Paragangliomas of the head and neck

Paragangliomas of the head and neck are rare tumours, representing <0.5% of all head and neck tumours.

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

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

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

One particular feature of head and neck paragangliomas is that they tend to be innervated by the parasympathetic system.

Location

They are divided according to location:

CT
  • useful when bone erosion occurs
  • moth-eaten pattern is typical
MRI
  • T1: may show a “salt and pepper” appearance; salt being blood products from haemorrhage (uncommon) and pepper being 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
Angiography
  • should demonstrate an intense tumour blush, with the most common feeding vessel being the ascending pharyngeal artery
Scintigraphy
  • will demonstrate high uptake with 111In labelled octreotide 7-8

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.

In the jugular region consider

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Article information

rID: 6744
System: Head & Neck
Section: Gamuts
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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