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Gazzetta Medica Italiana - Archivio per le Scienze Mediche 2019 May;178(5):340-3

DOI: 10.23736/S0393-3660.18.03823-8

Copyright © 2018 EDIZIONI MINERVA MEDICA

language: English

Ten years after excision of endolymphatic sac adenocarcinoma: follow-up of a clinical case

Osama METWALY

Department of Otorhinolaryngology, Kasr El Ainy School of Medicine, Cairo University, Cairo, Egypt



Endolymphatic sac tumor is a rare neoplasm with benign histopathological appearance and locally destructive behavior which usually invades the posterior petrous bone, the mastoid, semicircular canal and the cerebellopontine angle structures. This tumor has been referred to as adenocarcinoma of endolymphatic sac, papillary adenomatous tumor, aggressive papillary adenoma and papillary tumor of temporal bone. These tumors can occur sporadically or in Von Hippel-Lindau (VHL) disease. Wide excision is the treatment of choice. Based on the anatomic site, it is difficult to completely remove, and so while there is a good prognosis, recurrences or persistence may be seen. This report presents a fifty-six years old female patient with endolymphatic sac tumor which was initially diagnosed as paraganglioma, complete excision of the neoplasm was done on 2007 at Kasr Alainy Hospital, Cairo University, Egypt. Now, follow-up work up for the patient was done without any detectable recurrence.


KEY WORDS: Endolymphatic sac - Adenocarcinoma - Paraganglioma - von Hippel-Lindau disease

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