Skip to main content
SpringerLink
Log in

Head and Neck Paragangliomas

  • Chapter
  • First Online:
Head and Neck Cancer

  • 2496 Accesses

Abstract

Head and neck paragangliomas are rare vascular tumors of neural crest origin that arise from extra-adrenal paraganglia of the autonomic system. The nomenclature of these tumors has been confusing throughout the literature. Most are benign tumors and rarely display malignant features. A majority of paragangliomas are sporadic with 10–30% representing familial cases. Most present as an asymptomatic mass in the head or neck, and MRI with and without contrast is the best initial imaging modality. Surgical resection is the preferred treatment for isolated paragangliomas, but observation versus radiation therapy should be considered for high surgical risk patients or multiple paragangliomas. The potential morbidity of surgical treatment must be weighed with the patient-oriented factors to determine an appropriate course of action. Rehabilitation of the surgical patient is sometimes necessary to assist with voice and swallowing dysfunction, baroreflex failure, and first-bite syndrome.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 169.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 279.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Similar content being viewed by others

References

  1. Martin TPC, Irving RM, Maher ER. The genetics of paragangliomas: a review. Clin Otolaryngol. 2007;32:7–11.

    Article  PubMed  CAS  Google Scholar 

  2. Sevilla MA, Hermsen MA, Weiss MM, et al. Chromosomal changes in sporadic and familial head and neck paragangliomas. Otolaryngol Head Neck Surg. 2009;140:724–9.

    Article  PubMed  Google Scholar 

  3. Martin TPC. What we call them: the nomenclature of head and neck paragangliomas. Clin Otolaryngol. 2006;31:185–6.

    Article  PubMed  CAS  Google Scholar 

  4. Blume-Peytavi U, Adler YD, Geilen CC, et al. Multiple cutaneous glomangioma: a pedigree of four generations and critical analysis of histologic and genetic differences of glomus tumors. J Am Acad Dermatol. 2000;42:633–9.

    Article  PubMed  CAS  Google Scholar 

  5. Barnes L, Everson J, Reichart P, Sidransky D, editors. World Health Organization Classification Of Tumours. Pathology and genetics of tumours of the head and neck. Lyon: IARC; 2005.

    Google Scholar 

  6. Lack E. Tumors of the adrenal gland and extra-adrenal paraganglioma. In: Atlas of tumor pathology, series 3, fasc 19. Washington, DC: Armed Forces Institute of Pathology; 1997.

    Google Scholar 

  7. Rodriguez-Cuevas S, Lau I, Rodriguez HP. High-altitude paragangliomas: diagnostic and therapeutic considerations. Cancer. 1986;57:672–6.

    Article  PubMed  CAS  Google Scholar 

  8. Pellitteri PK, Rinaldo A, Myssiorek D, Jasckson CG, et al. Paragangliomas of the head and neck. Oral Oncol. 2004;40:563–75.

    Article  PubMed  Google Scholar 

  9. Arts HA, Fagan PA. Vagal body tumors. Otolaryngol Head Neck Surg. 1991;105:78–85.

    PubMed  CAS  Google Scholar 

  10. Carney JA. The triad of gastric epitheloid leiomyosarcoma, functioning extra-adrenal paraganglioma, and pulmonary chondroma. Cancer. 1979;43:372–82.

    Google Scholar 

  11. Myssiorek D. Head and neck paragangliomas: an overview. Otolaryngol Clin North Am. 2001;34(5):829–36.

    Article  PubMed  CAS  Google Scholar 

  12. Baysal BE, van Schothorst EM, Farr JE, et al. Repositioning the hereditary paraganglioma critical region on chromosome band 11q23. Hum Genet. 1999;104:219–25.

    Article  PubMed  CAS  Google Scholar 

  13. Neumann HP, Erlic Z, Boedeker CC, et al. Clinical predictors for germline mutations in head and neck paraganglioma patients: cost reduction strategy in genetic diagnostic process as fall-out. Cancer Res. 2009;69(8):3650–6.

    Article  PubMed  CAS  Google Scholar 

  14. Burnichon N, Rohmer V, Amar L, et al. The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. J Clin Endocrinol Metab. 2009;94:2817–27.

    Article  PubMed  CAS  Google Scholar 

  15. Knudson Jr AG. Genetics of human cancer. Annu Rev Genet. 1986;20:231–51.

    Article  PubMed  Google Scholar 

  16. Baysal BE. Genetics of familial paragangliomas: past, present, and future. Otolaryngol Clin North Am. 2001;34(5):863–79.

    Article  PubMed  CAS  Google Scholar 

  17. Kovrishko NM. Postnatal development and structural characteristic of the principle paraganglia in man. Fed Proc. 1964;22(Suppl):740.

    Google Scholar 

  18. Wasserman PG, Savargaonkar P. Paragangliomas: classification, pathology, and differential diagnosis. Otolaryngol Clin North Am. 2001;34(5):845–62.

    Article  PubMed  CAS  Google Scholar 

  19. Coupland RE. Electron microscope observation on the structure of the rat adrenal medulla: the ultrastructure and organization of chromaffin cells in the normal adrenal medulla. Normal innervations. J Anat. 1965;99:231–54.

    PubMed  CAS  Google Scholar 

  20. Mandolis S, Shohet JA, Jackson CG, Glasscock III ME. Malignant glomus tumors. Laryngoscope. 1999;109:30–4.

    Article  Google Scholar 

  21. Patel YC. Somatostatin and its receptor family. Front Neuro­endocrinol. 1999;20:157–98.

    Article  PubMed  CAS  Google Scholar 

  22. Netterville JL, Reilly KM, Rovertson D, et al. Carotid body tumors. A review of 30 patients with 46 tumors. Laryngoscope. 1995;105:115–26.

    Article  PubMed  CAS  Google Scholar 

  23. Lawson W. The neuroendocrine nature of the glomus cells: an experimental, ultrastructural, and histochemical tissue culture study. Laryngoscope. 1980;90:120–44.

    Article  PubMed  CAS  Google Scholar 

  24. Netterville JL, Jackson CG, Miller FR, et al. Vagal paraganglioma: a review of 46 patients treated during a 20-year period. Arch Otolaryngol Head Neck Surg. 1998;124:1133–40.

    PubMed  CAS  Google Scholar 

  25. Weed DT, Netterville JL, O’Malley B. Paragangliomas of the head and neck. In: Harrison LB, Sessions RB, Hong WK, editors. Head and neck cancer: a multidisciplinary approach. Philadelphia, PA: Lippincott-Raven; 1999. p. 777–98.

    Google Scholar 

  26. Myssiorek D, Palestro CJ. 111Indium pentetreotide scanning detection of familial paragangliomas. Laryngoscope. 1998;108:228–31.

    Article  PubMed  CAS  Google Scholar 

  27. Telischi FF, Bustillo A, Whiteman ML, et al. Octreotide scintigraphy for detection of paragangliomas. Otolaryngol Head Neck Surg. 2000;122:358–62.

    Article  PubMed  CAS  Google Scholar 

  28. Cohen SM, Netterville JL. Surgical management of vagal paragangliomas. Oper Tech Otolaryngol. 2004;15:196–200.

    Article  Google Scholar 

  29. Mendenhall WM, Hinerman RW, Amdur RJ, et al. Treatment of paragangliomas with radiation therapy. Otolaryngol Clin North Am. 2001;34(5):1007–20.

    Article  PubMed  CAS  Google Scholar 

  30. 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.

    Article  PubMed  Google Scholar 

  31. Evenson LJ, Mendenhall WM, Parsons JT, et al. Radiotherapy in the management of chemodectomas of the carotid body and glomus vagale. Head Neck. 1998;20:609–13.

    Article  PubMed  CAS  Google Scholar 

  32. Mendenhall WM, Parsons JT, Stringer SP, et al. Radiotherapy in the management of temporal bone chemodectoma. Skull Base Surg. 1995;5:83–91.

    Article  PubMed  CAS  Google Scholar 

  33. Netterville JL, Stone RE, Lukens LS, et al. Silastic medialization and arytenoid adduction: the Vanderbilt experience. A review of 116 ­phonosurgical procedures. Ann Otol Rhinol Laryngol. 1993;102:413–24.

    PubMed  CAS  Google Scholar 

  34. Netterville JL, Vrabec JT. Unilateral palatal adhesion for paralysis after high vagal injury. Arch Otolaryngol Head Neck Surg. 1994;120:218–21.

    PubMed  CAS  Google Scholar 

Download references

Author information

Authors and Affiliations

  1. Department of Otolaryngology – Head and Neck Surgery, The James Cancer Hospital and Solove Research Institute at the Ohio State University Comprehensive Cancer Center, 456 W. 10th Ave., Suite 4a, Columbus, OH, 43210, USA

    Matthew O. Old

Authors
  1. Matthew O. Old
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. James L. Netterville
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Matthew O. Old .

Editor information

Editors and Affiliations

  1. , Dept of Radio-Oncology, Genolier Swiss Medical Network, 4 Route du Muids, Genolier, CH-1272, Switzerland

    Jacques Bernier

Rights and permissions

Reprints and permissions

Copyright information

© 2011 Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Old, M.O., Netterville, J.L. (2011). Head and Neck Paragangliomas. In: Bernier, J. (eds) Head and Neck Cancer. Springer, New York, NY. https://doi.org/10.1007/978-1-4419-9464-6_40

Download citation

  • DOI: https://doi.org/10.1007/978-1-4419-9464-6_40

  • Published:

  • Publisher Name: Springer, New York, NY

  • Print ISBN: 978-1-4419-9463-9

  • Online ISBN: 978-1-4419-9464-6

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics

Search

Navigation