Introduction
Background
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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Pathophysiology
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:
- Jugular bulb: Tumors here are commonly called glomus jugulare tumors.4,5,6,7,8,9 These 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.
- Middle ear cavity: Tumors here are commonly called glomus tympanicum tumors.9,10,11,12,13 They arise from the glomus bodies that run with the tympanic branch of the glossopharyngeal nerve. Glomus tympanicum tumors are the most common primary neoplasms of the middle ear.
- Vagus nerve: Tumors in this area are commonly called glomus vagale tumors because of their usual close association with the vagus nerve.14,15,16 Specifically, they arise infratemporally along the course of the cervical vagus nerve.
- Carotid body: 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.3,16,17,18
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.
Frequency
International
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.
Mortality/Morbidity
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.
- Glomus tumors are soft-tissue masses and can cause bulges that are aesthetically unpleasant.
- Glomus jugulare and tympanicum tumors both can cause pulsatile tinnitus and conductive hearing loss.
- Cranial nerve (CN) deficits are also fairly common. Glomus tympanicum tumors can compress CN VII and CN VIII of the middle ear. Glomus jugulare tumors, as a result of their course through the jugular foramen, can compress CN IX, CN X, and CN XI.
- Functioning tumors, which are rare, can increase the risk of mortality. These active tumors secrete catecholamines, which can lead to clinical manifestations of hypertension, headaches, palpitations, and tachycardia.
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Sex
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.
Age
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.
Anatomy
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
Presentation
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.
Preferred Examination
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
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 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
Differential Diagnoses
Chondrosarcoma
Giant Cell Tumor
Osteoblastoma
Schwannoma, Cranial Nerve
Other Problems to Be Considered
Meningiomas
Neural lesions - Neurilemoma, neurofibroma, chordoma
Aberrant carotid artery
Exposed jugular bulb
Carcinomas - Primary and metastatic
Hypervascular lymphadenopathy
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References
Glenner GG, Grimley PM. Tumors of the extra-adrenal paraganglion system (including chemoreceptors). In: Atlas of Tumor Pathology, Second Series. Armed Forces Institute of Pathology;1974.
Marlowe SD, Swartz JD, Koenigsberg R, et al. Metastatic hypernephroma to the larynx: an unusual presentation. Neuroradiology. 1993;35(3):242-3. [Medline].
Mafee MF, Raofi B, Kumar A, Muscato C. Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and simulating lesions. Role of MR imaging. Radiol Clin North Am. Sep 2000;38(5):1059-76. [Medline].
Patel SJ, Sekhar LN, Cass SP, Hirsch BE. Combined approaches for resection of extensive glomus jugulare tumors. A review of 12 cases. J Neurosurg. Jun 1994;80(6):1026-38. [Medline].
Pluta RM, Ram Z, Patronas NJ, Keiser H. Long-term effects of radiation therapy for a catecholamine-producing glomus jugulare tumor. Case report. J Neurosurg. Jun 1994;80(6):1091-4. [Medline].
Li G, Chang S, Adler JR Jr, Lim M. Irradiation of glomus jugulare tumors: a historical perspective. Neurosurg Focus. 2007;23(6):E13. [Medline].
Henzel M, Hamm K, Gross MW, Surber G, Kleinert G, Failing T. Fractionated stereotactic radiotherapy of glomus jugulare tumors. Local control, toxicity, symptomatology, and quality of life. Strahlenther Onkol. Oct 2007;183(10):557-62. [Medline].
Varma A, Nathoo N, Neyman G, Suh JH, Ross J, Park J. Gamma knife radiosurgery for glomus jugulare tumors: volumetric analysis in 17 patients. Neurosurgery. Nov 2006;59(5):1030-6; discussion 1036. [Medline].
Pemberton LS, Swindell R, Sykes AJ. Radical radiotherapy alone for glomus jugulare and tympanicum tumours. Oncol Rep. Dec 2005;14(6):1631-3. [Medline].
Cheng A, Niparko JK. Imaging quiz case 2. Glomus tympanicum tumor of the temporal bone. Arch Otolaryngol Head Neck Surg. May 1997;123(5):549, 551-2. [Medline].
O''Leary MJ, Shelton C, Giddings NA, et al. Glomus tympanicum tumors: a clinical perspective. Laryngoscope. Oct 1991;101(10):1038-43. [Medline].
Salami A, Mora R, Dellepiane M. Piezosurgery in the exeresis of glomus tympanicum tumours. Eur Arch Otorhinolaryngol. Jan 4 2008;[Medline].
Coulier B, Mailleux P, Lefrancq M. Images in clinical radiology. Glomus tympanicum: CT diagnosis. JBR-BTR. Nov-Dec 2003;86(6):359. [Medline].
Biller HF, Lawson W, Som P, Rosenfeld R. Glomus vagale tumors. Ann Otol Rhinol Laryngol. Jan 1989;98(1 Pt 1):21-6. [Medline].
Cheng AG, Maronian NC, Futran ND. Cerebrospinal fluid leak in the neck: a rare complication of glomus vagale excision. Otolaryngol Head Neck Surg. Feb 2006;134(2):334-5. [Medline].
Karatas E, Sirikci A, Baglam T, Mumbuc S, Durucu C, Tutar E. Synchronous bilateral carotid body tumor and vagal paraganglioma: A case report and review of literature. Auris Nasus Larynx. Mar 2008;35(1):171-5. [Medline].
Piazza P, Di Lella F, Menozzi R, Bacciu A, Sanna M. Absence of the contralateral internal carotid artery: a challenge for management of ipsilateral glomus jugulare and glomus vagale tumors. Laryngoscope. Aug 2007;117(8):1333-7. [Medline].
Smith JJ, Passman MA, Dattilo JB, Guzman RJ, Naslund TC, Netterville JL. Carotid body tumor resection: does the need for vascular reconstruction worsen outcome?. Ann Vasc Surg. Jul 2006;20(4):435-9. [Medline].
Som PM, Curtin HD. Head and Neck Imaging. 3rd ed. Mosby-Year Book;1996:932-6, 1484-96.
Vogl TJ, Juergens M, Balzer JO, et al. Glomus tumors of the skull base: combined use of MR angiography and spin-echo imaging. Radiology. Jul 1994;192(1):103-10. [Medline].
van den Berg R, van Gils AP, Wasser MN. Imaging of head and neck paragangliomas with three-dimensional time-of- flight MR angiography. AJR Am J Roentgenol. Jun 1999;172(6):1667-73. [Medline].
Jackson CG. Neurotologic skull base surgery for glomus tumors. Diagnosis for treatment planning and treatment options. Laryngoscope. Nov 1993;103(11 Pt 2 Suppl 60):17-22. [Medline].
Woods CI, Strasnick B, Jackson CG. Surgery for glomus tumors: the Otology Group experience. Laryngoscope. Nov 1993;103(11 pt 2 suppl 60):65-70. [Medline].
van der Mey AG, Frijns JH, Cornelisse CJ, et al. Does intervention improve the natural course of glomus tumors? A series of 108 patients seen in a 32-year period. Ann Otol Rhinol Laryngol. Aug 1992;101(8):635-42. [Medline].
Further Reading
Keywords
chemodectoma, nonchromaffin paraganglioma, glomus body tumors, paraganglioma, glomus jugulare tumors, glomus tympanicum tumors, glomus vagale tumors, carotid body glomus tumors, carotid body tumors
