History

The clinical course of temporal bone glomus tumors reflects their slow growth and paucity of symptoms. Often, a significant delay in diagnosis occurs, and tumors may be large when first identified.

The most common symptoms are conductive hearing loss (69%) and pulsatile tinnitus (55%).^[3] Other aural signs and symptoms are ear fullness, otorrhea, hemorrhage, bruit, and the presence of a middle ear mass. Significant ear pain is uncommon. Involvement of the inner ear produces vertigo and sensorineural hearing loss.

Cranial nerve involvement produces hoarseness and dysphagia. The presence of jugular foramen syndrome (paresis of cranial nerves IX-XI) is pathognomonic for this tumor, but it usually follows 1 year after the initial symptoms of hearing loss and pulsatile tinnitus. Less commonly, glomus tumors produce facial nerve palsy, hypoglossal nerve palsy, or Horner syndrome.

Headache, hydrocephalus, and elevated intracranial pressure may be produced by intracranial extension of the tumor. Ataxia and brainstem symptoms may also develop. Involvement of the dural sinuses may mimic sinus thrombosis.

In about 2-4% of cases, the first or leading symptoms are hypertension and tachycardia (pheochromocytoma-like symptoms) produced by catecholamines, norepinephrine, or dopamine excreted by the tumor.^[3] Also, somatostatin, vasoactive intestinal polypeptide (VIP), calcitonin, and neuron-specific enolase may be produced by the tumor. Other related symptoms include headache, perspiration, pallor, and nausea.^[3]

Differential Diagnoses

Ibrahim R, Ammori MB, Yianni J, Grainger A, Rowe J, Radatz M. Gamma Knife radiosurgery for glomus jugulare tumors: a single-center series of 75 cases. J Neurosurg. 2017 May. 126 (5):1488-1497. [QxMD MEDLINE Link].
Forbes JA, Brock AA, Ghiassi M, Thompson RC, Haynes DS, Tsai BS. Jugulotympanic paragangliomas: 75 years of evolution in understanding. Neurosurg Focus. 2012 Aug. 33(2):E13. [QxMD MEDLINE Link].
Kumar K, Ahmed R, Bajantri B, Singh A, Abbas H, Dejesus E, et al. Tumors Presenting as Multiple Cranial Nerve Palsies. Case Rep Neurol. 2017 Jan-Apr. 9 (1):54-61. [QxMD MEDLINE Link].
Cândido DNC, de Oliveira JG, Borba LAB. Microsurgical resection of glomus jugulare tumors with facial nerve reconstruction: 3-dimensional operative video. Oper Neurosurg (Hagerstown). 2019 Jan 1. 16 (1):E1. [QxMD MEDLINE Link].
Fernández-de Thomas RJ, De Jesus O. Glomus Jugulare. https://www.ncbi.nlm.nih.gov/books/NBK560489/. 2022 Jan. [QxMD MEDLINE Link]. [Full Text].
Rosenwasser H. Carotid body tumor of the middle ear and mastoid. 1945. 41:64-67.
Okada O, Demitsu T, Manabe M, Yoneda K. A case of multiple subungual glomus tumors associated with neurofibromatosis type 1. J Dermatol. 1999 Aug. 26(8):535-7. [QxMD MEDLINE Link].
Havekes B, van der Klaauw AA, Hoftijzer HC, Jansen JC, van der Mey AG, Vriends AH. Reduced quality of life in patients with head-and-neck paragangliomas. Eur J Endocrinol. 2008 Feb. 158(2):247-53. [QxMD MEDLINE Link].
Semaan MT, Megerian CA. Current assessment and management of glomus tumors. Curr Opin Otolaryngol Head Neck Surg. 2008 Oct. 16(5):420-6. [QxMD MEDLINE Link].
Sharma MS, Gupta A, Kale SS, Agrawal D, Mahapatra AK, Sharma BS. Gamma knife radiosurgery for glomus jugulare tumors: Therapeutic advantages of minimalism in the skull base. Neurol India. 2008 Jan-Mar. 56(1):57-61. [QxMD MEDLINE Link].
Chou T, Pan SC, Shieh SJ, Lee JW, Chiu HY, Ho CL. Glomus Tumor: Twenty-Year Experience and Literature Review. Ann Plast Surg. 2016 Mar. 76 Suppl 1:S35-40. [QxMD MEDLINE Link].
Motegi H, Terasaka S, Yamaguchi S, Kobayashi H, Asaoka K, Iwasaki Y. [A case of catecholamine-secreting glomus jugulare tumor: treatment strategy and perioperative management]. No Shinkei Geka. 2008 Nov. 36(11):1029-34. [QxMD MEDLINE Link].
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. 1994 Jun. 80(6):1091-4. [QxMD MEDLINE Link].
Wanna GB, Sweeney AD, Haynes DS, Carlson ML. Contemporary management of jugular paragangliomas. Otolaryngol Clin North Am. Apr 2015. 48(2):331-41. [QxMD MEDLINE Link]. [Full Text].
Ehret F, Kufeld M, Fürweger C, et al. Image-guided robotic radiosurgery for glomus jugulare tumors - multicenter experience and review of the literature. Head Neck. 2021 Jan. 43 (1):35-47. [QxMD MEDLINE Link].
Sallabanda K, Barrientos H, Isernia Romero DA, et al. Long-term outcomes after radiosurgery for glomus jugulare tumors. Tumori. 2018 Aug. 104 (4):300-06. [QxMD MEDLINE Link].
Wakefield DV, Venable GT, VanderWalde NA, Michael LM 2nd, Sorenson JM, Robertson JH, et al. Comparative Neurologic Outcomes of Salvage and Definitive Gamma Knife Radiosurgery for Glomus Jugulare: A 20-Year Experience. J Neurol Surg B Skull Base. 2017 Jun. 78 (3):251-255. [QxMD MEDLINE Link].
Winford TW, Dorton LH, Browne JD, Chan MD, Tatter SB, Oliver ER. Stereotactic Radiosurgical Treatment of Glomus Jugulare Tumors. Otol Neurotol. 2017 Apr. 38 (4):555-562. [QxMD MEDLINE Link].
Dobberpuhl MR, Maxwell S, Feddock J, St Clair W, Bush ML. Treatment Outcomes for Single Modality Management of Glomus Jugulare Tumors With Stereotactic Radiosurgery. Otol Neurotol. 2016 Oct. 37 (9):1406-10. [QxMD MEDLINE Link].
Schuster D, Sweeney AD, Stavas MJ, Tawfik KY, Attia A, Cmelak AJ, et al. Initial radiographic tumor control is similar following single or multi-fractionated stereotactic radiosurgery for jugular paragangliomas. Am J Otolaryngol. 2016 May-Jun. 37 (3):255-8. [QxMD MEDLINE Link].
Ong V, Bourcier AJ, Florence TJ, et al. Stereotactic radiosurgery for glomus jugulare tumors: systematic review and meta-analysis. World Neurosurg. 2022 Jun. 162:e49-e57. [QxMD MEDLINE Link].
Lior U, Rotem H, Uzi N, et al. LINAC radiosurgery for glomus jugulare tumors: retrospective - cohort study of 23 patients. Acta Neurochir (Wien). 2020 Apr. 162 (4):839-44. [QxMD MEDLINE Link].
P. Masson. Le glomus neurmoyo-arterial des regions tactiles et ses tumeurs. Lyon Chir. 1924. 16:257-280.
Sen C, Hague K, Kacchara R, Jenkins A, Das S, Catalano P. Jugular foramen: microscopic anatomic features and implications for neural preservation with reference to glomus tumors involving the temporal bone. Neurosurgery. 2001 Apr. 48(4):838-47; discussion 847-8. [QxMD MEDLINE Link].
Rodríguez-Justo M, Aramburu-González JA, Santonja C. Glomangiosarcoma of abdominal wall. Virchows Arch. 2001 Apr. 438(4):418-20. [QxMD MEDLINE Link].
Singh VK, Badhwar S, D'Souza J, Indrajit IK. Glomus Tympanicum. Med J Armed Forces India. 2004 Apr. 60 (2):200-3. [QxMD MEDLINE Link].
Moffat DA, Hardy DG. Surgical management of large glomus jugulare tumours: infra- and trans-temporal approach. J Laryngol Otol. 1989 Dec. 103(12):1167-80. [QxMD MEDLINE Link].
Lim M, Gibbs IC, Adler JR, Chang SD. Efficacy and safety of stereotactic radiosurgery for glomus jugulare tumors. Neurosurg Focus. 2004 Aug 15. 17(2):E11:E11. [QxMD MEDLINE Link]. [Full Text].
Lior U, Rotem H, Uzi N, Roberto S. LINAC radiosurgery for glomus jugulare tumors: retrospective - cohort study of 23 patients. Acta Neurochir (Wien). Apr 2020. 162(4):839-844. [QxMD MEDLINE Link]. [Full Text].
Carlson ML, Sweeney AD, Wanna GB, Netterville JL, Haynes DS. Natural history of glomus jugulare: a review of 16 tumors managed with primary observation. Otolaryngol Head Neck Surg. 2015 Jan. 152 (1):98-105. [QxMD MEDLINE Link].
Chen PG, Nguyen JH, Payne SC, Sheehan JP, Hashisaki GT. Treatment of glomus jugulare tumors with gamma knife radiosurgery. Laryngoscope. September 2010. 120:1856-1862. [QxMD MEDLINE Link].
Jacob JT, Pollock BE, Carlson ML, Driscoll CL, Link MJ. Stereotactic radiosurgery in the management of vestibular schwannoma and glomus jugulare: indications, techniques, and results. Otolaryngol Clin North Am. 2015 Jun. 48 (3):515-26. [QxMD MEDLINE Link].
Li D, Zeng XJ, Hao SY, Wang L, Tang J, Xiao XR, et al. Less-aggressive surgical management and long-term outcomes of jugular foramen paragangliomas: a neurosurgical perspective. J Neurosurg. 2016 Nov. 125 (5):1143-1154. [QxMD MEDLINE Link].
Sharma M, Meola A, Bellamkonda S, Jia X, Montgomery J, Chao ST, et al. Long-Term Outcome Following Stereotactic Radiosurgery for Glomus Jugulare Tumors: A Single Institution Experience of 20 Years. Neurosurgery. 2018 Nov 1. 83 (5):1007-1014. [QxMD MEDLINE Link].
Patel NS, Link MJ, Driscoll CLW, Pollock BE, Lohse CM, Carlson ML. Hearing Outcomes After Stereotactic Radiosurgery for Jugular Paraganglioma. Otol Neurotol. 2018 Jan. 39 (1):99-105. [QxMD MEDLINE Link].
Sheehan JP, Tanaka S, Link MJ, et al. Gamma Knife surgery for the management of glomus tumors: a multicenter study. J Neurosurg. 2012 Aug. 117(2):246-54. [QxMD MEDLINE Link].
Hafez RF, Morgan MS, Fahmy OM. An intermediate term benefits and complications of gamma knife surgery in management of glomus jugulare tumor. World J Surg Oncol. 2016 Feb 15. 14 (1):36. [QxMD MEDLINE Link].
El Majdoub F, Hunsche S, Igressa A, Kocher M, Sturm V, Maarouf M. Stereotactic LINAC-Radiosurgery for Glomus Jugulare Tumors: A Long-Term Follow-Up of 27 Patients. PLoS One. 2015. 10 (6):e0129057. [QxMD MEDLINE Link].
Sallabanda K, Barrientos H, Isernia Romero DA, Vargas C, Gutierrez Diaz JA, Peraza C, et al. Long-term outcomes after radiosurgery for glomus jugulare tumors. Tumori. 2018 Aug. 104 (4):300-306. [QxMD MEDLINE Link].
Gaynor BG, Elhammady MS, Jethanamest D, Angeli SI, Aziz-Sultan MA. Incidence of cranial nerve palsy after preoperative embolization of glomus jugulare tumors using Onyx. J Neurosurg. 2014 Feb. 120(2):377-81. [QxMD MEDLINE Link].
Ehret F, Kufeld M, Fürweger C, et al. Single-session image-guided robotic radiosurgery and quality of life for glomus jugulare tumors. Head Neck. 2020 Sep. 42 (9):2421-30. [QxMD MEDLINE Link].
Prabhu SS, DeMonte F. Complete resection of a complex glomus jugulare tumor with extensive venous involvement. Case report. Neurosurg Focus. 2004 Aug 15. 17(2):E12. [QxMD MEDLINE Link].
Li D, Zeng XJ, Hao SY, Wang L, Tang J, Xiao XR, et al. Less-aggressive surgical management and long-term outcomes of jugular foramen paragangliomas: a neurosurgical perspective. J Neurosurg. 2016 Feb 26. 1-12. [QxMD MEDLINE Link].
Al-Mefty O, Teixeira A. Complex tumors of the glomus jugulare: criteria, treatment, and outcome. J Neurosurg. 2002 Dec. 97(6):1356-66. [QxMD MEDLINE Link].
Gigliotti MJ, Hasan S, Liang Y, Chen D, Fuhrer R, Wegner RE. A 10-year experience of linear accelerator-based stereotactic radiosurgery/radiotherapy (SRS/SRT) for paraganglioma: A single institution experience and review of the literature. J Radiosurg SBRT. 2018. 5 (3):183-190. [QxMD MEDLINE Link]. [Full Text].
Eustacchio S, Trummer M, Unger F, Schröttner O, Sutter B, Pendl G. The role of Gamma Knife radiosurgery in the management of glomus jugular tumours. Acta Neurochir Suppl. 2002. 84:91-7. [QxMD MEDLINE Link].
Pokhrel D, Mallory R, Bush M, et al. Feasibility study of stereotactic radiosurgery treatment of glomus jugulare tumors via HyperArc VMAT. Med Dosim. 2022 Jun 15. S0958-3947(22)00050-4. [QxMD MEDLINE Link].

Media Gallery

Lateral view of the initial carotid arteriogram of a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations. Urine catecholamine levels were elevated, and a pheochromocytoma was suspected. She underwent a negative exploratory laparotomy. She subsequently developed palsies of the IX, X, XI, and XII cranial nerves on the right side. A norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension was identified on radiologic and arteriographic imaging. Arrows delineate the tumor blush. The arrowhead demonstrates a branch of the middle meningeal artery providing blood supply to the tumor. This branch was embolized.
A significant decrease of tumor vascular blush (arrows) following embolization of a norepinephrine-secreting glomus jugulare tumor with intracranial and cervical extension.
CT imaging demonstrates the extent of bony destruction (white and black arrows) by the tumor. The normal jugular foramen on the left (arrow head) is shown for comparison. The patient subsequently underwent surgical resection of the extracranial portion of this extensive tumor. The remaining intracranial portion was treated with radiation therapy (54 Gy). Follow-up evaluations, including imaging and laboratory investigations, demonstrated long-term control of both tumor growth and catecholamine production.
Lateral carotid arteriogram obtained 22 years after radiation therapy in a 20-year-old woman who presented in June 1970 with episodic hypertension, headaches, and palpitations.
Corresponding MRI of the tumor depicted in the previous image indicating no evidence of tumor growth over time.

of 5

Glomus Jugulare Tumors Clinical Presentation

History

References

Tables

Contributor Information and Disclosures

What would you like to print?