Head and neck paragangliomas: Experience in 126 patients with 162 tumours

• Published in: Acta otorrinolaringologica espanola Vol. 66; no. 6; p. 332
• Main Authors: González-Orús Álvarez-Morujo, Ricardo José, Arístegui Ruiz, Miguel Ángel, da Costa Belisario, Julia, Martinez Guirado, Tomás, Scola Yurrita, Bartolomé
• Format: Journal Article
• Language: English; Spanish
• Published: Spain 01-11-2015
• Subjects: Adult; Cranial Nerve Injuries - epidemiology; Dura Mater - pathology; Female; Genetic Predisposition to Disease; Germ-Line Mutation; Head and Neck Neoplasms - epidemiology; Head and Neck Neoplasms - genetics; Head and Neck Neoplasms - radiotherapy; Head and Neck Neoplasms - surgery; Humans; Male; Middle Aged; Neoplasm Invasiveness; Neoplastic Syndromes, Hereditary - epidemiology; Paraganglioma - epidemiology; Paraganglioma - genetics; Paraganglioma - radiotherapy; Paraganglioma - surgery; Postoperative Complications - epidemiology; Retrospective Studies; Watchful Waiting; Multifocal paraganglioma; Surgical approaches; Tratamiento; Management; Paraganglioma de cabeza y cuello; Paraganglioma multicéntrico; Head and neck paraganglioma; Abordaje quirúrgico
• ISSN: 1988-3013
• DOI: 10.1016/j.otorri.2014.11.002

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.
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.
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).
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.