Hemangioblastoma of the optic nerve

A 43-year-old woman presented with progressive loss of vision in the right eye. Magnetic resonance imaging (MRI) showed a prominently enhancing lesion of the optic nerve, thought preoperatively to represent an optic nerve meningioma or optic neuritis. Histological examination of the excised tumor showed this lesion to be hemangioblastoma. Her family history was unremarkable. However, subsequent review of the preoperative MRIs and postoperative imaging studies showed two small cerebellar lesions, probably hemangioblastomas, and renal, pancreatic, and adnexal cysts, establishing the diagnosis of von Hippel-Lindau disease.