagale tumours, in 6% of carotid body tumours, and in 2-4% of glomus tympanicum tumours.

When completely imaged with CT and contrast-enhanced MRI usually little differential is present. During work-up, however, numerous entities should be considered. 

In the middle ear/petrous temporal bone consider:

In the jugular and carotid region consider:

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Article information

rID: 6744
System: Head & Neck
Tag: cases
Synonyms or Alternate Spellings:
  • Head and neck paraganglioma
  • Head and neck paragangliomas
  • Paraganglioma of the head and neck

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Cases and figures

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    Figure 1: distribution of paragangliomas
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    Cb Trecon
    Case 1: carotid body tumour
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    Figure 2: histology: carotid body tumour
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