title: Management of von Hippel-Lindau disease-associated CNS lesions.
abstract: Patients with von Hippel-Lindau disease (VHL) often harbor significant disease burden within the CNS, specifically craniospinal-axis hemangioblastomas and endolymphatic sac tumors (ELSTs). The majority (60-80%) of patients with VHL harbor hemangioblastomas, and 10-15% will develop ELSTs. Advances in the understanding of the natural history and outcomes associated with the surgical management of VHL-associated tumors have led to improved management of patients with VHL. Optimizing indications for surgical intervention and refining of surgical techniques for these lesions can reduce patient morbidity associated with the management of this syndrome. In this article, we review the various aspects of perioperative management of patients with VHL, surgical indications and general operative principles for the management of hemangioblastomas and ELSTs, and outcomes associated with the surgical treatment of these tumors.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, NIH, Bethesda, MD, USA.
authors.forename: Joshua J
authors.initials: JJ
authors.lastname: Wind
authors.forename: Russell R
authors.initials: RR
authors.lastname: Lonser
mesh_terms: D016543:Central Nervous System Neoplasms / Q000150:complications / Q000175:diagnosis / Q000473:pathology / Q000628:therapy*; D004712:Endolymphatic Sac / Q000473:pathology / Q000503:physiopathology / Q000601:surgery; D018325:Hemangioblastoma / Q000150:complications / Q000235:genetics / Q000473:pathology / Q000601:surgery; D006801:Humans; D016896:Treatment Outcome; D051794:Von Hippel-Lindau Tumor Suppressor Protein / Q000502:physiology*; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000175:diagnosis / Q000473:pathology* / Q000628:therapy*
chemical_list: D051794:Von Hippel-Lindau Tumor Suppressor Protein; C497446:VHL protein, human