Paragangliomas of the head and neck
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Paragangliomas of the head and neck are rare, representing <0.5% of all head and neck tumors. 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 tumors 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 tumor 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, which can differentiate into cells of either the parasympathetic or sympathetic nervous system. 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:
carotid body tumor (or chemodectoma)
laryngeal/thyroid paraganglioma 11,12
arising from the laryngeal paraganglia
may grow medially into the submucosal space or laterally into the thyroid bed
useful when bone erosion occurs
a moth-eaten pattern is typical
T1: may show a “salt and pepper” appearance; salt representing blood products from hemorrhage (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
intense tumor blush
the most common feeding vessel is the ascending pharyngeal artery
high uptake on 111In labeled octreotide 7-8
Treatment and prognosis
Treatment is usually by excision. Preoperative endovascular embolization is often used to reduce tumor 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 tumors, in 6% of carotid body tumors, and in 2-4% of glomus tympanicum tumors.
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:
lymph node metastasis/mass
- 1. Rao AB, Koeller KK, Adair CF. From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 19 (6): 1605-32. Radiographics (full text) - Pubmed citation
- 2. Netterville JL, Jackson CG, Miller FR et-al. Vagal paraganglioma: a review of 46 patients treated during a 20-year period. Arch. Otolaryngol. Head Neck Surg. 1998;124 (10): 1133-40. doi:10.1001/archotol.124.10.1133 - Pubmed citation
- 3. Kollert M, Minovi AA, Draf W et-al. Cervical paragangliomas-tumor control and long-term functional results after surgery. Skull Base. 2006;16 (4): 185-91. doi:10.1055/s-2006-950386 - Free text at pubmed - Pubmed citation
- 4. Lee JH, Barich F, Karnell LH et-al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer. 2002;94 (3): 730-7. doi:10.1002/cncr.10252 - Pubmed citation
- 5. Kumar V, Abbas AK, Fausto N et-al. Robbins and Cotran pathologic basis of disease. W B Saunders Co. (2005) ISBN:0721601871. Read it at Google Books - Find it at Amazon
- 6. Swartz JD, Loevner LA. Imaging of the Temporal Bone. Thieme Medical Pub. (2008) ISBN:1588903451. Read it at Google Books - Find it at Amazon
- 7. Whiteman ML, Serafini AN, Telischi FF et-al. 111In octreotide scintigraphy in the evaluation of head and neck lesions. AJNR Am J Neuroradiol. 18 (6): 1073-80. AJNR Am J Neuroradiol (abstract) - Pubmed citation
- 8. Intenzo CM, Jabbour S, Lin HC et-al. Scintigraphic imaging of body neuroendocrine tumors. Radiographics. 27 (5): 1355-69. doi:10.1148/rg.275065729 - Pubmed citation
- 9. Prashant R, Sugoto M, Mark J. et al. Manual of Head and Neck Imaging. Springer. 2014. chapter 1, page 16
- 10. Anna Szymańska, Marcin Szymański, Elżbieta Czekajska-Chehab, Wiesław Gołąbek, Małgorzata Szczerbo-Trojanowska. Diagnosis and management of multiple paragangliomas of the head and neck. (2015) European Archives of Oto-Rhino-Laryngology. 272 (8): 1991. doi:10.1007/s00405-014-3126-z - Pubmed
- 11. Brownlee R & Shockley W. Thyroid Paraganglioma. Ann Otol Rhinol Laryngol. 1992;101(4):293-9. doi:10.1177/000348949210100402 - Pubmed
- 12. Ramadan O. Laryngeal Paraganglioma: A Review Article. Otolaryngol Open J. 2017; SE(6): S1-S10.