title: Head and neck paragangliomas: Report of 175 patients (1989-2010).
abstract: 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.
authors.affiliation: Department of Otorhinolaryngology-Head and Neck Surgery, University Medical Center of the Johannes Gutenberg University Mainz, Mainz, Germany. konstantinos.papaspyrou@unimedizin-mainz.de
authors.forename: Konstantinos
authors.initials: K
authors.lastname: Papaspyrou
authors.forename: Torsten
authors.initials: T
authors.lastname: Mewes
authors.forename: Heidi
authors.initials: H
authors.lastname: Rossmann
authors.forename: Christian
authors.initials: C
authors.lastname: Fottner
authors.forename: Brigitte
authors.initials: B
authors.lastname: Schneider-Raetzke
authors.forename: Oliver
authors.initials: O
authors.lastname: Bartsch
authors.forename: Mathias
authors.initials: M
authors.lastname: Schreckenberger
authors.forename: Karl J
authors.initials: KJ
authors.lastname: Lackner
authors.forename: Ronald G
authors.initials: RG
authors.lastname: Amedee
authors.forename: Wolf J
authors.initials: WJ
authors.lastname: Mann
mesh_terms: D002291:Carcinoma, Papillary / Q000473:pathology; D005260:Female; D006258:Head and Neck Neoplasms / Q000235:genetics / Q000473:pathology*; D006579:Heterozygote; D006801:Humans; D008297:Male; D009154:Mutation; D009378:Neoplasms, Multiple Primary / Q000473:pathology; D010235:Paraganglioma / Q000235:genetics / Q000473:pathology*; D012189:Retrospective Studies; D013385:Succinate Dehydrogenase / Q000235:genetics; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000235:genetics
chemical_list: D013385:Succinate Dehydrogenase; D051794:Von Hippel-Lindau Tumor Suppressor Protein