Adenocarcinoma del saco endolinfático en la enfermedad de von Hippel-Lindau. A propósito de un caso

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.