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Glomus Jugulare Paraganglioma

Last Updated: September 20, 2020

Figure 1: (Top Left) CT demonstrates a typical permeative lucent appearance of this glomus jugulare tumor in the left petrous apex. These lesions tend to be low signal intensity on T1WI (top right) and hyperintense with a salt-and-pepper appearance on T2WI (bottom).

BASIC DESCRIPTION

  • Benign neuroendocrine tumor of neural crest origin arising near the jugular foramen

PATHOLOGY

  • Arises from glomus bodies, which function as chemoreceptors
    • Located within jugular bulb, cranial nerve IX (CN-IX) tympanic branch and CN-X auricular branch
  • Classically spreads through the middle ear in a superior–lateral vector

    • May involve CN-VII mastoid segment
  • Arterial supply from the ascending pharyngeal artery
  • Familial or sporadic

    • Associated with multiple endocrine neoplasia type 1 (MEN 1) syndrome, neurofibromatosis type 1 (NF-1), and multiple myocutaneous neuromas
    • Patients are at increased risk of thyroid malignancy
  • Chief and sustentacular cells within fibromuscular stroma are characteristic microscopic features
  • Neurosecretory granules on electron microscopy

CLINICAL FEATURES

  • Usually afflicts middle-aged and older adults (40–60 years old)
  • Female gender predilection (male/female ratio, 1:4)
  • Common presenting signs/symptoms
    • Pulsatile tinnitus
    • Cranial neuropathy involving CN-IX to CN-XII
  • Treatment: surgical resection and radiation; radiosurgery; ±presurgical tumor embolization

IMAGING FEATURES

  • General
    • Lobulated solid mass of variable size; often large at presentation
    • Hallmark “salt-and-pepper” MRI appearance
      • T1 hyperintense “salt” due to subacute hemorrhage; T1 hypointense “pepper” due to arterial flow voids (more commonly seen in larger tumors)
      • Adjacent bony changes: permeative-destructive
    • Involvement of middle ear common; might invade jugular vein or sigmoid sinus
  • CT

    • Soft tissue mass centered near the jugular foramen
    • Avid enhancement on contrast-enhanced CT
    • ±Adjacent permeative-destructive bony changes
  • MRI

    • T1WI: hyperintense “salt” due to subacute hemorrhage, hypointense “pepper” due to arterial flow voids (more commonly seen in larger tumors)
    • T2WI: hyperintense, hypointense flow voids (“pepper”)
    • DWI: hyperintense signal that might represent “T2 shinethrough,” hypercellularity, or increased density of axons
    • T1WI+C: avid enhancement
    • MRV: might show jugular vein and/or sigmoid sinus involvement/occlusion
  • PET/CT

    • Avid fluorodeoxyglucose (FDG) uptake, which can be useful in metastatic evaluation or evaluating treatment response

IMAGING RECOMMENDATIONS

  • MRI without and with intravenous contrast, temporal bone CT to evaluate for adjacent bony changes; consider MRV and PET/CT

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

Contributor: Rachel Seltman, MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.20

References

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Kemeny AA. Contemporary management of jugular paragangliomas (glomus tumours): microsurgery and radiosurgery. Acta Neurochir (Wien) 2009;151:419–421.

Mafee MF, Raofi B, Kumar A, 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.

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

Ramina R, Maniglia JJ, Fernandes YB, et al. Tumors of the jugular foramen: diagnosis and management. Neurosurgery 2005;57(1 Suppl):59–68; discussion 68.

Rao AB, Koeller KK, Adair CF, 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,

van den Berg R. Imaging and management of head and neck paragangliomas. Eur Radiol 2005;15:1310–1318.

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