title: Hemangioblastoma of the optic nerve: case report.
abstract: An optic nerve hemangioblastoma arising in the optic nerve of a patient with von Hippel-Lindau syndrome is reported. This represents the 10th published example of a hemangioblastoma arising at this site, the second description of the magnetic resonance imaging features of a hemangioblastoma at this location, and the second patient from whom an optic nerve hemangioblastoma has been removed with the goal of preserving the optic nerve as well as vision. A majority of these optic nerve hemangioblastomas have arisen in patients with von Hippel-Lindau syndrome. All patients experienced progressive loss of vision either to blindness or surgical intervention, although a vast majority of the lesions were sharply demarcated from the adjacent nerve and, thus, potentially resectable. Optic nerve hemangioblastomas are a rare cause of blindness but potentially preventable when treated with a conservative surgical approach aided by neuroimaging guidance. The recognition of discordance between the degree of vision loss and the extent or progression of a retinal hemangioblastoma may be an important clue to diagnosis in the patient with von Hippel-Lindau syndrome.
authors.affiliation: Department of Pathology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: D J
authors.initials: DJ
authors.lastname: Kerr
authors.forename: B W
authors.initials: BW
authors.lastname: Scheithauer
authors.forename: G M
authors.initials: GM
authors.lastname: Miller
authors.forename: M J
authors.initials: MJ
authors.lastname: Ebersold
authors.forename: T J
authors.initials: TJ
authors.lastname: McPhee
mesh_terms: D000328:Adult; D003390:Cranial Nerve Neoplasms / Q000473:pathology* / Q000601:surgery; D005260:Female; D018325:Hemangioblastoma / Q000473:pathology* / Q000601:surgery; D006801:Humans; D009900:Optic Nerve / Q000473:pathology* / Q000601:surgery; D009901:Optic Nerve Diseases / Q000473:pathology* / Q000601:surgery; D006623:von Hippel-Lindau Disease / Q000150:complications