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Carotid Body Glomus Tumor (Glomus Caroticum; Carotid Body Paraganglioma)

Last Updated: March 27, 2020

Open Table of Contents: Carotid Body Glomus Tumor (Glomus Caroticum; Carotid Body Paraganglioma)

Figure 1: The low signal intensity mass splaying the right internal and external carotid artery on T1WI (top row left) is in a typical location for carotid body paraganglioma. This lesion tends to be hyperintense on T2WI (top row right) containing low-signal intensity flow voids due to its high vascularity. The lesion enhances avidly on postcontrast imaging (bottom) due to this feature of hypervascularity as well.

Figure 2: T1FS Post-Contrast (top row left) and T2FS (top row right) images demonstrate an avidly enhancing mass with associated internal flow voids splaying the left internal and external carotid arteries in a patient with multiple paragangliomas. Digital subtraction angiography (bottom row left) performed prior to embolization demonstrates a hypervascular mass splaying the left internal and external carotid arteries. Following embolization a whole body 68Ga-DOTANOC PET/CT (bottom row right) was performed to assess for additional sites of disease. 68Ga-DOTANOC localizes to organs and tumors which express specific subtypes of somatostatin receptors (neuroendocrine tumors, carcinoid, paragangliomas etc). The selected image demonstrates a 68Ga-DOTANOC avid mass in the right carotid space compatible with an additional glomus caroticum paraganglioma.

Basic Description

  • Benign, hypervascular neuroendocrine tumor of neural crest origin

Pathology

  • Glomus Caroticum (GC) arise from glomus bodies within the carotid body at the carotid bifurcation
  • Composed of chemoreceptor cells of neural crest origin
  • Arterial supply from the ascending pharyngeal artery
  • Sporadic >> familial
    • 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
    • Multiple tumors more common in familial cases
  • May also develop as a response to chronic hypoxia (COPD/chronic lung disease, high altitude)
  • Chief cells rests (zellballen) and sustentacular cells 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
  • Slight male gender predilection
  • Common presenting signs/symptoms
    • Pulsatile, painless mass at the angle of the mandible with gradual enlargement
    • CN 10 and CN 12 neuropathy
    • Hormonally-active tumors (catecholamine secretion) rare: palpitations, flushing, hypertension
  • Treatment

    • Surgical resection based on Shamblin classification: tumor size and degree of contact with ICA
    • Higher classification predicts surgical morbidity (CN neuropathy)
    • ± Presurgical embolization to reduce bleeding
    • Serial imaging follow-up with smaller, asymptomatic tumors

Imaging Features

  • General
    • Lobulated, enhancing mass centered within the carotid bifurcation
    • Splays the internal and external carotid arteries
      • Internal carotid artery displaced posterolaterally
      • External carotid artery displaced anteromedially
      • Jugular vein displaced posteriorly
    • Single or multiple tumors
    • Variable size
    • 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)
  • CT

    • Well-marginated soft tissue mass centered within carotid bifurcation
    • Avid enhancement on contrast-enhanced CT
  • MRI

    • T1WI: heterogenous 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
    • MRA: ICA-ECA splaying
  • Nuclear Medicine

    • 123I-MIBG: Radiopharmaceutical localizes to catecholamine producing tumors. Sensitivity for paraganglioma 57%-78%
    • 111I-Ocrtreotide: Radiopharmaceutical localizes to tumors and tissue expressing somatostatin receptors. Sensitivity for paraganglioma 94%.
    • 68Ga-DOTANOC and 68Ga-DOTATATE PET/CT: Emerging diagnostic PET/CT agent used for detection of somatostatin expressing tumors (neuroendocrine tumors, paraganglioma etc).

Imaging Recommendations

  • Contrast-enhanced CT or MRI without and with IV contrast with catheter angiography
  • Evaluate for multiple tumors
  • Imaging tumor surveillance if familial

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

Contributor: Rachel Seltman MD, Jacob A. Eitel MD

DOI: https://doi.org/10.18791/nsatlas.v1.03.01.04

References

Arya S, et al. Carotid body tumors: objective criteria to predict the Shamblin group on MR imaging. AJNR Am J Neuroradiol. 2008;29:1349-1354.

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.

Mettler, Fred A., and Milton J. Guiberteau. Essentials of Nuclear Medicine Imaging. 6th ed., Elsevier Saunders, 2012.

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

Olsen WL, et al. MR imaging of paragangliomas. AJR Am J Roentgenol. 1987;148:201-104.

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

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;19:1605-1032.

Rippe DJ, et al. Carotid body tumor: flow sensitive pulse sequences and MR angiography. J Comput Assist Tomogr. 1989;13:874-877.

Thabet MH, et al. Cervical paragangliomas: diagnosis, management and complications. J Laryngol Otol. 2001;115:467-474.

Wang SJ, et al. Surgical management of carotid body tumors. Otolaryngol Head Neck Surg. 2000;123:202-206.

Wieneke JA, et al. Paraganglioma: carotid body tumor. Head Neck Pathol. 2009;3:303-306.

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