title: Head and neck paragangliomas: Experience in 126 patients with 162 tumours.
abstract: BACKGROUND
  Head and neck paragangliomas are rare tumours of a neuroendocrine nature. The aim of this study was to show our experience in the management of these kind of tumours.
  
  
  METHODS
  This was a retrospective study of head and neck paragangliomas diagnosed between 1978 and 2014. A total of 126 patients with 162 tumours were analysed. The paragangliomas included 88 jugulotympanic tumours, 53 carotid tumours and 21 vagal paragangliomas.
  
  
  RESULTS
  Mean age at diagnosis was 53.1 years; 87 patients were female (69.1%) and 39, male (30.9%). Multifocality was present in 24 patients (19.1%). Germline mutations were found in 20 patients analysed; SDHD and SDHB were the most frequent. Surgery was performed on 72 isolated paragangliomas: these were 9 carotid, 21 tympanic, 37 jugular and 5 vagal paragangliomas. There were 25 isolated tumours that were observed periodically: 7 carotid, 3 tympanic, 9 jugular and 6 vagal paragangliomas; 5 jugular tumours were irradiated. Multifocal paragangliomas were individually treated, with a total of 26 surgical procedures and 36 tumours resected, 9 irradiated and 12 tumours periodically observed. Postoperative cranial nerve deficits in isolated carotid paragangliomas were lower (15%) compared with jugular tumours (45.5%, P=.04). Nerve deficit was found more frequently in tumours with intradural extension (100%) than in extradural tumours (37.5%, P=.007).
  
  
  CONCLUSIONS
  Management of head and neck paragangliomas include surgery, radiotherapy and wait and scan policies. A combination of all of them is usually needed in patients with multifocal paragangliomas.
authors: Servicio de ORL, Hospital General Universitario Gregorio Marañón Madrid, España. Electronic address: ricardomorujo@hotmail.com.
authors: Ricardo José
authors: RJ
authors: González-Orús Álvarez-Morujo
authors: Servicio de ORL, Hospital General Universitario Gregorio Marañón Madrid, España.
authors: Miguel Ángel
authors: MÁ
authors: Arístegui Ruiz
authors: Servicio de ORL, Hospital General Universitario Gregorio Marañón Madrid, España.
authors: Julia
authors: J
authors: da Costa Belisario
authors: Servicio de ORL, Hospital General Universitario Gregorio Marañón Madrid, España.
authors: Tomás
authors: T
authors: Martinez Guirado
authors: Servicio de ORL, Hospital General Universitario Gregorio Marañón Madrid, España.
authors: Bartolomé
authors: B
authors: Scola Yurrita
mesh_terms: D000328:Adult; D020209:Cranial Nerve Injuries / Q000453:epidemiology; D004388:Dura Mater / Q000473:pathology; D005260:Female; D020022:Genetic Predisposition to Disease; D018095:Germ-Line Mutation; D006258:Head and Neck Neoplasms / Q000453:epidemiology* / Q000235:genetics / Q000532:radiotherapy / Q000601:surgery; D006801:Humans; D008297:Male; D008875:Middle Aged; D009361:Neoplasm Invasiveness; D009386:Neoplastic Syndromes, Hereditary / Q000453:epidemiology; D010235:Paraganglioma / Q000453:epidemiology* / Q000235:genetics / Q000532:radiotherapy / Q000601:surgery; D011183:Postoperative Complications / Q000453:epidemiology; D012189:Retrospective Studies; D057832:Watchful Waiting
keywords: Abordaje quirúrgico; Head and neck paraganglioma; Management; Multifocal paraganglioma; Paraganglioma de cabeza y cuello; Paraganglioma multicéntrico; Surgical approaches; Tratamiento