Head and neck paragangliomas: Report of 175 patients (1989-2010)

Head Neck. 2012 May;34(5):632-7. doi: 10.1002/hed.21790. Epub 2011 Jun 20.


Background: Attention of the otorhinolaryngologist needs to be drawn to the versatile aspects of head and neck paragangliomas (PGLs).

Methods: This study is a retrospective, nonrandomized clinical study of all 175 individuals with PGLs treated in our department between 1989 and 2010. A genetic analysis was performed on 86 patients.

Results: The 175 patients presented 224 head and neck PGLs as well as 2 thyroid papillary carcinomas. Genetic analysis resulted in 1 patient positive for a von Hippel-Lindau (VHL) gene mutation and 34 for succinate dehydrogenase (SDH) gene mutations (22 SDHD, 7 SDHC, and 5 SDHB), 12 of the latter carrying a novel mutation. Thirty-three patients (18.9%) had multiple PGLs and 11 patients (6.3%) had a malignant paraganglioma. SDH-mutation carriers had multiple tumors in 64.7% and malignant paragangliomas in 20.6%.

Conclusions: Multifocal occurrence, potential malignancy, genetic aspects, possible coincidence of thyroid carcinoma, and hormone production have to be considered in patients with head and neck PGLs.

MeSH terms

  • Carcinoma, Papillary / pathology
  • Female
  • Head and Neck Neoplasms / genetics
  • Head and Neck Neoplasms / pathology*
  • Heterozygote
  • Humans
  • Male
  • Mutation
  • Neoplasms, Multiple Primary / pathology
  • Paraganglioma / genetics
  • Paraganglioma / pathology*
  • Retrospective Studies
  • Succinate Dehydrogenase / genetics
  • Von Hippel-Lindau Tumor Suppressor Protein / genetics


  • Succinate Dehydrogenase
  • Von Hippel-Lindau Tumor Suppressor Protein