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Glomus Jugulare Paraganglioma
Karen L. Salzman, MD; Kalen Riley, MD, MBA
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KEY FACTS

  • Terminology

    • Imaging

      • Top Differential Diagnoses

        • Clinical Issues

          TERMINOLOGY

          • Abbreviations

            • Glomus jugulare paraganglioma (GJP)
          • Synonyms

            • Glomus jugulotympanicum paraganglioma, jugular foramen (JF) paraganglioma, chemodectoma
          • Definitions

            • Benign tumor arising from neural crest progenitor cells (glomus bodies) located in & around JF

          IMAGING

          • General Features

            • CT Findings

              • MR Findings

                • Angiographic Findings

                  • Nuclear Medicine Findings

                    • Imaging Recommendations

                      DIFFERENTIAL DIAGNOSIS

                        PATHOLOGY

                        • General Features

                          • Staging, Grading, & Classification

                            • Gross Pathologic & Surgical Features

                              • Microscopic Features

                                CLINICAL ISSUES

                                • Presentation

                                  • Demographics

                                    • Natural History & Prognosis

                                      • Treatment

                                        DIAGNOSTIC CHECKLIST

                                        • Consider

                                          • Image Interpretation Pearls

                                            Selected References

                                            1. Jaiswal SK et al: The utility of 68Ga-DOTATATE PET/CT in localizing primary/metastatic pheochromocytoma and paraganglioma in children and adolescents - a single-center experience. J Pediatr Endocrinol Metab. 34(1):109-19, 2021
                                            2. Manzoor NF et al: Contemporary management of jugular paragangliomas with neural preservation. Otolaryngol Head Neck Surg. 164(2):391-8, 2021
                                            3. Cass ND et al: The changing paradigm of head and neck paragangliomas: what every otolaryngologist needs to know. Ann Otol Rhinol Laryngol. 129(11):1135-43, 2020
                                            4. Ishak C et al: Jugular foramen paragangliomas: preoperative transcatheter particle embolization. J Cerebrovasc Endovasc Neurosurg. 22(4):273-81, 2020
                                            5. Isaacson B et al: Pathophysiology of sensorineural hearing loss in jugular foramen paraganglioma. Laryngoscope. 129(1):67-75, 2019
                                            6. Patel NS et al: Long-term tumor control following stereotactic radiosurgery for jugular paraganglioma using 3D volumetric segmentation. J Neurosurg. 1-9, 2018
                                            7. Patel NS et al: Hearing outcomes after stereotactic radiosurgery for jugular paraganglioma. Otol Neurotol. 39(1):99-105, 2018
                                            8. Chang CA et al: (68)Ga-DOTATATE and (18)F-FDG PET/CT in paraganglioma and pheochromocytoma: utility, patterns and heterogeneity. Cancer Imaging. 16(1):22, 2016
                                            9. Li D et al: Less-aggressive surgical management and long-term outcomes of jugular foramen paragangliomas: a neurosurgical perspective. J Neurosurg. 1-12, 2016
                                            10. Prasad SC et al: Strategies and long-term outcomes in the surgical management of tympanojugular paragangliomas. Head Neck. 38(6):871-85, 2016
                                            11. Schmalbach CE et al: Managing vascular tumors-open approaches. Otolaryngol Clin North Am. 49(3):777-90, 2016
                                            12. Baysal BE et al: 15 Years of paraganglioma: genetics and mechanism of pheochromocytoma-paraganglioma syndromes characterized by germline SDHB and SDHD mutations. Endocr Relat Cancer. 22(4):T71-82, 2015
                                            13. Griauzde J et al: Imaging of vascular lesions of the head and neck. Radiol Clin North Am. 53(1):197-213, 2015
                                            14. Hofman MS et al: Somatostatin receptor imaging with 68Ga DOTATATE PET/CT: clinical utility, normal patterns, pearls, and pitfalls in interpretation. Radiographics. 35(2):500-6, 2015
                                            15. Scheick SM et al: Long-term outcomes after radiosurgery for temporal bone paragangliomas. Am J Clin Oncol. 41(3):223-6, 2015
                                            16. Wanna GB et al: Contemporary management of jugular paragangliomas. Otolaryngol Clin North Am. 48(2):331-41, 2015
                                            17. Gilbo P et al: Radiotherapy for benign head and neck paragangliomas: a 45-year experience. Cancer. 120(23):3738-43, 2014
                                            18. Collins N et al: Contiguous bilateral head and neck paragangliomas in a carrier of the SDHB germline mutation. J Vasc Surg. 55(1):216-9, 2012
                                            19. Shin SH et al: Vertebral artery involvement by tympanojugular paragangliomas: management and outcomes with a proposed addition to the fisch classification. Audiol Neurootol. 17(2):92-104, 2012
                                            20. Sismanis A: Pulsatile tinnitus: contemporary assessment and management. Curr Opin Otolaryngol Head Neck Surg. 19(5):348-57, 2011
                                            21. Aliabadi H et al: Bevacizumab fails to treat temporal paraganglioma: discussion and case illustration. J Neurooncol. 98(3):427-30, 2010
                                            22. Borba LA et al: Surgical management of glomus jugulare tumors: a proposal for approach selection based on tumor relationships with the facial nerve. J Neurosurg. 112(1):88-98, 2010
                                            23. Chen PG et al: Treatment of glomus jugulare tumors with gamma knife radiosurgery. Laryngoscope. 120(9):1856-62, 2010
                                            24. Christie A et al: A comparative review of multidetector CT angiography and MRI in the diagnosis of jugular foramen lesions. Clin Radiol. 65(3):213-7, 2010
                                            25. Fayad JN et al: Jugular foramen tumors: clinical characteristics and treatment outcomes. Otol Neurotol. 31(2):299-305, 2010
                                            26. Karaman E et al: Management of jugular paragangliomas in otolaryngology practice. J Craniofac Surg. 21(1):117-20, 2010
                                            27. Navarro Martín A et al: Successful treatment of glomus jugulare tumours with gamma knife radiosurgery: clinical and physical aspects of management and review of the literature. Clin Transl Oncol. 12(1):55-62, 2010
                                            28. Alaani A et al: The crucial role of imaging in determining the approach to glomus tympanicum tumours. Eur Arch Otorhinolaryngol. 266(6):827-31, 2009
                                            29. Fayad JN et al: Treatment of recurrent and residual glomus jugulare tumors. Skull Base. 19(1):92-8, 2009
                                            30. Guss ZD et al: Radiosurgery for glomus jugulare: history and recent progress. Neurosurg Focus. 27(6):E5, 2009
                                            31. Miller JP et al: Radiosurgery for glomus jugulare tumors. Otolaryngol Clin North Am. 42(4):689-706, 2009
                                            32. Boedeker CC et al: Malignant head and neck paragangliomas in SDHB mutation carriers. Otolaryngol Head Neck Surg. 137(1):126-9, 2007
                                            33. Piazza P et al: Absence of the contralateral internal carotid artery: a challenge for management of ipsilateral glomus jugulare and glomus vagale tumors. Laryngoscope. 117(8):1333-7, 2007
                                            34. Feigl GC et al: Intracranial glomus jugulare tumors: volume reduction with Gamma Knife surgery. J Neurosurg. 105 Suppl:161-7, 2006
                                            35. Hirunpat S et al: Nasopharyngeal extension of glomus tympanicum: an unusual clinical and imaging manifestation. AJNR Am J Neuroradiol. 27(9):1820-2, 2006
                                            36. Robinson DH: Vascular lesions of the skull base: endovascular prospective for the otolaryngologist. Otolaryngol Clin North Am. 38(4):737-71, 2005
                                            37. Mafee MF et al: Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and simulating lesions. Role of MR imaging. Radiol Clin North Am. 38(5):1059-76, 2000
                                            38. Weissman JL et al: Imaging of tinnitus: a review. Radiology. 216(2):342-9, 2000
                                            39. Rao AB et al: From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 19(6):1605-32, 1999
                                            40. Lustig LR et al: The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation. Am J Otol. 17(4):658-68, 1996
                                            41. Vogl TJ et al: Glomus tumors of the skull base: combined use of MR angiography and spin-echo imaging. Radiology. 192(1):103-10, 1994
                                            42. Weber AL et al: Radiologic evaluation of the jugular foramen. Anatomy, vascular variants, anomalies, and tumors. Neuroimaging Clin N Am. 4(3):579-98, 1994
                                            43. Gulya AJ: The glomus tumor and its biology. Laryngoscope. 103(11 Pt 2 Suppl 60):7-15, 1993
                                            44. Olsen WL et al: MR imaging of paragangliomas. AJR Am J Roentgenol. 148(1):201-4, 1987
                                            Related Anatomy
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                                            Related Differential Diagnoses
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                                            References
                                            Tables

                                            Tables

                                            KEY FACTS

                                            • Terminology

                                              • Imaging

                                                • Top Differential Diagnoses

                                                  • Clinical Issues

                                                    TERMINOLOGY

                                                    • Abbreviations

                                                      • Glomus jugulare paraganglioma (GJP)
                                                    • Synonyms

                                                      • Glomus jugulotympanicum paraganglioma, jugular foramen (JF) paraganglioma, chemodectoma
                                                    • Definitions

                                                      • Benign tumor arising from neural crest progenitor cells (glomus bodies) located in & around JF

                                                    IMAGING

                                                    • General Features

                                                      • CT Findings

                                                        • MR Findings

                                                          • Angiographic Findings

                                                            • Nuclear Medicine Findings

                                                              • Imaging Recommendations

                                                                DIFFERENTIAL DIAGNOSIS

                                                                  PATHOLOGY

                                                                  • General Features

                                                                    • Staging, Grading, & Classification

                                                                      • Gross Pathologic & Surgical Features

                                                                        • Microscopic Features

                                                                          CLINICAL ISSUES

                                                                          • Presentation

                                                                            • Demographics

                                                                              • Natural History & Prognosis

                                                                                • Treatment

                                                                                  DIAGNOSTIC CHECKLIST

                                                                                  • Consider

                                                                                    • Image Interpretation Pearls

                                                                                      Selected References

                                                                                      1. Jaiswal SK et al: The utility of 68Ga-DOTATATE PET/CT in localizing primary/metastatic pheochromocytoma and paraganglioma in children and adolescents - a single-center experience. J Pediatr Endocrinol Metab. 34(1):109-19, 2021
                                                                                      2. Manzoor NF et al: Contemporary management of jugular paragangliomas with neural preservation. Otolaryngol Head Neck Surg. 164(2):391-8, 2021
                                                                                      3. Cass ND et al: The changing paradigm of head and neck paragangliomas: what every otolaryngologist needs to know. Ann Otol Rhinol Laryngol. 129(11):1135-43, 2020
                                                                                      4. Ishak C et al: Jugular foramen paragangliomas: preoperative transcatheter particle embolization. J Cerebrovasc Endovasc Neurosurg. 22(4):273-81, 2020
                                                                                      5. Isaacson B et al: Pathophysiology of sensorineural hearing loss in jugular foramen paraganglioma. Laryngoscope. 129(1):67-75, 2019
                                                                                      6. Patel NS et al: Long-term tumor control following stereotactic radiosurgery for jugular paraganglioma using 3D volumetric segmentation. J Neurosurg. 1-9, 2018
                                                                                      7. Patel NS et al: Hearing outcomes after stereotactic radiosurgery for jugular paraganglioma. Otol Neurotol. 39(1):99-105, 2018
                                                                                      8. Chang CA et al: (68)Ga-DOTATATE and (18)F-FDG PET/CT in paraganglioma and pheochromocytoma: utility, patterns and heterogeneity. Cancer Imaging. 16(1):22, 2016
                                                                                      9. Li D et al: Less-aggressive surgical management and long-term outcomes of jugular foramen paragangliomas: a neurosurgical perspective. J Neurosurg. 1-12, 2016
                                                                                      10. Prasad SC et al: Strategies and long-term outcomes in the surgical management of tympanojugular paragangliomas. Head Neck. 38(6):871-85, 2016
                                                                                      11. Schmalbach CE et al: Managing vascular tumors-open approaches. Otolaryngol Clin North Am. 49(3):777-90, 2016
                                                                                      12. Baysal BE et al: 15 Years of paraganglioma: genetics and mechanism of pheochromocytoma-paraganglioma syndromes characterized by germline SDHB and SDHD mutations. Endocr Relat Cancer. 22(4):T71-82, 2015
                                                                                      13. Griauzde J et al: Imaging of vascular lesions of the head and neck. Radiol Clin North Am. 53(1):197-213, 2015
                                                                                      14. Hofman MS et al: Somatostatin receptor imaging with 68Ga DOTATATE PET/CT: clinical utility, normal patterns, pearls, and pitfalls in interpretation. Radiographics. 35(2):500-6, 2015
                                                                                      15. Scheick SM et al: Long-term outcomes after radiosurgery for temporal bone paragangliomas. Am J Clin Oncol. 41(3):223-6, 2015
                                                                                      16. Wanna GB et al: Contemporary management of jugular paragangliomas. Otolaryngol Clin North Am. 48(2):331-41, 2015
                                                                                      17. Gilbo P et al: Radiotherapy for benign head and neck paragangliomas: a 45-year experience. Cancer. 120(23):3738-43, 2014
                                                                                      18. Collins N et al: Contiguous bilateral head and neck paragangliomas in a carrier of the SDHB germline mutation. J Vasc Surg. 55(1):216-9, 2012
                                                                                      19. Shin SH et al: Vertebral artery involvement by tympanojugular paragangliomas: management and outcomes with a proposed addition to the fisch classification. Audiol Neurootol. 17(2):92-104, 2012
                                                                                      20. Sismanis A: Pulsatile tinnitus: contemporary assessment and management. Curr Opin Otolaryngol Head Neck Surg. 19(5):348-57, 2011
                                                                                      21. Aliabadi H et al: Bevacizumab fails to treat temporal paraganglioma: discussion and case illustration. J Neurooncol. 98(3):427-30, 2010
                                                                                      22. Borba LA et al: Surgical management of glomus jugulare tumors: a proposal for approach selection based on tumor relationships with the facial nerve. J Neurosurg. 112(1):88-98, 2010
                                                                                      23. Chen PG et al: Treatment of glomus jugulare tumors with gamma knife radiosurgery. Laryngoscope. 120(9):1856-62, 2010
                                                                                      24. Christie A et al: A comparative review of multidetector CT angiography and MRI in the diagnosis of jugular foramen lesions. Clin Radiol. 65(3):213-7, 2010
                                                                                      25. Fayad JN et al: Jugular foramen tumors: clinical characteristics and treatment outcomes. Otol Neurotol. 31(2):299-305, 2010
                                                                                      26. Karaman E et al: Management of jugular paragangliomas in otolaryngology practice. J Craniofac Surg. 21(1):117-20, 2010
                                                                                      27. Navarro Martín A et al: Successful treatment of glomus jugulare tumours with gamma knife radiosurgery: clinical and physical aspects of management and review of the literature. Clin Transl Oncol. 12(1):55-62, 2010
                                                                                      28. Alaani A et al: The crucial role of imaging in determining the approach to glomus tympanicum tumours. Eur Arch Otorhinolaryngol. 266(6):827-31, 2009
                                                                                      29. Fayad JN et al: Treatment of recurrent and residual glomus jugulare tumors. Skull Base. 19(1):92-8, 2009
                                                                                      30. Guss ZD et al: Radiosurgery for glomus jugulare: history and recent progress. Neurosurg Focus. 27(6):E5, 2009
                                                                                      31. Miller JP et al: Radiosurgery for glomus jugulare tumors. Otolaryngol Clin North Am. 42(4):689-706, 2009
                                                                                      32. Boedeker CC et al: Malignant head and neck paragangliomas in SDHB mutation carriers. Otolaryngol Head Neck Surg. 137(1):126-9, 2007
                                                                                      33. Piazza P et al: Absence of the contralateral internal carotid artery: a challenge for management of ipsilateral glomus jugulare and glomus vagale tumors. Laryngoscope. 117(8):1333-7, 2007
                                                                                      34. Feigl GC et al: Intracranial glomus jugulare tumors: volume reduction with Gamma Knife surgery. J Neurosurg. 105 Suppl:161-7, 2006
                                                                                      35. Hirunpat S et al: Nasopharyngeal extension of glomus tympanicum: an unusual clinical and imaging manifestation. AJNR Am J Neuroradiol. 27(9):1820-2, 2006
                                                                                      36. Robinson DH: Vascular lesions of the skull base: endovascular prospective for the otolaryngologist. Otolaryngol Clin North Am. 38(4):737-71, 2005
                                                                                      37. Mafee MF et al: Glomus faciale, glomus jugulare, glomus tympanicum, glomus vagale, carotid body tumors, and simulating lesions. Role of MR imaging. Radiol Clin North Am. 38(5):1059-76, 2000
                                                                                      38. Weissman JL et al: Imaging of tinnitus: a review. Radiology. 216(2):342-9, 2000
                                                                                      39. Rao AB et al: From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 19(6):1605-32, 1999
                                                                                      40. Lustig LR et al: The variable relationship between the lower cranial nerves and jugular foramen tumors: implications for neural preservation. Am J Otol. 17(4):658-68, 1996
                                                                                      41. Vogl TJ et al: Glomus tumors of the skull base: combined use of MR angiography and spin-echo imaging. Radiology. 192(1):103-10, 1994
                                                                                      42. Weber AL et al: Radiologic evaluation of the jugular foramen. Anatomy, vascular variants, anomalies, and tumors. Neuroimaging Clin N Am. 4(3):579-98, 1994
                                                                                      43. Gulya AJ: The glomus tumor and its biology. Laryngoscope. 103(11 Pt 2 Suppl 60):7-15, 1993
                                                                                      44. Olsen WL et al: MR imaging of paragangliomas. AJR Am J Roentgenol. 148(1):201-4, 1987