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.
They are divided according to location:
- carotid body tumour (or chemodectoma)
- glomus tympanicum tumour
- glomus jugulotympanicum tumour
- glomus jugulare tumour
- glomus vagale tumour
- useful when bone erosion occurs
- moth-eaten pattern is typical
- 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
- should demonstrate an intense tumour blush, with the most common feeding vessel being the ascending pharyngeal artery
- will demonstrate high uptake with 111In labelled octreotide 7-8
Treatment and prognosis
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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