Imaging of Head and Neck Glomus Tumors (Paragangliomas) 

Updated: Feb 26, 2017
  • Author: Robert A Koenigsberg, MSc, DO, FAOCR; Chief Editor: L Gill Naul, MD  more...
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Practice Essentials

Imaging is the primary investigative modality for glomus tumors of the head and neck (paragangliomas). A combination of contrast-enhanced computed tomography (CT) scanning, magnetic resonance imaging (MRI), and angiography is ideal for proper diagnosis and localization of the tumors. Lesions show a characteristic signature on the images, which is based on its location. [1, 2, 3, 4, 5, 6, 7, 8, 9]

Glomus tumors are displayed in the images below.

Glomus jugulare tumor. Computed tomography scan de Glomus jugulare tumor. Computed tomography scan demonstrates a permeative destructive skull-base mass with involvement of the mastoid air cells. Reprinted with permission. Copyright Springer-Verlag.
Glomus jugulare tumor. Selective external carotid Glomus jugulare tumor. Selective external carotid angiogram demonstrates a vascular skull-base mass. Reprinted with permission. Copyright Springer-Verlag.
Glomus jugulare tumor. Axial contrast-enhanced T1- Glomus jugulare tumor. Axial contrast-enhanced T1-weighted magnetic resonance image shows an enhancing right skull-base mass. Reprinted with permission. Copyright Springer-Verlag.

See 10 Patients with Neck Masses: Identifying Malignant versus Benign, a Critical Images slideshow, to help identify and manage several types of masses.

Currently, MRI is frequently the imaging study of choice for primary diagnosis, followed by contrast-enhanced CT imaging. Angiography remains of paramount importance if the diagnosis is obscure or if embolization is contemplated. [10, 11, 12]

Glomus tumors (paragangliomas) represent 0.6% of neoplasms of the head and neck and 0.03% of all neoplasms; they are the most common tumors of the inner ear and the second most common tumors of the temporal bone after schwannomas. About 80% of all glomus tumors are carotid body tumors or glomus jugulare tumors. [13, 14, 15, 8, 16, 17, 18]

Haller introduced glomus tumors of the head and neck into the medical record in 1762 when he described a mass at the carotid bifurcation that had a glomus body–like structure. In 1950, Mulligan renamed this type of neoplasm as a chemodectoma to reflect its origins from chemoreceptor cells. In 1974, Glenner and Grimley renamed the tumor paraganglioma on the basis of its anatomic and physiologic characteristics. They also created a classification method based on the location, innervation, and microscopic appearance of the tumors. [19]

Anatomy

Glomus tumors of the head and neck are associated with 4 primary locations, the jugular bulb, middle ear cavity, vagus nerve, and carotid body. They are highly vascular, locally invasive, slow-growing tumors that frequently involve critical neurovascular structures. [8, 16]

Tumors in the jugular bulb region are commonly called glomus jugulare tumors [20, 21, 22, 23, 24, 25] ; they arise in the adventitia of the dome of the jugular bulb. This is the most common type of glomus tumor of the head and neck. [20, 21, 17]

Tumors in the area of the middle ear cavity are commonly called glomus tympanicum tumors(see the image below) [25, 26, 27, 28, 2] ; they arise from the glomus bodies that run with the tympanic branch of the glossopharyngeal nerve. Although glomus tympanicum tumors are the most common primary neoplasms of the middle ear, these tumors are the rarest of head and neck glomus tumors. [26, 13, 29, 9]

Glomus tympanicum tumor. Axial computed tomography Glomus tympanicum tumor. Axial computed tomography image shows a small vascular mass along the right tympanic membrane. Note the adjacent opacified mastoid air cells. Reprinted with permission. Copyright Springer-Verlag.

Tumors in region of the vagus nerve are commonly called glomus vagale tumors because of their usual close association with the vagus nerve (see the image below). [30, 31, 14, 32] Specifically, they arise infratemporally along the course of the cervical vagus nerve.

Glomus vagale tumor. Contrast-enhanced computed to Glomus vagale tumor. Contrast-enhanced computed tomography scan demonstrating a large vascular mass along the course of the left internal carotid artery and jugular vein above the level of the carotid bifurcation. Reprinted with permission. Copyright Springer-Verlag.

Carotid body glomus tumors, also called carotid body tumors, occur at the bifurcation of the common carotid artery and arise from the tissue of the normal carotid body (see the image below). [13, 14, 33, 15]

Carotid body tumor. Computed tomography scan demon Carotid body tumor. Computed tomography scan demonstrates an enhancing carotid bifurcation mass. Reprinted with permission. Copyright Springer-Verlag.

Although glomus tumors usually appear as solitary lesions at 1 site, multiple lesions at multiple sites are not uncommon (see the image below). Because they are parts of the neuroendocrine system, these tumors are highly vascularized. Clusters of tumor cells (type I cells interspersed with type II cells), called zellballen, are surrounded by a dense network of capillary caliber blood vessels.

Multiple glomus tumors. Angiogram obtained in a fe Multiple glomus tumors. Angiogram obtained in a female patient with bilateral carotid body tumors, bilateral glomus vagale tumors, and left glomus jugulare tumors with corresponding angiographically enhancing masses. Reprinted with permission. Copyright Springer-Verlag.

Limitations of techniques

CT imaging is excellent at demonstrating cervical masses along the course of the carotid artery, but findings of skull-base soft-tissue details can be limited. However, CT imaging is superb for demonstrating characteristic bony destructive skull-base changes. CT scanning is also best in the diagnosis of glomus tumors when a satisfactory bolus of contrast material is administered. If peak tumor opacification is missed at CT scanning, the mass can be misconstrued for a nonenhancing schwannoma or nodal lesion. [10, 11, 12]

MRI can demonstrate soft-tissue masses and their relationships to adjacent structures well in multiple imaging planes. This capability is particularly helpful in skull-base imaging, in which both extracranial and intracranial components can be evaluated. MRIs can fail to depict enhancement if the contrast agent bolus is inadequate. MRI is inherently limited in its ability to show subtle areas of bony destruction, which may be important for proper diagnosis. [12]

Angiography is typically reserved for patients who are undergoing preoperative evaluation or for cases in which the presence of neovascularity can help in focusing the differential diagnosis. Angiography is a minimally invasive test and, therefore, not the imaging study of choice. Rarely, diagnostic angiography can show soft-tissue neovascularity in other types of abnormalities, such as those encountered with hypervascular lymphadenopathy or nodular fasciitis. These tumors can have profound neovascularity that mimics that of glomus tumors. [11]

Radiologic intervention

Embolization is a common technique used as the lone treatment option or as a precursor to surgical excision. As a result of the highly vascular nature of these neoplasms, embolization is an effective technique that is aimed at starving the lesion of its blood supply and inducing necrosis. This is the primary and, at times, the only treatment option for glomus jugulare tumors because of the difficulty in excising many of the tumors. In combination with surgical excision, embolization is often used to reduce blood loss, and it has been proven to be highly effective. [18]

In bilateral lesions, especially of the vagale type, embolization is often required as the sole course of treatment for 1 of the lesions, in tandem with surgical excision of the other. This approach is used because of the proximity of the lesion to the vagus nerve and the occasionally inevitable perioperative damage to the nerve during excision.

As a result of their inherent neovascularity, catheter-directed embolization is appropriate in the treatment of chemodectomas, particularly if surgical removal is contemplated. The use of microcatheters allows precise delivery of embolic agents into masses and embolization of multiple, small, feeding trunks. Although glomus tumors may arise from multiple arterial territories, embolization is typically limited to the feeders of the external carotid branch artery.

In the author's practice, glomus tumor embolization is performed in a preoperative setting. Nevertheless, some authors suggest that embolization alone may be beneficial in the treatment of these tumors.

Special concerns

Whenever external carotid embolization is performed, care must be taken to avoid inadvertent extracranial-intracranial embolization and the subsequent risk of stroke. Occult occipital-vertebral connections may be present or open, or they may become apparent on angiograms, only after partial embolization is performed.

Because pterygopalatine–internal carotid collaterals, as well as middle meningeal–middle cerebral communications, may exist, these should be recognized before embolization. In addition, the ophthalmic artery should be identified before external carotid embolization, and care should be taken to identify any existing ethmoidal-ophthalmic arterial communications.

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Computed Tomography

Contrast-enhanced CT scans demonstrate enhancing soft-tissue masses at characteristic locations key to the diagnosis. Nonenhanced CT imaging can demonstrate glomus tumors, but the demonstration of a strongly enhancing mass is typical in the diagnosis of a glomus tumor. [2, 10]

CT scanning demonstrates carotid body tumors at the level of the carotid bifurcation, respectively splaying the internal and external carotid arteries medially and laterally. These tumors can vary in size, but their location within the bifurcation is critical for diagnosis (see the image below). [4]

Carotid body tumor. Computed tomography scan demon Carotid body tumor. Computed tomography scan demonstrates an enhancing carotid bifurcation mass. Reprinted with permission. Copyright Springer-Verlag.

Glomus vagale tumors are masses with similarly strong enhancement. [32] Glomus valgale tumors are paragangliomas involving the vagus nerve. These tumors are seen along the course of the jugular vein and internal carotid artery above the level of the carotid bifurcation but below the skull base. These tumors can vary in size, and they can displace adjacent vascular structures (see the image below). [32]

Glomus vagale tumor. Contrast-enhanced computed to Glomus vagale tumor. Contrast-enhanced computed tomography scan demonstrating a large vascular mass along the course of the left internal carotid artery and jugular vein above the level of the carotid bifurcation. Reprinted with permission. Copyright Springer-Verlag.

Glomus jugulare tumors are enhancing soft-tissue masses at the skull base, but skull-base artifact can mask their presence. These tumors are seen within the jugular foramen; the demonstration of bone erosion of the jugular foramen and petrous apex is often a key finding in the diagnosis (see the image below). Careful review of bone windows is necessary.

Glomus jugulare tumor. Computed tomography scan de Glomus jugulare tumor. Computed tomography scan demonstrates a permeative destructive skull-base mass with involvement of the mastoid air cells. Reprinted with permission. Copyright Springer-Verlag.

Glomus tympanicum tumors are demonstrated in the images below.

Glomus tympanicum tumor. Axial computed tomography Glomus tympanicum tumor. Axial computed tomography image shows a small vascular mass along the right tympanic membrane. Note the adjacent opacified mastoid air cells. Reprinted with permission. Copyright Springer-Verlag.
Glomus tympanicum tumor. Coronal computed tomograp Glomus tympanicum tumor. Coronal computed tomography scan confirms the presence of a known hypotympanic mass consistent with glomus tympanicum. Reprinted with permission. Copyright Springer-Verlag.

The degree of confidence is high. The presence of strongly enhancing neck masses in typical perivascular locations leads to a high degree of confidence regarding the diagnosis.

Hypervascular lymphadenopathy may result in false-positive findings, which can be seen in a variety of disorders such as metastatic papillary carcinoma of the thyroid gland. In these instances, the location is a key finding.

The lack of sufficient contrast enhancement can be troublesome and may result in false-negative findings. In this case, glomus tumors can mimic schwannomas, neurofibromas, or nonenhancing lymphadenopathies. Potentially, small vascular tumors can be missed if they are not easily distinguishable from the adjacent vascular structures.

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Magnetic Resonance Imaging

Similar to CT imaging, contrast-enhanced MRI demonstrates enhancing soft-tissue masses at characteristic locations; these findings are important for diagnosis. Nonenhanced MRI can demonstrate glomus tumors, but the demonstration of a strongly enhancing mass is typical in the diagnosis of a glomus tumor. [10, 11, 12, 4]

As with most soft-tissue tumors, glomus tumors are isointense on T1-weighted MRIs and hyperintense on T2-weighted MRIs, relative to skeletal muscle (see the images below).

Glomus jugulare tumor. Axial contrast-enhanced T1- Glomus jugulare tumor. Axial contrast-enhanced T1-weighted magnetic resonance image shows an enhancing right skull-base mass. Reprinted with permission. Copyright Springer-Verlag.
Glomus jugulare tumor. Coronal T1-weighted contras Glomus jugulare tumor. Coronal T1-weighted contrast-enhanced magnetic resonance image shows a mass protruding into the jugular foramen. Reprinted with permission. Copyright Springer-Verlag.
Carotid body tumor. Contrast-enhanced T1-weighted Carotid body tumor. Contrast-enhanced T1-weighted magnetic resonance image shows a strongly enhancing mass overlying the right carotid bifurcation. Reprinted with permission. Copyright Springer-Verlag.
Carotid body tumor. Axial T2-weighted magnetic res Carotid body tumor. Axial T2-weighted magnetic resonance image demonstrates splaying of the external and internal carotid arteries. Reprinted with permission. Copyright Springer-Verlag.

Contrast-enhanced imaging can show intense tumor enhancement, which again is a key finding in the diagnosis. In addition, a salt-and-pepper fine vascular pattern can be seen in the tumors; this finding is suggestive of intrinsic tumor neovascularity and is particularly well demonstrated on T2-weighted images (see the image below).

Glomus jugulare tumor. Axial T2-weighted magnetic Glomus jugulare tumor. Axial T2-weighted magnetic resonance image shows salt-and-pepper vascularity in the substance of the tumor. Reprinted with permission. Copyright Springer-Verlag.

MRIs can show densely enhancing carotid body tumors at the level of the carotid bifurcation, which respectively splay the internal and external carotid arteries medially and laterally (see the images below).

Carotid body tumor. Contrast-enhanced T1-weighted Carotid body tumor. Contrast-enhanced T1-weighted magnetic resonance image shows a strongly enhancing mass overlying the right carotid bifurcation. Reprinted with permission. Copyright Springer-Verlag.
Carotid body tumor. Axial T2-weighted magnetic res Carotid body tumor. Axial T2-weighted magnetic resonance image demonstrates splaying of the external and internal carotid arteries. Reprinted with permission. Copyright Springer-Verlag.

Glomus jugulare tumors are particularly well demonstrated by using MRI, which can show that enhancing soft-tissue masses protrude both intracranially and extracranially at the skull base. Direct coronal imaging can show tumoral relationships to adjacent structures such as the brainstem and skull base, and deep cervical soft-tissue structures are extraordinarily well depicted.

The degree of confidence is high. Tumor allocation and intense tumor enhancement are of paramount importance in diagnosis.

Hypervascular lymphadenopathy may result in false-positive findings, which can be seen in a variety of disorders such as metastatic papillary carcinoma of the thyroid gland. In particular, MRI findings can be confusing if T2-weighted images show a salt-and-pepper pattern. In such instances, the location is a key finding.

As with CT imaging, the lack of sufficient contrast enhancement can be troublesome and may result in false-negative findings. In this case, glomus tumors can mimic schwannomas, neurofibromas, or nonenhancing lymphadenopathies if an insufficient amount of contrast material is administered. Potentially, small vascular tumors can be missed if they are not clearly distinguishable from the adjacent vascular structures.

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Ultrasonography

Doppler ultrasonography can demonstrate cervical masses when they are imaged below the angle of the mandible, above the sternum, or in a superficial location not hidden by bone. Ultrasonography can demonstrate the extent of the masses and show their locations. In glomus tumors, the diagnosis of carotid body tumors is possible with ultrasonographic cervical imaging, but this modality does not suitably reveal the location of glomus vagale, jugulare, and tympanicum tumors.

Because of tumor neovascularity, Doppler ultrasonographic sampling of cervical masses, such as carotid body tumors, can be helpful in the diagnosis. In addition, if recognized, increased flow velocities in the external carotid artery or in the jugular vein can provide an indirect clue to the diagnosis of a vascular mass that is above the ultrasonographic imaging field.

The degree of confidence is high if these tumors are found by using ultrasonography. Hypervascular lymphadenopathy can mimic a glomus tumor and cause a false-positive result. Many glomus tumors are seen in the neck above the level of the mandibular angle, rendering the diagnosis impossible with the use of ultrasonography alone; a false-negative finding can result.

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Angiography

Glomus tumors of the head and neck are typically highly vascular, as shown on angiograms. This finding differentiates them from other types of neck neoplasia (see the images below).

Carotid body tumor. Lateral angiogram shows a dens Carotid body tumor. Lateral angiogram shows a densely vascular mass at the level of the carotid bifurcation that causes splaying of the internal and external carotid arteries. Reprinted with permission. Copyright Springer-Verlag.
Carotid body tumor. Postembolization with polyviny Carotid body tumor. Postembolization with polyvinyl alcohol. Many feeding arteries arising from the occipital artery and superior thyroid arteries were embolized to achieve this result. Reprinted with permission. Copyright Springer-Verlag.
Angiogram in a patient who had a biopsy-proven ren Angiogram in a patient who had a biopsy-proven renal cell carcinoma that metastasized to a laryngeal lymph node and formed a hypervascular mass that mimicked a glomus tumor. This mass was not in a typical location for a glomus tumor, despite its vascular appearance. Reprinted with permission. Copyright Springer-Verlag.

Typical carotid body tumors are situated in the carotid bifurcation and derive their arterial supply from regional external carotid branch arteries. These include the ascending pharyngeal and occipital arteries.

Glomus vagale and jugulare tumors are encountered higher in the neck and at the skull base; therefore, these masses concomitantly involve higher external carotid branch vessels (see the images below).

Glomus vagale tumor. Angiogram demonstrates a dens Glomus vagale tumor. Angiogram demonstrates a densely vascular mass. Reprinted with permission. Copyright Springer-Verlag.
Glomus jugulare tumor. Selective external carotid Glomus jugulare tumor. Selective external carotid angiogram demonstrates a vascular skull-base mass. Reprinted with permission. Copyright Springer-Verlag.
Glomus jugulare tumor. Lateral angiogram demonstra Glomus jugulare tumor. Lateral angiogram demonstrates a large vascular mass arising from branches of the external carotid artery. Note the anterior bowing of the internal carotid artery and extension of the mass through the skull base. Reprinted with permission. Copyright Springer-Verlag.
Multiple glomus tumors. Angiogram obtained in a fe Multiple glomus tumors. Angiogram obtained in a female patient with bilateral carotid body tumors, bilateral glomus vagale tumors, and left glomus jugulare tumors with corresponding angiographically enhancing masses. Reprinted with permission. Copyright Springer-Verlag.

The ascending pharyngeal, tympanic, and occipital arteries dominate the arterial blood supply. The neovascularity may be extremely intense, and arteriovenous fistulae may be present. Rarely, the internal carotid and vertebral arteries may contribute feeders to the neoplasms. Typically, these tumors are evaluated, with attention paid to all potential feeding arteries. Care is taken to evaluate occult contralateral or ipsilateral masses, which can be occasionally overlooked during cross-sectional imaging.

The degree of confidence is high. The hallmark of a glomus tumor is its intrinsic neovascularity. At times, lymph node neovascularity can be difficult to differentiate from glomus tumors. In these situations, the location is a key feature.

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