Advertisement

Glomus Tumors

  • Jenny Yan
  • Kristin Janson Redmond
Chapter

Abstract

Glomus tumors, or paragangliomas, are tumors of neuroendocrine origin that often present in the head and neck near the carotid body, jugulotympanic and vagal paraganglia. Parasympathetic derived tumor types are often benign and non-catecholamine secreting but can exert mass effects on nearby structures that result in loss of cranial nerve function, most commonly CN VIII and CN IX. Diagnosis of paragangliomas is achieved through biochemical screens of high catecholamine levels, MRI, angiograms, and biopsy showing Zellballen nesting patterns. Historical treatment has been surgical removal but can result in cranial nerve damage and other complications. Stereotactic radiosurgery (SRS) has emerged as a new preferred method of treatment, especially for small <3 cm tumors and has shown high tumor and symptom control rates in multiple studies. Suggested dosing parameters and contouring are outlined in the following chapter along with reviews of SRS studies in the last 17 years. Follow-up of paragangliomas includes lifelong surveillance using biochemical screening or imaging tests.

Keywords

Paragangliomas Stereotactic radiosurgery Glomus jugulare Carotid body tumor Jugulotympanic tumor 

Abbreviations

3DCRT

Three-dimensional conformal radiation therapy

GK

Gamma Knife

GTR

Gross total resection

HSRT

Hypofractionated stereotactic radiation therapy

IMRT

Intensity-modulated radiation therapy

LINAC

Linear accelerator

MEN2B

Multiple endocrine neoplasia type 2

MIBG

Meta-iodobenzylguanidine

NF1

Neurofibromatosis type 1

PET

Positron-emission tomography

RT

Radiation therapy

SRS

Stereotactic radiosurgery

STR

Subtotal resection

VHL

Von Hippel-Lindau

References

  1. 1.
    Lee JH, Barich F, Karnell LH, et al. National Cancer Data Base report on malignant paragangliomas of the head and neck. Cancer. 2002;94(3):730–7.PubMedCrossRefGoogle Scholar
  2. 2.
    Wegner RE, Rodriguez KD, Heron DE, et al. Linac-based stereotactic body radiation therapy for treatment of glomus jugulare tumors. Radiother Oncol. 2010;97(3):395–8.PubMedCrossRefGoogle Scholar
  3. 3.
    Erickson D, Kudva YC, Ebersold MJ, et al. Benign paragangliomas: clinical presentation and treatment outcomes in 236 patients. J Clin Endocrinol Metab. 2001;86(11):5210–6.PubMedCrossRefGoogle Scholar
  4. 4.
    Ramlawi B, David EA, Kim MP, et al. Contemporary surgical management of cardiac paragangliomas. Ann Thorac Surg. 2012;93(6):1972–6.PubMedCrossRefGoogle Scholar
  5. 5.
    Young WF Jr. Paragangliomas: clinical overview. Ann N Y Acad Sci. 2006;1073:21–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Boedeker CC, Neumann HP, Maier W, et al. Malignant head and neck paragangliomas in SDHB mutation carriers. Otolaryngol Head Neck Surg. 2007;137(1):126–9.PubMedCrossRefGoogle Scholar
  7. 7.
    Fishbein L, Merrill S, Fraker DL, et al. Inherited mutations in pheochromocytoma and paraganglioma: why all patients should be offered genetic testing. Ann Surg Oncol. 2013;20(5):1444–50.PubMedPubMedCentralCrossRefGoogle Scholar
  8. 8.
    Burnichon N, Briere JJ, Libe R, et al. SDHA is a tumor suppressor gene causing paraganglioma. Hum Mol Genet. 2010;19(15):3011–20.PubMedPubMedCentralCrossRefGoogle Scholar
  9. 9.
    Lee JA, Duh QY. Sporadic paraganglioma. World J Surg. 2008;32(5):683–7.PubMedCrossRefGoogle Scholar
  10. 10.
    Fliedner SM, Lehnert H, Pacak K. Metastatic paraganglioma. Semin Oncol. 2010;37(6):627–37.PubMedPubMedCentralCrossRefGoogle Scholar
  11. 11.
    Lenders JW, Duh QY, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: an endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915–42.PubMedCrossRefGoogle Scholar
  12. 12.
    Fayad JN, Keles B, Brackmann DE. Jugular foramen tumors: clinical characteristics and treatment outcomes. Otol Neurotol. 2010;31(2):299–305.PubMedCrossRefGoogle Scholar
  13. 13.
    Stoeckli SJ, Schuknecht B, Alkadhi H, et al. Evaluation of paragangliomas presenting as a cervical mass on color-coded Doppler sonography. Laryngoscope. 2002;112(1):143–6.PubMedCrossRefGoogle Scholar
  14. 14.
    Navarro Martin A, Maitz A, Grills IS, 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. 2010;12(1):55–62.PubMedCrossRefGoogle Scholar
  15. 15.
    Olsen WL, Dillon WP, Kelly WM, et al. MR imaging of paragangliomas. AJR Am J Roentgenol. 1987;148(1):201–4.PubMedCrossRefGoogle Scholar
  16. 16.
    Rao AB, Koeller KK, Adair CF. From the archives of the AFIP. Paragangliomas of the head and neck: radiologic-pathologic correlation. Armed Forces Institute of Pathology. Radiographics. 1999;19(6):1605–32.PubMedCrossRefGoogle Scholar
  17. 17.
    Monabati A, Hodjati H, Kumar PV. Cytologic findings in carotid body tumors. Acta Cytol. 2002;46(6):1101–4.PubMedCrossRefGoogle Scholar
  18. 18.
    Duet M, Sauvaget E, Petelle B, et al. Clinical impact of somatostatin receptor scintigraphy in the management of paragangliomas of the head and neck. J Nucl Med. 2003;44(11):1768–74.Google Scholar
  19. 19.
    Namba K, Niimi Y, Song J, et al. Use of Dyna-CT angiography in neuroendovascular decision-making: a case report. Interv Neuroradiol. 2009;15(1):68–72.CrossRefGoogle Scholar
  20. 20.
    Intenzo CM, Jabbour S, Lin HC, et al. Scintigraphic imaging of body neuroendocrine tumors. Radiographics. 2007;27(5):1355–69.PubMedCrossRefGoogle Scholar
  21. 21.
    Timmers HJ, Chen CC, Carrasquillo JA, et al. Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-FDG) positron emission tomography. J Natl Cancer Inst. 2012;104(9):700–8.PubMedPubMedCentralCrossRefGoogle Scholar
  22. 22.
    Dorobisz K, Dorobisz T, Temporale H, et al. Diagnostic and therapeutic difficulties in carotid body paragangliomas, based on clinical experience and a review of the literature. Adv Clin Exp Med. 2016;25(6):1173–7.PubMedCrossRefGoogle Scholar
  23. 23.
    Offergeld C, Brase C, Yaremchuk S, et al. Head and neck paragangliomas: clinical and molecular genetic classification. Clinics (Sao Paulo). 2012;68(Suppl 1):19–28.CrossRefGoogle Scholar
  24. 24.
    Schuster D, Sweeney AD, Stavas MJ, et al. Initial radiographic tumor control is similar following single or multi-fractionated stereotactic radiosurgery for jugular paragangliomas. Am J Otolaryngol. 2016;37(3):255–8.PubMedCrossRefGoogle Scholar
  25. 25.
    Varma A, Nathoo N, Neyman G, et al. Gamma knife radiosurgery for glomus jugulare tumors: volumetric analysis in 17 patients. Neurosurgery. 2006;59(5):1030–6. discussion 1036PubMedCrossRefGoogle Scholar
  26. 26.
    Sethi RV, Sethi RK, Herr MW, et al. Malignant head and neck paragangliomas: treatment efficacy and prognostic indicators. Am J Otolaryngol. 2013;34(5):431–8.PubMedCrossRefGoogle Scholar
  27. 27.
    Moskovic DJ, Smolarz JR, Stanley D, et al. Malignant head and neck paragangliomas: is there an optimal treatment strategy? Head Neck Oncol. 2010;2:23.PubMedPubMedCentralCrossRefGoogle Scholar
  28. 28.
    Green JD Jr, Brackmann DE, Nguyen CD, et al. Surgical management of previously untreated glomus jugulare tumors. Laryngoscope. 1994;104(8 Pt 1):917–21.PubMedGoogle Scholar
  29. 29.
    Litle VR, Reilly LM, Ramos TK. Preoperative embolization of carotid body tumors: when is it appropriate? Ann Vasc Surg. 1996;10(5):464–8.PubMedCrossRefGoogle Scholar
  30. 30.
    Valavanis A. Preoperative embolization of the head and neck: indications, patient selection, goals, and precautions. AJNR Am J Neuroradiol. 1986;7(5):943–52.PubMedGoogle Scholar
  31. 31.
    Suarez C, Rodrigo JP, Bodeker CC, et al. Jugular and vagal paragangliomas: systematic study of management with surgery and radiotherapy. Head Neck. 2013;35(8):1195–204.PubMedCrossRefGoogle Scholar
  32. 32.
    Feigl GC, Horstmann GA. Intracranial glomus jugulare tumors: volume reduction with Gamma Knife surgery. J Neurosurg. 2006;105(Suppl):161–8.PubMedGoogle Scholar
  33. 33.
    Gerosa M, Visca A, Rizzo P, et al. Glomus jugulare tumors: the option of gamma knife radiosurgery. Neurosurgery. 2006;59(3):561–9. discussion 561–9PubMedCrossRefGoogle Scholar
  34. 34.
    Dobberpuhl MR, Maxwell S, Feddock J, et al. Treatment outcomes for single modality management of glomus jugulare tumors with stereotactic radiosurgery. Otol Neurotol. 2016;37(9):1406–10.PubMedPubMedCentralCrossRefGoogle Scholar
  35. 35.
    Ibrahim R, Ammori MB, Yianni J, et al. Gamma Knife radiosurgery for glomus jugulare tumors: a single-center series of 75 cases. J Neurosurg. 2016;8:1–10.Google Scholar
  36. 36.
    Scheick SM, Morris CG, Amdur RJ, et al. Long-term outcomes after radiosurgery for temporal bone paragangliomas. Am J Clin Oncol. 2018;41(3):223–6.PubMedGoogle Scholar
  37. 37.
    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;14(1):36.PubMedPubMedCentralCrossRefGoogle Scholar
  38. 38.
    Martin IT, Avila Rdel M, Herrera MZ, et al. Role of radiosurgery in the management of glomus tumors. Head Neck. 2016;38(Suppl 1):E798–804.PubMedCrossRefGoogle Scholar
  39. 39.
    El Majdoub F, Hunsche S, Igressa A, et al. Stereotactic LINAC-radiosurgery for glomus jugulare tumors: a long-term follow-up of 27 patients. PLoS One. 2015;10(6):e0129057.PubMedPubMedCentralCrossRefGoogle Scholar
  40. 40.
    Liscak R, Urgosik D, Chytka T, et al. Leksell Gamma Knife radiosurgery of the jugulotympanic glomus tumor: long-term results. J Neurosurg. 2014;121(Suppl):198–202.PubMedGoogle Scholar
  41. 41.
    Sager O, Beyzadeoglu M, Dincoglan F, et al. Evaluation of linear accelerator-based stereotactic radiosurgery in the management of glomus jugulare tumors. Tumori. 2014;100(2):184–8.PubMedCrossRefGoogle Scholar
  42. 42.
    Chun SG, Nedzi LA, Choe KS, et al. A retrospective analysis of tumor volumetric responses to five-fraction stereotactic radiotherapy for paragangliomas of the head and neck (glomus tumors). Stereotact Funct Neurosurg. 2014;92(3):153–9.PubMedCrossRefGoogle Scholar
  43. 43.
    de Andrade EM, Brito J, Mario SD, et al. Stereotactic radiosurgery for the treatment of glomus jugulare tumors. Surg Neurol Int. 2013;4(Suppl 6):S429–35.PubMedPubMedCentralCrossRefGoogle Scholar
  44. 44.
    Hurmuz P, Cengiz M, Ozyigit G, et al. Robotic stereotactic radiosurgery in patients with unresectable glomus jugulare tumors. Technol Cancer Res Treat. 2013;12(2):109–13.PubMedCrossRefGoogle Scholar
  45. 45.
    Genc A, Bicer A, Abacioglu U, et al. Gamma knife radiosurgery for the treatment of glomus jugulare tumors. J Neuro-Oncol. 2010;97(1):101–8.CrossRefGoogle Scholar
  46. 46.
    Miller JP, Semaan M, Einstein D, et al. Staged Gamma Knife radiosurgery after tailored surgical resection: a novel treatment paradigm for glomus jugulare tumors. Stereotact Funct Neurosurg. 2009;87(1):31–6.PubMedCrossRefGoogle Scholar
  47. 47.
    Ganz JC, Abdelkarim K. Glomus jugulare tumours: certain clinical and radiological aspects observed following gamma knife radiosurgery. Acta Neurochir. 2009;151(5):423–6.PubMedCrossRefGoogle Scholar
  48. 48.
    Sharma MS, Gupta A, Kale SS, et al. Gamma knife radiosurgery for glomus jugulare tumors: therapeutic advantages of minimalism in the skull base. Neurol India. 2008;56(1):57–61.PubMedCrossRefGoogle Scholar
  49. 49.
    Lim M, Bower R, Nangiana JS, et al. Radiosurgery for glomus jugulare tumors. Technol Cancer Res Treat. 2007;6(5):419–23.PubMedCrossRefGoogle Scholar
  50. 50.
    Henzel M, Hamm K, Gross MW, et al. Fractionated stereotactic radiotherapy of glomus jugulare tumors. Local control, toxicity, symptomatology, and quality of life. Strahlenther Onkol. 2007;183(10):557–62.PubMedCrossRefGoogle Scholar
  51. 51.
    Poznanovic SA, Cass SP, Kavanagh BD. Short-term tumor control and acute toxicity after stereotactic radiosurgery for glomus jugulare tumors. Otolaryngol Head Neck Surg. 2006;134(3):437–42.PubMedCrossRefGoogle Scholar
  52. 52.
    Bitaraf MA, Alikhani M, Tahsili-Fahadan P, et al. Radiosurgery for glomus jugulare tumors: experience treating 16 patients in Iran. J Neurosurg. 2006;105(Suppl):168–74.PubMedGoogle Scholar
  53. 53.
    Feigenberg SJ, Mendenhall WM, Hinerman RW, et al. Radiosurgery for paraganglioma of the temporal bone. Head Neck. 2002;24(4):384–9.PubMedCrossRefGoogle Scholar
  54. 54.
    Sheehan J, Kondziolka D, Flickinger J, et al. Gamma knife surgery for glomus jugulare tumors: an intermediate report on efficacy and safety. J Neurosurg. 2005;102(Suppl):241–6.PubMedCrossRefGoogle Scholar
  55. 55.
    Pollock BE. Stereotactic radiosurgery in patients with glomus jugulare tumors. Neurosurg Focus. 2004;17(2):E10.PubMedCrossRefGoogle Scholar
  56. 56.
    Maarouf M, Voges J, Landwehr P, et al. Stereotactic linear accelerater-based radiosurgery for the treatment of patients with glomus jugulare tumors. Cancer. 2003;97(4):1093–8.PubMedCrossRefGoogle Scholar
  57. 57.
    Saringer W, Khayal H, Ertl A, et al. Efficiency of gamma knife radiosurgery in the treatment of glomus jugulare tumors. Minim Invasive Neurosurg. 2001;44(3):141–6.PubMedCrossRefGoogle Scholar
  58. 58.
    Jordan JA, Roland PS, McManus C, et al. Stereotastic radiosurgery for glomus jugulare tumors. Laryngoscope. 2000;110(1):35–8.PubMedCrossRefGoogle Scholar
  59. 59.
    Liscak R, Vladyka V, Wowra B, et al. Gamma Knife radiosurgery of the glomus jugulare tumour – early multicentre experience. Acta Neurochir. 1999;141(11):1141–6.PubMedCrossRefGoogle Scholar
  60. 60.
    Guss ZD, Batra S, Limb CJ, et al. Radiosurgery of glomus jugulare tumors: a meta-analysis. Int J Radiat Oncol Biol Phys. 2011;81(4):e497–502.PubMedPubMedCentralCrossRefGoogle Scholar
  61. 61.
    Lee JS, DuBois SG, Coccia PF, et al. Increased risk of second malignant neoplasms in adolescents and young adults with cancer. Cancer. 2016;122(1):116–23.PubMedCrossRefGoogle Scholar
  62. 62.
    Chen AM, Farwell DG, Luu Q, et al. Evaluation of the planning target volume in the treatment of head and neck cancer with intensity-modulated radiotherapy: what is the appropriate expansion margin in the setting of daily image guidance? Int J Radiat Oncol Biol Phys. 2011;81(4):943–9.PubMedCrossRefGoogle Scholar
  63. 63.
    Dupin C, Lang P, Dessard-Diana B, et al. Treatment of head and neck paragangliomas with external beam radiation therapy. Int J Radiat Oncol Biol Phys. 2014;89(2):353–9.PubMedCrossRefGoogle Scholar
  64. 64.
    Hinerman RW, Amdur RJ, Morris CG, et al. Definitive radiotherapy in the management of paragangliomas arising in the head and neck: a 35-year experience. Head Neck. 2008;30(11):1431–8.PubMedCrossRefGoogle Scholar
  65. 65.
    Rogers L, Zhang P, Vogelbaum MA, et al. Intermediate-risk meningioma: initial outcomes from NRG oncology/RTOG-0539. Int J Radiat Oncol Biol Phys. 2017;93(3):S139–40.CrossRefGoogle Scholar
  66. 66.
    Truong MT, Zhang Q, Rosenthal DI, et al. Quality of life and performance status from a substudy conducted within a prospective phase 3 randomized trial of concurrent accelerated radiation plus cisplatin with or without cetuximab for locally advanced head and neck carcinoma: NRG oncology radiation therapy oncology group 0522. Int J Radiat Oncol Biol Phys. 2017;97:687–99.PubMedCrossRefGoogle Scholar
  67. 67.
    Chino JP, Sampson JH, Tucci DL, et al. Paraganglioma of the head and neck: long-term local control with radiotherapy. Am J Clin Oncol. 2009;32(3):304–7.PubMedCrossRefGoogle Scholar
  68. 68.
    Gittleman HR, Lim D, Kattan M, et al. An independently validated nomogram for individualized estimation of survival among patients with newly diagnosed glioblastoma: NRG Oncology/RTOG 0525 and 0825. Int J Radiat Oncol Biol Phys. 2017;96(2):S92.CrossRefGoogle Scholar

Copyright information

© Springer International Publishing AG, part of Springer Nature 2018

Authors and Affiliations

  1. 1.Department of Radiation Oncology and Molecular Radiation ServicesJohns Hopkins UniversityBaltimoreUSA

Personalised recommendations