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Glomus Vagale Paraganglioma (GVP)

Last Updated: March 27, 2020

Open Table of Contents: Glomus Vagale Paraganglioma (GVP)

Figure 1: T1WI (top row left) 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 row right) and avidly enhancing on postcontrast T1WI (bottom).

Basic Description

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

Pathology

  • Arises from glomus bodies within CN 10 nodose ganglion
  • Composed of chemoreceptor cells of neural crest origin
  • Arterial supply from the ascending pharyngeal artery
  • Familial or sporadic
    • Associated with NF-1, MEN-2, and von Hippel-Lindau (VHL), and multiple paraganglioma syndromes
    • Medullary thyroid carcinoma, adrenal pheochromocytomas, multiple paragangliomas, renal, and pancreatic tumors
  • Chief cells rests (zellballen) within fibromuscular stroma are characteristic microscopic features
  • Neurosecretory granules on electron microscopy

Clinical Features

  • 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
    • CN 9-12 neuropathy (CN 10 most commonly); vocal cord paralysis, hoarseness
  • Treatment: surgical resection versus observation; high surgical morbidity with loss of CN 10 function (vocal cord paralysis)

    • If bilateral, only one side is resected
  • Prognosis: must outweigh risks and benefits of surgery; progressive CN 10 neuropathy if untreated; rare malignant potential

Imaging Features

  • 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 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

      • T1 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

Imaging Recommendations

  • MRI without and with IV 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

References

Eriksen C, et al. Vagal paragangliomas: a report of nine cases. Am J Otolaryngol. 1991;12:278-287.

Mafee MF, et al. Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and simulating lesions. Role of MR imaging. Radiol Clin North Am. 2000;38:1059-1076.

Muhm M, et al. Diagnostic and therapeutic approaches to carotid body tumors. Review of 24 patients. Arch Surg. 1997;132:279-284.

Netterville JL, et al. Vagal paraganglioma: a review of 46 patients treated during a 20-year period. Arch Otolaryngol Head Neck Surg. 1998;124:1133-1140.

Olsen WL, et al. MR imaging of paragangliomas. AJR Am J Roentgenol. 1987;148:201-204.

Osborn AG, Salzman KL, Jhaveri MD. Diagnostic Imaging (3rd ed). Philadelphia, PA: Elsevier, 2016.

Paal E: Head and neck pathology–radiology classics: vagal paraganglioma. Head Neck Path. 2007;1:35-37.

Rao AB, et al. From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 1999;19:1605-1632.

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