title: Spinal cord hemangioblastoma : diagnosis and clinical outcome after surgical treatment.
abstract: OBJECTIVE
  Spinal cord hemangioblastoma is an uncommon vascular neoplasm with a benign nature and is associated with von Hippel-Lindau (VHL) disease in 20-30% of patients. Total removal of these tumors without significant neurological deficit remains a great challenge. The purpose of this study was to investigate the efficacy of VHL mutation analysis and to evaluate surgical outcome of patients with spinal cord hemangioblastomas.
  
  
  METHODS
  This study included nine patients treated for spinal cord hemangioblastomas at our institute between December 1994 and March 2006. There were four male and five female patients. Mean age was 37.8 years. The mean follow-up period was 22.4 months. Magnetic resonance imaging (MRI) of the complete neuraxis was done in all cases and VHL mutation analysis was performed in three cases for a definite diagnosis.
  
  
  RESULTS
  Six patients had intramedullary tumor, and the remaining patients had intradural extramedullary lesions. Five patients were associated with VHL disease. The von Hippel-Lindau mutation analysis was done in three patients and two of them showed VHL gene abnormality. Tumors were located in the cervical cord in five cases and in the thoracic cord in four cases. All patients underwent surgical intervention, and total removal was achieved in six cases. All patients showed improvement or, at least, clinically stationary state. Surgical complications did not develop in any cases.
  
  
  CONCLUSIONS
  Spinal hemangioblastoma in this series has been safely and effectively removed via a posterior approach. Postoperatively, clinical outcome was excellent in the majority of cases. The VHL mutation analysis was useful in patients with family history and in those with multiple hemangioblastomas.
authors.affiliation: Department of Neurosurgery , Samsung Medical Center, Sungkyunkwan University, School of Medicine, Seoul, Korea.
authors.forename: Joon Ho
authors.initials: JH
authors.lastname: Na
authors.forename: Hyeong Soo
authors.initials: HS
authors.lastname: Kim
authors.forename: Whan
authors.initials: W
authors.lastname: Eoh
authors.forename: Jong Hyun
authors.initials: JH
authors.lastname: Kim
authors.forename: Jong Soo
authors.initials: JS
authors.lastname: Kim
authors.forename: Eun-Sang
authors.initials: ES
authors.lastname: Kim