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Glomus Vagale Paraganglioma

Last Updated: September 21, 2020

Figure 1: (Top Left) T1WI demonstrates a low-signal-intensity mass in the typical location of the vagus nerve along the carotid space. The lesion can be easily mistaken for a lymph node. As with other paragangliomas, these lesions are typically hyperintense with salt-and-pepper vascular flow voids on T2WI (top right) and avidly enhancing on postcontrast T1WI (bottom).


  • Benign, hypervascular neuroendocrine tumor of neural crest origin
  • Less common than glomus caroticum (carotid body tumor) and glomus jugulare


  • Arises from glomus bodies within cranial nerve X (CN X) nodose ganglion
  • Composed of chemoreceptor cells of neural crest origin
  • Arterial supply from the ascending pharyngeal artery
  • Familial or sporadic
    • Associated with neurofibromatosis type 1 (NF-1), multiple endocrine neoplasia type 2 (MEN-2), and von Hippel-Lindau (VHL) disease, and multiple paraganglioma syndromes
    • Medullary thyroid carcinoma, adrenal pheochromocytomas, multiple paragangliomas, and renal and pancreatic tumors
  • Chief cells rests (zellballen) within fibromuscular stroma are characteristic microscopic features
  • Neurosecretory granules on electron microscopy


  • Usually afflicts middle-aged adults (40–50 years old); younger at presentation if familial
  • Female gender predilection
  • Common presenting signs/symptoms
    • Pulsatile, painless lateral neck mass
    • CNs IV to XII neuropathy (CN X most commonly); vocal cord paralysis, hoarseness
  • Treatment: surgical resection versus observation; high surgical morbidity with loss of CN X function (vocal cord paralysis)
    • If bilateral, only one side is resected
  • Prognosis: must outweigh risks and benefits of surgery; progressive CN X neuropathy if untreated; rare malignant potential


  • General
    • Lobulated, enhancing mass centered within the nasopharyngeal/suprahyoid carotid space ~2 cm below the jugular foramen
    • Displaces the internal carotid artery anteromedially, jugular vein posterolaterally, and parapharyngeal fat anterolaterally
      • No splaying of the internal carotid artery (ICA) and internal jugular vein (IJV); splaying suggests carotid body paraganglioma
    • Single or multiple
    • Variable size
    • Right-sided position more common than left
    • Hallmark “salt-and-pepper” MRI appearance
      • T1WI hyperintense “salt” due to subacute hemorrhage, hypointense “pepper” due to arterial flow voids (more commonly seen in larger tumors)
    • ±Adjacent permeative destruction of skull base
  • CT
    • Well-marginated soft-tissue mass centered within the suprahyoid carotid space ~2 cm from the jugular foramen
    • Avid enhancement on contrast-enhanced CT
    • ±Adjacent permeative-destructive bony changes
  • MRI
    • T1WI: heterogeneous signal, ±hyperintense areas of subacute hemorrhage (“salt”) is an uncommon finding, hypointense flow voids (“pepper”)
    • T2WI: heterogeneously hyperintense, hypointense flow-voids
    • T1WI+C: avid early enhancement
    • Magnetic resonance angiogram (MRA): anteromedial displacement of internal carotid artery


  • MRI without and with intravenous contrast from base of skull to carotid bifurcation; ±CT to evaluate for adjacent bony changes
  • Evaluate for multiple tumors
  • Imaging tumor surveillance if familial

For more information, please see the corresponding chapter in Radiopaedia.

Contributor: Rachel Seltman, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.21


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