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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


  • 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


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