title: [Adenocarcinoma of the endolymphatic sac in von Hippel-Lindau disease. A case report].
abstract: Tumors originating in the endolymphatic system are extremely rare, and very few articles have been published about this type of tumors. Up to 15% of cases are associated to von Hippel-Lindau disease. This article describes the case of a patient previously diagnosed with von Hippel-Landau disease that presented a sudden loss of hearing in the left ear. Specific imaging tests (computed tomography [CT] and magnetic resonance imaging [MRI]) showed the presence of a neoplasm originating in the left endolymphatic sac. Histological analysis of the surgical specimen determined it corresponded to an adenocarcinoma of the endolymphatic sac.
authors.affiliation: Servicio de Radiología, Clínica Universitaria, Facultad de Medicina, Universidad de Navarra, Pamplona, Spain. paulamartinezmiravete@yahoo.es
authors.forename: P
authors.initials: P
authors.lastname: Martínez-Miravete
authors.forename: P
authors.initials: P
authors.lastname: Domínguez
authors.forename: J L
authors.initials: JL
authors.lastname: Zubieta
authors.forename: M
authors.initials: M
authors.lastname: Manrique
mesh_terms: D000230:Adenocarcinoma / Q000150:complications*; D000328:Adult; D004428:Ear Neoplasms / Q000150:complications*; D004712:Endolymphatic Sac; D006801:Humans; D008297:Male; D006623:von Hippel-Lindau Disease / Q000150:complications*