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

Last Updated: September 6, 2020

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

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

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

Figure 2: T1FS postcontrast (top left) and T2FS (top 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 left) performed before embolization demonstrates a hypervascular mass splaying the left internal and external carotid arteries. After embolization, a whole-body  68 Ga-DOTANOC PET/CT (bottom right) was performed to assess for additional sites of disease.  68 Ga-DOTANOC localizes to organs and tumors that express specific subtypes of somatostatin receptors (neuroendocrine tumors, carcinoid, paragangliomas, etc). The selected image demonstrates a  68 Ga-DOTANOC avid mass in the right carotid space compatible with an additional glomus caroticum paraganglioma.

Figure 2: T1FS postcontrast (top left) and T2FS (top 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 left) performed before embolization demonstrates a hypervascular mass splaying the left internal and external carotid arteries. After embolization, a whole-body 68Ga-DOTANOC PET/CT (bottom right) was performed to assess for additional sites of disease. 68Ga-DOTANOC localizes to organs and tumors that 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 are more common in familial cases
  • May also develop as a response to chronic hypoxia (chronic obstructive pulmonary disease/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) are 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” magnetic resonance imaging (MRI) appearance
      • T1 hyperintense “salt” due to subacute hemorrhage, hypointense “pepper” due to arterial flow voids (more commonly seen in larger tumors)
  • Computed Tomography (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: internal carotid artery (ICA)-external carotid artery (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 positron emission tomography (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 intravenous contrast with catheter angiography
  • Evaluate for multiple tumors
  • Imaging tumor surveillance if familial

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

Contributors: Rachel Seltman, MD, and 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. Philadelphia, PA: 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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