Abstract
Vagal paragangliomas are very rare benign vascular tumors of neuroendocrine nature, and are much less frequent than carotid and jugulo-tympanic tumors. The goal of this retrospective study is to review the clinical and genetic findings, surgical treatment, and complications of vagal paragangliomas, as well as to discuss the management options. During the period 1990–2013, 17 patients with vagal paragangliomas were referred to our institution. There were ten patients with isolated tumors, and seven with multicentric paragangliomas. There were nine women and eight men. Mean age of patients was 51.4 years. Five cases had a positive family history of paraganglioma (29.4 %). Germline mutations of SDH genes were found in six of our patients (35.3 %). Many options were considered in the management of vagal paragangliomas. Surgical treatment was performed in 11 young patients (64.7 %) using different approaches: in 4 patients the tumor was resected through a transcervical approach; in 3 through a transcervical-transmandibular approach; in 1 it was resected using a transcervical-transmastoid approach, and in 3 a type A infratemporal fossa approach was performed. In all operated cases, the removal of the tumor led to sacrificing of the vagus nerve. Postoperative hypoglossal nerve deficit was reported in 4 cases (36.3 %). In six elderly patients (35.3 %), we decided to “wait-and-scan” in order to avoid creating greater morbidity than that of the tumor itself. Many factors should be considered in the treatment of vagal paragangliomas: the age and general condition of the patient, the biological behavior of the tumor, tumor size, genetic results, bilaterality, multicentricity, lower cranial nerve function, and of course the potential morbidity of the surgical treatment itself. Rehabilitation and, possibly surgery, are necessary to treat postoperative lower cranial nerve deficits.
Similar content being viewed by others
References
Eriksen C, Girdhar-Gopal H, Lowry LD (1991) Vagal paragangliomas: a report of nine cases. Am J Otolaryngol 12:278–287
Netterville JL, Jackson CG, Miller FR, Wanamaker JR (1998) A review of 46 patients treated during a 20-year period. Arch Otolaryngol Head Neck Surg 124:1133–1140
Lozano FS, Gómez JL, Mondillo MC, González-Porras JR, González-Sarmiento R, Muñoz A (2008) Surgery of vagal paragangliomas: six patients and review of the literature. Surg Oncol 17(4):281–287
Shin SH, Piazza P, De Donato G, Sivalingam S, Lauda L, Vitullo F, Sanna M (2012) Management of vagal paragangliomas including application of internal carotid artery stenting. Audiol Neurootol 17(1):39–53
Boedeker CC, Hensen EF, Neumann HP, Maier W, van Nederveen FH, Suárez C et al (2013) Genetics of hereditary head and neck paragangliomas. Head Neck. doi:10.1002/hed.23436
Boedeker CC, Ridder GJ, Schipper J (2005) Paragangliomas of the head and neck: diagnosis and treatment. Fam Cancer 4:55–59
Browne JD, Fish U, Valavanis A (1993) Surgical therapy of glomus vagale tumors. Skull Base Surg 3:182–192
Biller HF, Lawson W, Som P, Rosenfeld R (1989) Glomus vagale tumors. Ann Otol Rhino Laryngol 98:21–26
Bradshaw JW, Jansen JC (2005) Management of vagal paraganglioma: is operative resection really the best option? Surgery 137:225–228
Borba LA, Al-Mefty O (1996) Intravagal paragangliomas: report of four cases. Neurosurgery 38:569–575
McNicol AM (2006) Histopathology and immunohistochemistry of adrenal medullary tumors and paragangliomas. Endocr Pathol 17(4):329–336
Miller RB, Boon MS, Atkins JP, Lowry LD (2000) Vagal paraganglioma: the Jefferson experience. Otolaryngol Head Neck Surg 122(4):482–487
Pellitteri PK, Rinaldo A, Myssiorek D et al (2004) Paragangliomas of the head and neck. Oral Oncol 40:563–575
Lack EE, Cubilla AL, Woodruf JM, Farr HW (1979) Paragangliomas of the head and neck region. A pathologic study of tumors from 71 patients. Hum Pathol 10:191–218
Suárez C, Rodrigo JP, Ferlito A, Cabanillas R, Shaha AR, Rinaldo A (2006) Tumors of familial origin in the head and neck. Oral Oncol 42:965–978
Pellitteri PK, Rinaldo A, Myssiorek D et al (2004) Paragangliomas of the head and neck. Oral Oncol 40:563–575
Myssiorek D (2001) Head and neck paragangliomas: an overview. Otolaryngol Clin N Am 34:829–836
Zanoletti E, Mazzoni A (2006) Vagal paraganglioma. Skull Base 16:161–167
Young AL, Baysal BE, Deb A, Young WF Jr (2002) Familial malignant catecholamine-secreting paraganglioma with prolonged survival associated with mutation in the succinate dehydrogenase B gene. J Clin Endocrinol Metab 87(9):4101–4105
Baysal BE (2001) Genetics of familial paragangliomas: past, present and future. Otolaryngol Clin N Am 34:863–879
Burninchon N, Rohmer V, Amar L et al (2009) The succinate dehydrogenase genetic testing in a large prospective series of patients with paragangliomas. J Clin Endocrinol Metab 94:2817–2827
Neumann HPH, Erilic Z, Boedeker CC et al (2009) Clinical predictors of of germline mutations in head and neck paraganglioma patients: cost genetic strategy in genetic diagnostic process as fall-out. Cancer Res 69:3650–3656
Weissman JL (2000) Case 21: glomus vagale tumor. Radiology 215:237–242
Clements DM, Hedges AR, Tudor GR (2002) Horner´s syndrome following excision of a vagal paraganglioma. Int J Clin Pract 56:626–627
Sykes JM, Ossoff RH (1986) Paragangliomas of the head and neck. Otolaryngol Clin N Am 19:755–767
Alkadhi H, Schuknecht B, Stoeckli SJ, Valvanis A (2002) Evaluation of topography and vascularization of cervical paragangliomas by magnetic resonance imaging and color duplex sonography. Neuroradiology 44:83–90
Kerr JT, Eusterman VD, Yoest SM, Andersen CA (2005) Pitfalls and imaging: differentiating intravagal and carotid body paragangliomas. Ear Nose Throat J 84:348–350
Thabet MH, Kotob H (2001) Cervical paragangliomas: diagnosis, management and complications. J Laryngol Otol 115:167–474
Stoeckli SJ, Schuknetcht B, Alkadhi H, Fish U (2003) Evaluation of paragangliomas presenting as a cervical mass on color-coded Doppler sonography. Laryngoscope 112:143–146
Kwekkeboom DJ, van Urk H, Pauw BK, Lamberts SW, Kooij PP, Hoogma RP et al (1993) Octeotride scintigraphy for the detection of paragangliomas. J Nucl Med 34:873–878
Forbes G, Earnest F, Jackson IT, Marsh WR, Jack CR, Cross SA (1986) Therapeutic embolization angiography for extra-axial lesions in the head. Mayo Clin Proc 61:427–441
Urquhart AC, Johnson JT, Myers EN, Schechter GL (1994) Glomus vagale: paraganglioma of the vagus nerve. Laryngoscope 104:440–445
Paris J, Facon F, Thomassin M, Zanaret M (2006) Cervical paragangliomas: neurovascular risk and therapeutic management. Eur Arch Otorhinolaryngol 263:860–865
Van Hulsteijn LT, Corssmit EP, Coremans IE, Smit JW, Jansen JC, Dekkers OM (2013) Regression and local control rates after radiotherapy for jugulotympanic paragangliomas: systematic review and meta-analysis. Radiother Oncol 106(2):161–168
Suárez C, Rodrigo JP, Mendenhall WM, Hamoir M, Silver CE, Grégoire V et al (2014) Carotid body paraganglioma: a systematic study on management with surgery and radiotherapy. Eur Arch Otorhinolaryngol 271(1):23–34
Hinerman RW, Mendenhall WM, Amdur RJ, Stringer SP, Antonelli P, Cassisi NJ (2001) Definitive radiotherapy in the management of chemodectomas arising in the temporal bone, carotid body and glomus vagale. Head Neck 23:363–371
Leonetti JP, Brackmann DE, Prass RL (1989) Improved preservation of facial nerve function in the infratemporal approach to the skull base. Otolaryngol Head Neck Surg 101:74–78
Sanna M, Khrais T, Menozi R, Piazza P (2006) Surgical removal of jugular paragangliomas after stenting of the intratemporal internal carotid artery: a preliminary report. Laryngoscope 116:742–746
Sanna M, Piazza P, Ditrapani G, Agarwal M (2004) Management of the internal caroid artery in tumors of lateral skull base: a preoperative permanent balloon occlusion without reconstruction. Otol Neurotol 25:998–1005
Isshiki N, Morita H, Okamura H, Hiramoto M (1974) Thyroplasty as a new phonosurgical technique. Acta Otolaryngol 78(5–6):451–457
Conflict of interest
Authors have not a financial relationship with the organization that sponsored the research.
Author information
Authors and Affiliations
Corresponding author
Rights and permissions
About this article
Cite this article
González-Orús Álvarez-Morujo, R., Arístegui Ruiz, M., Martin Oviedo, C. et al. Management of vagal paragangliomas: review of 17 patients. Eur Arch Otorhinolaryngol 272, 2403–2414 (2015). https://doi.org/10.1007/s00405-014-3141-0
Received:
Accepted:
Published:
Issue Date:
DOI: https://doi.org/10.1007/s00405-014-3141-0