Disclaimer: The information contained within the Grand Rounds Archive is intended for use by doctors and other health care professionals. These documents were prepared by resident physicians for presentation and discussion at a conference held at Baylor College of Medicine in Houston, Texas. No guarantees are made with respect to accuracy or timeliness of this material. This material should not be used as a basis for treatment decisions, and is not a substitute for professional consultation and/or peer-reviewed medical literature. Therapeutic Options for Treatment of Glomus Tumors of the Temporal Bone Glomus tumors arise from paraganglionic chemoreceptor cells and are part of the APUD system of cells of neural crest origin. Paragangliomas of the jugular-tympanic area are the most common tumors involving the middle ear. In 1941, Guild described the distribution of normal glomus bodies within the temporal bone. Then in 1945, Rosenwasser correctly recognized and reported the first case of a tumor arising from these cells. After this report, many new cases were reported and many older cases were reclassified as true glomus tumors. These neoplasms can occur at any age but the peak incidence is in the fifth decade. Glomus tumors occur four to five times as often in women as in men and there is a predilection for them in the Caucasian race. The majority of these tumors arise from along the jugular bulb or Jacobson's nerve (85%), while 12% arise from glomus bodies overlying the promontory and three percent have their origin attributed to Arnold's nerve. The most common presenting symptoms include conductive hearing loss and pulsatile tinnitus. Other symptoms may include aural hemorrhage or otorrhea, otalgia and facial palsy. When the tumors enlarge within the jugular foramen neuropathies of IX, X, XI and XII occur. Anterior expansion toward the clivus may produce abducens and trigeminal palsies. Aquino's sign is blanching of the tympanic mass with gentle pressure on the carotid artery. The sign of Brown is the pulsation sign elicited by pneumatic compression and abolished with further compression. These tumors may be multicentric. Association of these tumors with other tumors arising from cells of neural crest origin (MEN I and pheochromocytoma) is not uncommon. This must be considered to avoid the devastating consequences of inducing general anesthesia in a patient with an unrecognized pheochromocytoma. Malignancy occurs in one to three percent of these tumors. Evaluation includes a careful history and physical examination. High resolution computed tomography provides accurate information about bony destruction and the extent of the disease. Arteriography remains the most useful radiologic modality available for the assess-ment of a glomus tumor. Prior to angiography, serum catacholamines and urinary vanillylmandelic acid (VMA) levels should be obtained to screen for tumors which secrete these vasoactive compounds. Therapeutic options for glomus tumors of the temporal bone include surgical excision, radiotherapy, combined therapy and in selected cases observation. Surgical excision offers the only chance for total tumor eradication. Gary Jackson writes that "...surgical management is curative and the treatment of choice." Proponents of radiotherapy argue that radiation provides for the amelioration of symptoms and arrests the progression of the glomus tumors providing a safe and efficacious modality of therapy. Many surgical approaches have been proposed for the resection of these tumors (see bibliography). No single approach is optimal for all tumors. Jackson and Glasscock proposed guidelines for a planned excision of these tumors. They suggest approaching type I glomus tympanicum tumors via a transcanal approach, and an extended facial recess approach for type II-IV glomus tympanicum tumors. Type I and II glomus jugulare lesions are treated by standard skull base techniques. Type III and IV tumors require an infratemporal fossa approach. The infratemporal fossa as described by Fisch provides for direct surgical access to the entire length of the intratemporal carotid artery and for control of the great venous sinuses. These infratemporal fossa approaches provide access as follows: Type A provides access to the temporal bone in its infralabyrinthine and apical compartments and its inferior surface. Type B provides access to the clivus and type C provides access to the parasellar region and nasopharynx. Utilizing these skull base approaches it is now possible to perform total extirpation of tumors that were previously considered unresectable. Skull base surgery is not undertaken without certain risks. Mortality rates range from four to seven percent. Reported complications include CSF leaks, new cranial nerve palsies, wound infections, and deafness. The risks of surgery are balanced out by the chance for total tumor eradication. The reported rate of local control for glomus tumors of the temporal bone with surgical treatment from several studies is shown in Table 4. Although radiotherapy is unable to provide complete tumor eradication, the success of radiotherapy in controlling the progression of symptoms is quite high. The reported rate of local control with radiation therapy for glomus tumors of the temporal bone from several studies is shown in Table 5. Complications of radiotherapy include osteoradionecrosis, brain necrosis, abscess formation, and radiation induced malignancies. Case Presentation A 69-year-old African American female had initially sought assistance from a hearing aid dispenser after experiencing several years of slow, progressive left-sided hearing loss. However, a medical referral was made when the presence of a unilateral conductive hearing loss and pulsatile mass on the tympanic membrane were discovered. CT examination of the temporal bones demonstrated an opacification of the left mastoid air cells and middle ear cavity. She was then referred to Baylor College of Medicine for further evaluation and treatment. Upon questioning, she reported that she could appreciate a rhythmic sound in her left ear that was synchronous with her heart rate. Examination revealed a pulsating tympanic membrane with dilated vasculature that extended onto the skin of the external canal. The sign of Aquino was negative and the Weber lateralized to the left. There were no cranial nerve deficits. Arteriography was obtained. This demonstrated a vascular mass of the left temporal bone supplied by the penetrating vessels of the external carotid artery. These findings were consistent with a glomus tympanicum. The options of surgical resection, radiotherapy and observation were explained to the patient. It was elected to observe the tumor for the time being and to offer her radiotherapy should the tumor enlarge or become symptomatic. Bibliography Alford BR, Guilford FR: A comprehensive study of tumors of the glomus jugulare. Laryngoscope 72:765-787, 1962. Aquino J: Glomus jugulare tumors. Arch Otolaryngol 65:263-268, 1957. Bartels LJ, Gurucharri M: Pediatric glomus tumors. Otolaryngol Head Neck Surg 98:392-395, 1988. Bataini J, Pontvert D, Jaulerry C, et al: Tumeurs du glomus jugulaire. Neurochirurgie 31:377-380, 1985. Bojrab DI, Glasscock ME, Roland PS, et al: Glomus tumors of the temporal bone. Washington DC: AAO-HNS, 1988. Boyle, JO, Shimm DS, Coulthard SW: Radiation therapy for paragangliomas of the temporal bone. Laryngoscope 100:896-901, 1990. Brackmann DE, Hitseleberger WE: Retrolabyrinthine approach technique and newer indications. Laryngoscope 88:286-297, 1978. Brandt TW, Jenkins HA, Coker NA: Facial paralysis as the initial presentation of an internal carotid artery aneurysm. Arch Otolaryngol Head Neck Surg 112:198-202, 1986. Brismar J, Cronqvist: Therapeutic embolization in the external carotid artery region. Radiologica Diagnosis 19:713-731, 1978. Brown, LA: Glomus jugulare tumor of the middle ear clinical aspects. Laryngoscope 53:281292, 1953. Bundgaard T, Tandrup O, Elbrond O, et al: Treatment of glomus tumours. A retrospective survey. Clin Otolaryngol 14:155-160, 1989. Cece JA, Lawson W, Biller HF: Complications in the management of large glomus jugulare tumors. Laryngoscope 97:152-157, 1987. Cole JM: Glomus jugulare tumor. Laryngoscope 87:1244-55, 1977. Conley JJ: Multiple paragangliomas in the head and neck. Ann Otol Rhinol Laryngol 65:356-360, 1956. Cummings BJ, Beale FA, Garrett PG: The treatment of glomus tumors in the temporal bone by megavoltage radiation. Cancer 53:2635-2640, 1984. Dawes PJ, Filippou M, Welch AR, et al: The management of glomus jugulare tumours. Clin Otolaryngol 12:15-24, 1987. Dayal VS, Hinojosa R, Amenta CA: Surgical inferences from study of temporal bones with glomus jugulare tumor. Otolaryngol Head Neck Surg 102:690-697, 1990. Dickens JW, Million RR, Singleton GT, et al: Chemodectomas arising in temporal bone structures. Laryngoscope 92:188-191, 1982. Ferrara P, Cimino A, Tortorici M: Role of radiation therapy in glomus tumor. Am J Otol 8:390-395, 1987. Fisch U: Infratemporal fossa approach for extensive tumors of the temporal bone and base of the skull, in Silverstein H, Norrell H (eds): Neurological Surgery of the Ear. Birmingham, Ala., Aesculapius, 1977. Fisch U: Infratemporal fossa approach for glomas tumors of the temporal bone. Ann Otol Rhinol Laryngol 92:474-479, 1982. Fisch U: La voie d'abord infratemporale pour les tumeurs du glomus jugulaire. Neurochirurgie 31:367-376, 1985. Fisch U, Fagan P, Valavanis A: The infratemporal fossa approach for the lateral skull base. Otolaryngol Clin North Am 17:513-552, 1984. Franklin DJ, Jenkins HA, Horowitz, BL: Management of petrous apex lesions. Arch Otolaryngol 115:1121-1125, 1989. Fuller AM, Brown HA, Harrison, EG: Chemodectomas of the glomus jugulare tumors. Laryngoscope 77:218-238, 1967. Gardner G, Cocke EW, Robertson, JT et al: Glomus jugulare tumours - combined treatment. J Laryngol Otol 95:437-580, 1981. Glasscock ME, Harris PF, Newsome G: Glomus tumors: Diagnosis and treatment. Laryngoscope 84:2006-2032, 1974. Glassock ME, Smith PG, Bond AG: Management of aneurysms of the petrous portion of the internal carotid artery by resection and primary anastomosis. Laryngoscope 93:1445-1453, 1983. Guild SR: The glomus jugulare, a nonchromaffin paraganglion, in man. Ann Otol Rhinol Laryngol 62:1045-1071, 1953. Gulya AJ, Glasscock ME, Jackson CG, et al: Skull-base surgery: Operative refinements. 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Pryzant RM, Chou JL, Easley JD: Twenty year experience with radiation therapy for temporal bone chemodectomas. Int J Radiation Oncology Biol Phys 17:1303-1307, 1989. Rosenwasser H: Carotid body tumor of the middle ear and mastoid. Arch Otolaryngol 41:64-7, 1945. Rosenwasser H: Current management glomus jugulare tumors. Ann Otol Rhinol Laryngol 76:603-10, 1967. Rosenwasser H: Glomus jugulare tumors. Arch Otolaryngol 88:37-77, 1968. Rosenwasser H: Glomus jugulare tumors. Arch Otolaryngol 89:186-192, 1969. Rosenwasser H: Metastasis from glomus jugulare tumors. Arch Otolaryngol 67:197-203, 1957. Schwaber MK, Glasscock ME, Nissen AJ, et al: Diagnosis and management of catecholamine secreting glomus tumors. Laryngoscope 94:1008-1015, 1984. Shambraugh GE: Surgical approach for so-called glomus jugulare tumors of the middle ear. Laryngoscope 65:185-198, 1955. Shapiro MJ, Neues DK: Technique for removal of glomus jugulare tumors. Arch Otolaryngol 79:219-224, 1961. 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Arch Otolaryngol 82:5-13, 1965. Tidwell TJ, Montague ED: Chemodectomas involving the temporal bone. Radiology 118:147-149, 1975. Wang M, Hussey DH, Doornbos JF: Chemodectoma of the temporal bone: A comparison of surgical and radiotherapeutic results. Int J Radiation Oncology Biol Phys 14:643-648, 1988. Zinreich ES, Lee D: Radiotherapy for the treatment of paragangiomas in the temporal bone. Ear Nose Throat J 65:181-4, 1986. Grand Rounds Archive | Department Home page BCM Public | BCM Intranet | Privacy Notices | Contact BCM | BCM Site Map | ©2001-2006 Baylor College of Medicine
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