Carotid Body Glomus Tumor (Glomus Caroticum; Carotid Body Paraganglioma)
Last Updated: March 27, 2020
- 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
- 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
- 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
- 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)
- Well-marginated soft tissue mass centered within carotid bifurcation
- Avid enhancement on contrast-enhanced CT
- 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
- 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).
- 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
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