A Rare Case of Endolymphatic Sac Tumour: Clinicopathologic Study and Surgical Management. (4th June 2014)

• Record Type: Journal Article
• Authors: Ferri, Emanuele; Amadori, Maurizio; Armato, Enrico; Pavon, Ida
• Other Names: De Diego Juan I. Academic Editor.
• Is Part Of: Case reports in otolaryngology. Volume 2014(2014)
• Publication Date: 2014-06-04
• Subjects: Otolaryngology -- Periodicals; Otolaryngology -- Case studies -- Periodicals; Otolaryngology; Otorhinolaryngologic Diseases; Otolaryngology; Periodicals; Case studies; Periodicals; Case Reports; Fulltext; Internet Resources; Periodicals; 617.51
• DOI: 10.1155/2014/376761
• Languages: English
• ISSNs: 2090-6765

Abstract

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.