Characteristics and management of sinonasal paragangliomas: a systematic review

Brandon K. Nguyen, Nirali M. Patel, Khashayar Arianpour Bs, Peter F. Svider, Adam J. Folbe, Wayne D. Hsueh, Jean Anderson Eloy

Research output: Contribution to journalReview articlepeer-review


Background: Paragangliomas of the nasal cavity and paranasal sinuses, although exceedingly rare, can exhibit frequent and aggressive recurrences. Our objective was to evaluate tumor characteristics, clinical course, manage-ment, and associated complications of sinonasal paragan-gliomas Methods: A systematic review of the literature was performed utilizing the Preferred Reporting Items for Systematic Reviews and Meta-Analysis (PRISMA) guide-lines, searching for sinonasal paraganglioma. Studies meeting inclusion criteria were assessed for level of evidence. Patient demographics, tumor characteristics, primary intervention, and other clinical characteristics were evaluated. Results: Forty-five relevant studies encompassing 54 patients with sinonasal paraganglioma were identified. The most common tumor locations were the nasal cavity (66.7%), ethmoid sinuses (22.2%), maxillary sinuses (7.4%), and sphenoid sinuses (3.7%). Common presenting symptoms included recurrent epistaxis (68.5%), nasal obstruction (53.7%), and headache (13.0%). Tumors were malignant in 28.6% of patients. Only 4 cases (7.4%) involved functional tumors. Initial management was always surgical, via either an open (63.0%) or endoscopic (33.3%) approach. Radiotherapy was used as adjunctive treatment in 10 cases (18.5%). Recurrence rate was 21.7% and occurred between 12 to 156 months after initial resection. The overall survival was 87.0% with a metastatic rate of 8.7%. Conclusion: Sinonasal paragangliomas are vascular neo-plasms manifesting clinically with recurrent epistaxis and nasal obstruction. Management goals are total resection with clear margins and long-term follow-up due to tendency for local recurrence. Radiotherapy has been utilized as ad-juvant therapy with variable results. Further randomized controlled studies may be invaluable in elucidating these findings.

Original languageEnglish (US)
Pages (from-to)413-426
Number of pages14
JournalInternational Forum of Allergy and Rhinology
Issue number4
StatePublished - Apr 2019

ASJC Scopus subject areas

  • Immunology and Allergy
  • Otorhinolaryngology


  • Nasal
  • Nasal cavity
  • Neuroen-docrine tumor
  • Paragangliomas
  • Sinonasal


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