title: Conservative management of endolymphatic sac tumors in von Hippel-Lindau disease: case report.
abstract: In endolymphatic sac tumors associated with von Hippel-Lindau disease, early detection and surgery have been warranted to avoid associated neurological morbidity. However, in lately discovered tumors, hearing preserving surgery is often impossible and timing of surgical resection is difficult to define. We report two cases of tumors revealed by a sudden and profound hearing loss and managed conservatively for more than 15 years without worsening of the neurological symptoms associated with the endolymphatic sac tumor. Tumor size remained stable for the first patient and a stuttering growth pattern was observed for the second patient. Initial observation may be considered a not unreasonable management paradigm in these cases.
authors.affiliation: Service de Neurochirurgie, Hôpital Beaujon, AP-HP, Clichy, France. matthieupeyre@hotmail.fr
authors.forename: Matthieu
authors.initials: M
authors.lastname: Peyre
authors.forename: Stephan
authors.initials: S
authors.lastname: Gaillard
authors.forename: Remy
authors.initials: R
authors.lastname: van Effenterre
authors.forename: Sophie
authors.initials: S
authors.lastname: Giraud
authors.forename: Stéphane
authors.initials: S
authors.lastname: Richard
mesh_terms: D000293:Adolescent; D018450:Disease Progression; D004712:Endolymphatic Sac / Q000473:pathology / Q000601:surgery*; D005260:Female; D034381:Hearing Loss / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D006801:Humans; D007759:Labyrinth Diseases / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D008297:Male; D010579:Petrous Bone / Q000473:pathology* / Q000601:surgery; D019292:Skull Base Neoplasms / Q000175:diagnosis / Q000209:etiology / Q000601:surgery*; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000601:surgery*