Head and Neck Pathology

, Volume 10, Issue 2, pp 201–205 | Cite as

Malignant Vagal Paraganglioma

  • Erin R. S. HamersleyEmail author
  • Amy Barrows
  • Angel Perez
  • Ashley Schroeder
  • James T. Castle
Sine qua non Radiology-Pathology


Paragangliomas are rare, typically benign neuroendocrine tumors that represent a small portion of head and neck tumors. A small percentage of these are known to have malignant potential. They arise from the carotid body, jugular bulb or vagus nerves. There is limited literature discussing the management of malignant vagal paragangliomas. We present a case of a 25 year old female with a left malignant vagal paraganglioma. The following case presentation will describe the presentation, classic radiologic findings, and management of a malignant vagal paraganglioma along with a review of the literature.


Paraganglioma Carotid body tumor Malignant paraganglioma 


  1. 1.
    Lozano FS, Gomez JL, Mondillo MC, et al. Surgery of vagal paragangliomas: six patients and review of literature. Surg Oncol. 2008;17:281–7.CrossRefPubMedGoogle Scholar
  2. 2.
    Mendenhall WM, Amdur RJ, Vaysberg M, et al. Head and neck paragangliomas. Head Neck. 2010;33:1530–4.CrossRefPubMedGoogle Scholar
  3. 3.
    Netterville JL, Jackson CG, Miller FR, Wanamaker JR, Glasscock ME. Vagal paraganglioma. Arch Otolaryngol Head Neck Surg. 1998;124:1133–40.CrossRefPubMedGoogle Scholar
  4. 4.
    Martin TP, Irving RM, Maher ER. The genetics of paragangliomas: a review. Clin Otolaryngol. 2007;32:7–11.CrossRefPubMedGoogle Scholar
  5. 5.
    Dziegielewski PT, Knox A, Liu R, Hung RW, Harris J. Familial paraganglioma syndrome: applying genetic screening in otolaryngology. J Otolaryngol Head Neck Surg. 2010;29:646–53.Google Scholar
  6. 6.
    Offergeld C, Brase C, Yaremchuk S, et al. Head and neck paragangliomas: clinical and molecular genetic classification. Clinics (Sao Paulo). 2012;67:19–28.CrossRefGoogle Scholar
  7. 7.
    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;32:304–7.CrossRefPubMedGoogle Scholar
  8. 8.
    Kataria T, Bisht SS, Mitra S, et al. Synchronous malignant vagal paraganglioma with contralateral carotid body paraganglioma treated by radiation therapy. Rare Tumors. 2010;2:e21.CrossRefPubMedPubMedCentralGoogle Scholar
  9. 9.
    Sreevatsa M, Srinivasarao R. Three cases of vagal nerve schwannoma and review of literature. Indian J Otolaryngol Head Neck Surg. 2011;63(4):310–2.CrossRefPubMedPubMedCentralGoogle Scholar
  10. 10.
    Shuch B, Ricketts C, Metwalli A, Pacak K, Linehan W. The genetic basis of pheochromocytoma and paraganglioma: implications for management. Urology. 2014;83(6):1225–32.CrossRefPubMedPubMedCentralGoogle Scholar
  11. 11.
    Martucci V, Pacak K. Pheochromocytoma and paraganglioma: diagnosis, genetics, management, and treatment. Curr Probl Cancer. 2014;38(1):7–41.CrossRefPubMedPubMedCentralGoogle Scholar

Copyright information

© Springer Science+Business Media New York (outside the USA) 2015

Authors and Affiliations

  • Erin R. S. Hamersley
    • 1
    • 3
    Email author
  • Amy Barrows
    • 1
  • Angel Perez
    • 1
  • Ashley Schroeder
    • 1
  • James T. Castle
    • 2
  1. 1.Department of OtolaryngologyNaval Medical Center PortsmouthPortsmouthUSA
  2. 2.Department of Anatomic PathologyNaval Medical Center PortsmouthPortsmouthUSA
  3. 3.ChesapeakeUSA

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