Glomus Jugulare Paraganglioma
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
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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
References
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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. doi.org/10.1148/radiographics.19.6.g99no251605.
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