Intralabyrinthine sporadic endolymphatic sac tumour

Endolymphatic sac tumours are benign, slowly growing tumours that invade the temporal bone, and present clinically in the form of unilateral hearing loss. They can be sporadic or occur in the context of Von Hippel-Lindau disease (VHL). The authors report a case of endolymphatic sac tumour arising in...

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Veröffentlicht in:European annals of otorhinolaryngology, head and neck diseases head and neck diseases, 2018-04, Vol.135 (2), p.123-125
Hauptverfasser: Lucas, C., Leclère, J.-C., Mornet, E., Marianowski, R.
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container_title European annals of otorhinolaryngology, head and neck diseases
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creator Lucas, C.
Leclère, J.-C.
Mornet, E.
Marianowski, R.
description Endolymphatic sac tumours are benign, slowly growing tumours that invade the temporal bone, and present clinically in the form of unilateral hearing loss. They can be sporadic or occur in the context of Von Hippel-Lindau disease (VHL). The authors report a case of endolymphatic sac tumour arising in the utricle presenting histological and immunohistochemical features corresponding to endolymphatic sac tumour in a patient without VHL. Endolymphatic sac tumours invade the posterior part of the petrous temporal bone. According to two studies concerning patients with Von Hippel-Lindau disease, endolymphatic sac tumours arise from the endolymphatic duct. This case of intralabyrinthine sporadic endolymphatic sac tumour supports this hypothesis for sporadic forms, indicating the need for labyrinthectomy associated with tumour resection to avoid recurrence.
doi_str_mv 10.1016/j.anorl.2017.10.002
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source Elsevier ScienceDirect Journals; Elektronische Zeitschriftenbibliothek - Frei zugängliche E-Journals
subjects Endolymphatic sac tumour
Tinnitus
Unilateral hearing loss
Von Hippel-Lindau disease
title Intralabyrinthine sporadic endolymphatic sac tumour
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