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ID 45273
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Author
Himei, Kengo
Ishii, Keita
Nose, Soichiro
Mimura, Tetsushige
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.
Keywords
retinal (papillary, optic disc) hemangioma
von Hippel-Lindau disease
pheochromocytoma
pancreatic neuroendocrine tumor
external beam radiation (radiotherapy)
Amo Type
Case Report
Published Date
2011-04
Publication Title
Acta Medica Okayama
Volume
volume65
Issue
issue2
Publisher
Okayama University Medical School
Start Page
135
End Page
141
ISSN
0386-300X
NCID
AA00508441
Content Type
Journal Article
language
英語
Copyright Holders
CopyrightⒸ 2011 by Okayama University Medical School
File Version
publisher
Refereed
True
PubMed ID
Web of Science KeyUT