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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Bibliographic Details
Published in:European annals of otorhinolaryngology, head and neck diseases head and neck diseases, 2018-04, Vol.135 (2), p.123-125
Main Authors: Lucas, C., Leclère, J.-C., Mornet, E., Marianowski, R.
Format: Article
Language:English
Subjects:
Ear Neoplasms - diagnosis
Ear Neoplasms - surgery
Endolymphatic Sac - pathology
Endolymphatic sac tumour
Humans
Magnetic Resonance Imaging
Male
Middle Aged
Neoplasm Invasiveness
Otologic Surgical Procedures - methods
Petrous Bone - pathology
Petrous Bone - surgery
Tinnitus
Tomography, X-Ray Computed - methods
Treatment Outcome
Unilateral hearing loss
Von Hippel-Lindau disease
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Summary: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.
ISSN:1879-7296
1879-730X
DOI:10.1016/j.anorl.2017.10.002