Intralabyrinthine sporadic endolymphatic sac tumour

Publication date: Available online 6 November 2017
Source:European Annals of Otorhinolaryngology, Head and Neck Diseases
Author(s): C. Lucas, J.-C. Leclère, E. Mornet, R. Marianowski
IntroductionEndolymphatic 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).Case summaryThe 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.DiscussionEndolymphatic 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.



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