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Paragangliomas de cabeza y cuello: experiencia en 126 pacientes con 162 tumores

2015; Elsevier BV; Volume: 66; Issue: 6 Linguagem: Inglês

10.1016/j.otorri.2014.11.002

1988-3013

Ricardo José González- Orús Álvarez- Morujo, Miguel Ángel Arístegui Ruiz, Julia da Costa Belisario, Tomás Martínez Guirado, Bartolomé Scola Yurrita,

Neurofibromatosis and Schwannoma Cases

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.