Updated: Jan 29, 2008
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.1
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Glomus tumors of the head and neck paraganglia are part of the extra-adrenal neuroendocrine system. At birth, small patches of paraganglionic cells can be widely dispersed throughout the body, mostly in association with autonomic nervous tissue. In the head and neck, such areas include the chemoreceptive areas (glomus tissue) of the carotid bifurcations, the aortic arch, and the temporal bone. Mafee et al noted that paraganglionic cells are also found, although less frequently, in areas of the orbit (in association with the ciliary ganglion), pterygopalatine fossa (in association with the pterygopalatine ganglion), buccal mucosa, nasopharynx, larynx,2 and dermis. However, progressive involution, which lasts until puberty, normally obliterates the paraganglionic cells of these and other extra-adrenal locations.3
Histologically, all paraganglia are composed of chief cells (type I cells; ie, chemoreceptive cells) and sustentacular cells (type II cells; ie, supporting cells). The specific ratio of the 2 types of cells determines the function of that particular paraganglion. Developmentally, both types of cells are of neuroectodermal origin; specifically, they arise from neural crest cells.
The major paraganglia that do not undergo involution are the carotid bodies. They line the medial wall of the bifurcation of the common carotid artery. These paraganglia are a functionally important chemoreceptive organ for homeostasis. Specifically, they detect changes in arterial partial pressures of oxygen and carbon dioxide and changes in pH and other blood-borne factors. Accordingly, the paraganglia can increase or decrease stimulation to the brainstem respiratory centers, which affects various cardiopulmonary functions, including respiratory rate and cardiac output.
Glomus tumors of the head and neck are associated with 4 primary locations, as follows:
Although glomus tumors usually appear as solitary lesions at 1 site, multiple lesions at multiple sites are not uncommon. 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. These vessels are characteristics of glomus tumors at pathologic evaluation.
Glomus tumors represent 0.6% of neoplasms of the head and neck and 0.03% of all neoplasms. Glomus jugulare tumors are the most common head and neck glomus tumors4,5 ; the rarest are glomus tympanicum tumors.10,3 Glomus tumors are the most common tumors of the inner ear. They are the second most common tumors of the temporal bone, second only to schwannomas.
The vast majority of glomus tumors are benign and slow to grow. Mortality rates are 9-15%, depending on the location of the tumor and the study; however, they can cause several clinically significant problems.
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Carotid body tumors have no sex predilection. However, studies have shown evidence of a female sex predilection with vagale and jugulare tumors, with female-to-male ratios of 2.7:1 and 5:1, respectively.
Studies indicate that the incidence of carotid body tumors peaks in patients aged 45-50 years, whereas the incidence of tumors of vagal and jugular origin peaks in those aged 50-60 years. Glomus tumors of the head and neck are extremely rare in pediatric patients.
Glomus jugulare tumors are typically located just under the skull base, at the bulb of the external jugular vein.4,5 The tumors may spread superiorly into the jugular foramen, causing CN IX, CN X, and CN XI deficits. The primary blood supply to jugulare tumors is via the ascending pharyngeal artery. In addition, the occipital and posterior auricular arteries can contribute to vascularization.
Glomus tympanicum tumors arise in the middle ear cavity along the tympanic membrane, and they typically receive the same blood supply as that of jugulare tumors.10,11
Glomus vagale tumors typically appear as a cervical mass. The tumors receive their vascularization primarily via the occipital and ascending pharyngeal arteries. Less commonly, the maxillary artery and the muscular branches of the vertebral arteries can contribute vascularization as well.14
Carotid body tumors lie in the carotid bifurcation and can cause it to splay. The blood supply for the tumors is typically derived from the external carotid artery. Vascularization also can arise from the internal carotid and vertebral arteries.3
History
The typical presentation of a glomus vagale tumor is that of a slow-growing mass in the parapharyngeal space. Patients with these tumors may present with CN palsies of either one nerve or a combination of the vagus, hypoglossal, accessory, and glossopharyngeal nerves late in the course of the disease. Vagal nerve deficits are most commonly late findings because the individual fibers are splayed across the surface of the tumor approximately 2 years after its onset.14
Patients with carotid body tumors are largely asymptomatic and have a mobile, nontender, growing, lateral neck mass. Some patients may report hoarseness and dysphagia associated with compression of the trachea and esophagus and/or vertigo and paresis resulting from CN compression.
Patients with glomus jugulare tumors and glomus tympanicum tumors often present with the same set of clinical features. The most common clinical presentation is that of pulsatile tinnitus. Other early symptoms include conduction hearing loss, aural pain, vertigo, and hoarseness. At later stages, deficits of the glossopharyngeal, vagus, and accessory nerves are common. In addition, deficits of the facial and vestibulocochlear nerves can be present.4,5
Because glomus jugulare and tympanicum tumors have identical clinical findings, the clinical differentiation of the 2 types usually is impossible. However, preoperative diagnosis of the tumor type is important because the type determines the surgical approach. Therefore, radiologic imaging is required in these patients.
Patients with functioning tumors may present with hypertension, headache, palpitations, and tachycardia resulting from increased levels of circulating catecholamines.
Physical examination
Lesions in all 3 locations are characterized by the aggressive local growth of the affected paraganglia. On gross inspection of resected lesions, carotid body and vagale tumors are generally ovoid and have well-circumscribed borders. Conversely, jugulare lesions appear almost malignant, but their shape is a result of the tortuous environment through which the tumors navigate during growth. Histologically, neoplasms in all 3 locations are usually benign. Malignancies and distal metastatic deposits, though reported, are rare and extremely uncommon in these lesions.
Causes
Glomus tumors are believed to result from an overresponse to a change in body homeostasis. An insidious link appears to exist between oxygen deprivation and glomus tumor incidence. Compensatory hypertrophy of the carotid body is known to occur only in patients with prolonged hypoxia and hypercapnia. Some studies have shown that long exposure to high altitudes appears to be correlated with a 10-fold higher incidence of carotid body tumors but no increase in the incidence of paragangliomas at other sites. Other studies are under way to explore the effects of smoking and other sources of long-term anoxia.
A familial component may predispose some patients to carotid body tumors. These patients also have a higher risk of having multiple tumors.
Laboratory studies
Routine laboratory studies are not helpful. In the rare patient with functioning lesions, preoperative and postoperative catecholamine measures may help confirm successful resection of the lesion. Otherwise, these lesions are silent in the laboratory workup.
Imaging studies
Imaging is the primary investigative modality for glomus tumors of the head and neck. A combination of contrast-enhanced CT, MRI, and angiography is ideal for proper diagnosis and localization of the tumors. Lesions show a characteristic signature on images, which is based on its location. 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.19,20,21
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 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, the mass can be misconstrued for a nonenhancing schwannoma or nodal lesion.19,20,21
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.21
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.20
Chondrosarcoma
Giant Cell Tumor
Osteoblastoma
Schwannoma, Cranial Nerve
Meningiomas
Neural lesions - Neurilemoma, neurofibroma, chordoma
Aberrant carotid artery
Exposed jugular bulb
Carcinomas - Primary and metastatic
Hypervascular lymphadenopathy
Routine plain-film radiography does not have a role in the diagnosis of glomus tumors.
Contrast-enhanced CT demonstrates enhancing soft-tissue masses at characteristic locations key to 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.13,19
CT 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.
Glomus vagale tumors are masses with similarly strong enhancement. 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.
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. Careful review of bone windows is necessary.
The degree of confidence is high. The presence of strongly enhancing neck masses in typical perivascular locations lead to a high degree of confidence regarding 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.
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.19,20,21
As with most soft-tissue tumors, glomus tumors are isointense on T1-weighted MRIs and hyperintense on T2-weighted MRIs, relative to skeletal muscle.
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.
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.
Glomus jugulare tumors are particularly well demonstrated by using MRI. Images 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.
Doppler ultrasonography (US) 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. US can demonstrate the extent of the masses and show their locations. In glomus tumors, the diagnosis of carotid body tumors is possible with US cervical imaging, but US does not suitably reveal the location of glomus vagale, jugulare, and tympanicum tumors.
Because of tumor neovascularity, Doppler US sampling of cervical masses, such as carotid body tumors, can be helpful in 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 US imaging field.
The degree of confidence is high if these tumors are found by using US.
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 by using US alone; a false-negative finding can result.
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.
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; 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.
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.
The preferred method of treatment for glomus tumors of the head and neck is surgery. However, because most paragangliomas are slow-growing and benign, radiation treatment alone or no treatment at all is preferred in elderly patients in whom the risks of surgery are relatively high and the tumor is unlikely to cause serious morbidity or mortality. If the patient is young, surgery is the best available option because it is the only option that allows total cure.4,5,6,7,8,9,12,15,16,17,18,22,23,24
Radiation therapy
Gamma-knife irradiation is commonly used in patients who are poor candidates for surgical excision or embolization because of their age or disease state or because of unacceptable morbidity. This procedure is expensive, and clear remission is not reported to date. However, gamma-knife irradiation may be of some use in controlling the tumor and, thereby, in preventing it from growing larger.5,6,7,8,9,12
Embolization
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.
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.
Typically, the author performs glomus tumor embolization by using polyvinyl alcohol, starting with 50-µm particles and progressing to 250- to 300-µm particles as warranted. Rarely, highly vascular shunts in the glomus tumors may require the use of liquid-adhesive embolic agents to close any rapidly forming fistulae. Occlusive coils implanted along feeding arterial pedicles can be helpful in closing feeders in difficult complex tumors; however, the author tends to use these devices sparingly. 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.
Surgery
Surgical excision is the treatment of choice, and current techniques are highly successful with relatively low morbidity rates: blood loss and CN neuropathies are the major complications. Depending on the specific type and location of the tumor, different surgical approaches are required.4,9,15,16,18,22,23,24
Most otologists can excise an intratympanic and/or intramastoid tumor via a transmeatal or transmastoid approach. Jugular bulb tumors require excision by surgeons experienced in neck and mastoid surgery. Tumors invading the carotid canal require an infratemporal approach; expertise in skull-base surgery is required. The treatment of transdural glomus tumors requires neurosurgical expertise.
Tumors involving the cavernous sinus or the foramen magnum may be unresectable, and any combination of subtotal resection, radiation therapy, and embolization may be required.
As a result of the highly vascular nature of glomus tumors, preoperative embolization is commonly used to reduce surgical blood loss. The only exception is in patients with glomus tympanicum tumors, in whom surgical blood loss is typically limited.
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chemodectoma, nonchromaffin paraganglioma, glomus body tumors, paraganglioma, glomus jugulare tumors, glomus tympanicum tumors, glomus vagale tumors, carotid body glomus tumors, carotid body tumors
Robert A Koenigsberg, DO, MSc, FAOCR, Director of Neuroradiology, Professor, Department of Radiology, Drexel University College of Medicine
Robert A Koenigsberg, DO, MSc, FAOCR is a member of the following medical societies: American Osteopathic Association, American Society of Interventional & Therapeutic Neuroradiology, American Society of Neuroradiology, and Radiological Society of North America
Disclosure: Nothing to disclose.
Cyrus Dastur, BS, Department of Neuroradiology, MCP Hahnemann University
Cyrus Dastur, BS is a member of the following medical societies: American Medical Student Association/Foundation
Disclosure: Nothing to disclose.
Robert Kim, BS, Department of Neuroradiology, Hahnemann University Hospital
Disclosure: Nothing to disclose.
Barton F Branstetter IV, MD, Assistant Professor of Radiology and Otolaryngology, University of Pittsburgh; Director of Head and Neck Imaging, Associate Director of Informatics, Department of Radiology, Division of Neuroradiology, University of Pittsburgh Medical Center
Barton F Branstetter IV, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Roentgen Ray Society, American Society of Neuroradiology, Pennsylvania Medical Society, and Radiological Society of North America
Disclosure: Nothing to disclose.
Bernard D Coombs, MB, ChB, PhD, Consulting Staff, Department of Specialist Rehabilitation Services, Hutt Valley District Health Board, New Zealand
Disclosure: Nothing to disclose.
C Douglas Phillips, MD, Professor, Departments of Radiology, Neurosurgery, and Otolaryngology, University of Virginia Health Sciences Center
C Douglas Phillips, MD is a member of the following medical societies: American College of Radiology, American Medical Association, American Society of Head and Neck Radiology, American Society of Neuroradiology, Association of University Radiologists, and Radiological Society of North America
Disclosure: Nothing to disclose.
Robert M Krasny, MD, Consulting Staff, Department of Radiology, The Angeles Clinic and Research Institute
Robert M Krasny, MD is a member of the following medical societies: American Roentgen Ray Society and Radiological Society of North America
Disclosure: Nothing to disclose.
Lawrence M Davis, MD, Assistant Professor of Diagnostic Imaging (Clinical), Department of Diagnostic Imaging, Brown Medical School
Lawrence M Davis, MD is a member of the following medical societies: American College of Radiology, American Roentgen Ray Society, American Society of Neuroradiology, Radiological Society of North America, and Rhode Island Medical Society
Disclosure: Nothing to disclose.
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