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Case Reports in Otolaryngology
Volume 2014 (2014), Article ID 376761, 6 pages
http://dx.doi.org/10.1155/2014/376761
Case Report

A Rare Case of Endolymphatic Sac Tumour: Clinicopathologic Study and Surgical Management

1Emergency Department, Otorhinolaryngology Unit, Otosurgery and Audiovestibology Section, General Hospital of Monselice, Via G. Marconi 19, Padua, 35043 Monselice, Italy
2Surgical Department, Otorhinolaryngology Unit, Otosurgery, Audiology and Vestibology Section, General Hospitals of Dolo and Mirano, Via Mariutto 76, Venice, 30035 Mirano, Italy
3Surgical Pathology Unit, General Hospitals of Dolo and Mirano, Via Mariutto 76, Venice, 30035 Mirano, Italy

Received 29 December 2013; Revised 21 May 2014; Accepted 21 May 2014; Published 4 June 2014

Academic Editor: Juan I. De Diego

Copyright © 2014 Emanuele Ferri et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

Objective. Endolymphatic sac tumor (ELST) is a rare neoplasm arising from the intrapetrous portion of the endolymphatic sac, either isolated or in association with the von Hippel-Lindau disease. We report a sporadic case of ELST with an overview of the literature and a discussion of clinic-radiological, histopathologic, and surgical findings. Case Report. A young woman presented with a progressive hearing loss in the left ear. Otoscopy showed a reddish, bleeding hypotympanic mass. CT demonstrated an expansile lytic mastoid lesion extending to the middle ear, with bone erosion. MRI confirmed a lesion of increased signal on T1-weighted sequences. The patient underwent a canal wall-down tympanoplasty with complete removal of the tumor. Histopathology was consistent with a papillary ELST. Immunohistochemistry was positive for cytokeratin and chromogranin A. Conclusion. This paper highlights the rarity of ELST, the need for an accurate neuroradiological and immunohistochemical study at the early stages, and the timeliness of surgical treatment.