title: Papillary adenoma of endolymphatic sac origin: a temporal bone tumor in von Hippel-Lindau disease. Case report.
abstract: This report describes a patient with von Hippel-Lindau disease who presented with an 8-year history of a slow-growing, locally invasive vascularized lesion of the temporal bone involving the cerebellopontine angle. The mass, studied by computerized tomography scanning and magnetic resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cystic tumor with characteristics that have been described in tumors with an endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.
authors.affiliation: Department of Neurosurgery, Hôpital de la Salpêtrière, Paris, France.
authors.forename: J C
authors.initials: JC
authors.lastname: Ouallet
authors.forename: K
authors.initials: K
authors.lastname: Marsot-Dupuch
authors.forename: R
authors.initials: R
authors.lastname: Van Effenterre
authors.forename: M
authors.initials: M
authors.lastname: Kujas
authors.forename: J M
authors.initials: JM
authors.lastname: Tubiana
mesh_terms: D000236:Adenoma / Q000175:diagnosis*; D000328:Adult; D003937:Diagnosis, Differential; D004712:Endolymphatic Sac / Q000473:pathology*; D005260:Female; D006801:Humans; D008279:Magnetic Resonance Imaging; D012888:Skull Neoplasms / Q000150:complications*; D013701:Temporal Bone / Q000473:pathology*; D014057:Tomography, X-Ray Computed; D006623:von Hippel-Lindau Disease / Q000150:complications*