Adrenal gland and paraganglia
Carotid body paraganglioma

Author: Nat Pernick, M.D. (see Authors page)

Revised: 23 May 2018, last major update February 2005

Copyright: (c) 2002-2018,, Inc.

PubMed Search: Carotid body [title] paraganglioma

Related: Paraganglioma, general, Aorticopulmonary, Cauda equina, Jugulotympanic, Laryngeal, Pigmented
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Cite this page: Pernick, N. Carotid body paraganglioma. website. Accessed August 24th, 2019.
Definition / general
  • Most common site of extra-adrenal paraganglioma
  • Present at angle of mandible / bifurcation of common carotid artery, as slow growing painless mass
  • May represent hyperplasia due to hypoxia, particularly at high altitudes
  • Also associated with hepatic cirrhosis, Carney triad (epithelioid leiomyosarcoma, pulmonary chondroma, functioning extra-adrenal paragangliomas)
  • May cause cranial nerve palsy, dysphagia or carotid sinus syndrome (bradycardia and syncope)
  • Rarely produces catecholamines
  • Mean age 40s
    • 90% benign, 90 - 95% solitary
  • May invade locally or spread to lymph nodes or lung (must rule out multicentric tumor)
  • Adhere to carotid bifurcation, but this does not represent malignant infiltration
  • May be familial (Arch Pathol Lab Med 1990;114:1272)
Case reports
  • Excision
Gross description
  • Mean 4 cm, range 2 - 8.5 cm
Microscopic (histologic) images

Images hosted on PathOut server:

Contributed by Dr. Ayesha Azam, University Hospital Coventry & Warwickshire (UK), Case of the Week #456:







Board review question #1
The genetic mutation most commonly associated with benign paragangliomas of the head and neck is:

A. NF 1
B. RET oncogene
C. Succinate dehydrogenase (SDH) mitochondrial complex
D. THEM127 and VHL

Board review answer #1
C. SDH gene complex
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