Glomus Vagale Paraganglioma (GVP)
Last Updated: March 27, 2020
- Benign, hypervascular neuroendocrine tumor of neural crest origin
- Less common than glomus caroticum (carotid body tumor) and glomus jugulare
- 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
- 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
- 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
- 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
- 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 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
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