title: Hemangioblastomatosis of the central nervous system without von Hippel-Lindau disease: a case report.
abstract: We report a very rare case of hemangioblastomatosis in a patient without von Hippel-Lindau disease (VHL). A 50-year-old woman had a history of surgical procedures for total removal of a cerebellar hemangioblastoma (HB). Twenty-one years after the last operation, she developed communicating hydrocephalus; computed tomographic (CT) scans of the brain showed no recurrence of HB in the posterior fossa. Subsequently, she underwent placement of a ventriculo-peritoneal shunt. One year later, she was readmitted because of progressive numbness and pain in the left lower limb. Magnetic resonance imaging (MRI) showed multiple Gd-enhancing tumors around the brain stem, in the cerebellum, and in the cervical and thoracolumbar regions of the spine. She underwent surgical removal of the tumors in the cerebellum and spinal cord. Although the extirpated tissues were histopathologically verified HB with less than 1% MIB-1 labeling index, surgery was followed by external beam radiation therapy with doses of 40 Gy to the whole brain, 10 Gy to the posterior fossa and 30 Gy to the whole spine. However, she subsequently developed quadriparesis and became bedridden.
authors.affiliation: Department of Neurosurgery, Gifu University School of Medicine, 1-1 Yanagido, Gifu, 501-1194, Japan. masayasu@cc.gifu-u.ac.jp
authors.forename: Masayasu
authors.initials: M
authors.lastname: Kato
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Ohe
authors.forename: Ayumi
authors.initials: A
authors.lastname: Okumura
authors.forename: Jun
authors.initials: J
authors.lastname: Shinoda
authors.forename: Asuka
authors.initials: A
authors.lastname: Nomura
authors.forename: Taro
authors.initials: T
authors.lastname: Shuin
authors.forename: Noboru
authors.initials: N
authors.lastname: Sakai
mesh_terms: D016543:Central Nervous System Neoplasms / Q000150:complications / Q000473:pathology* / Q000628:therapy; D002528:Cerebellar Neoplasms / Q000473:pathology / Q000601:surgery; D005260:Female; D005682:Gadolinium; D018325:Hemangioblastoma / Q000150:complications / Q000473:pathology* / Q000628:therapy; D006801:Humans; D006849:Hydrocephalus / Q000209:etiology / Q000601:surgery; D008279:Magnetic Resonance Imaging; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D011782:Quadriplegia / Q000209:etiology; D013120:Spinal Cord Neoplasms / Q000601:surgery; D014057:Tomography, X-Ray Computed; D017287:Ventriculoperitoneal Shunt; D006623:von Hippel-Lindau Disease / Q000473:pathology*
chemical_list: D005682:Gadolinium