title: Long-term effect of external beam radiotherapy of optic disc hemangioma in a patient with von Hippel-Lindau disease.
abstract: An 18-year-old woman with a 2-year history of hypertension and headache was diagnosed with noradrenalin-secreting bilateral adrenal pheochromocytomas with paragangliomas in the background of von Hippel-Lindau disease with family histories and a missense mutation, 712C to T (Arg167Trp) in the VHL gene. She had optic disc hemangioma in the left eye which gradually enlarged and caused serous retinal detachment on the macula in one year. Low-dose external beam radiation (20 Gy) was administered to the left eye using a lens-sparing single lateral technique. She underwent craniotomy for cerebellar hemangioblastoma at the age of 22 years and total pancreatectomy for multiple neuroendocrine tumors at the age of 24 years. In the 6-year follow-up period after the radiotherapy, the optic disc hemangioma gradually reduced in size and its activity remained low, allowing good central vision to be maintained. External beam radiation is recommended as a treatment option for the initial therapy for optic disc hemangioma.
authors.affiliation: Department of Ophthalmology, Okayama University Medical School and Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama 700-8558, Japan. matsuot@cc.okayama-u.ac.jp
authors.forename: Toshihiko
authors.initials: T
authors.lastname: Matsuo
authors.forename: Kengo
authors.initials: K
authors.lastname: Himei
authors.forename: Kouichi
authors.initials: K
authors.lastname: Ichimura
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Yanai
authors.forename: Soichiro
authors.initials: S
authors.lastname: Nose
authors.forename: Tetsushige
authors.initials: T
authors.lastname: Mimura
authors.forename: Yasuyuki
authors.initials: Y
authors.lastname: Miyoshi
authors.forename: Tomoyasu
authors.initials: T
authors.lastname: Tsushima
mesh_terms: D000328:Adult; D005260:Female; D006391:Hemangioma / Q000150:complications / Q000532:radiotherapy*; D006801:Humans; D009898:Optic Disk; D019574:Optic Nerve Neoplasms / Q000150:complications / Q000532:radiotherapy*; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors; D006623:von Hippel-Lindau Disease / Q000150:complications*
chemical_list: D042461:Vascular Endothelial Growth Factor A