title: Functional outcome after resection of spinal cord hemangioblastomas associated with von Hippel-Lindau disease.
abstract: OBJECTIVE
  Spinal cord hemangioblastomas are a common protean manifestation of von Hippel-Lindau (VHL) disease and can be associated with significant morbidity. To better define expected outcome and optimal management of these tumors in the context of this neoplasia syndrome, the authors analyzed the findings from patients with VHL disease who underwent resection of spinal cord hemangioblastomas.
  
  
  METHODS
  Consecutive patients with VHL disease who underwent surgery for spinal cord hemangioblastomas with > 6 months follow-up were included in the study. Serial clinical examinations, functional scores, imaging findings, and operative records were analyzed.
  
  
  RESULTS
  One hundred eight patients (57 male, 51 female) underwent 156 operations for resection of 218 spinal cord hemangioblastomas. One hundred forty-six operations (94%) were performed for symptom-producing tumors. The most common presenting symptoms included hypesthesia (64% of resections), hyperreflexia (57%), dysesthesia (43%), and weakness (36%). Mean follow-up was 7.0 +/- 5.0 years (range 0.5-20.9 years). Complete resection was achieved for 217 tumors (99.5%). At 6-months follow-up, patients were stable or improved after 149 operations (96%) and worse after 7 operations (4%). Ventral tumors (OR 15.66, 95% CI 2.54-96.45; p = 0.003) or completely intramedullary tumors (OR 10.74, 95% CI 2.07-55.66; p = 0.005) were associated with an increased risk of postoperative worsening. The proportion of patients remaining functionally stable at 2, 5, 10, and 15 years' follow-up was 93, 86, 78, and 78%. Long-term functional decline was caused by extensive VHL-associated CNS disease (6 patients), VHL-associated visceral disease (1 patient), or non-VHL disease (2 patients).
  
  
  CONCLUSIONS
  Resection of symptomatic spinal cord hemangioblastomas is a safe and effective means of preserving neurological function in patients with VHL disease. Tumor location (ventral or completely intramedullary) can be used to assess functional risk associated with surgery. Long-term decline in neurological function is usually caused by VHL-associated disease progression.
authors.affiliation: Surgical Neurology Branch, National Institute of Neurological Disorders and Stroke, National Institutes of Health, Bethesda, Maryland 20892-1414, USA.
authors.forename: Gautam U
authors.initials: GU
authors.lastname: Mehta
authors.forename: Ashok R
authors.initials: AR
authors.lastname: Asthagiri
authors.forename: Kamran D
authors.initials: KD
authors.lastname: Bakhtian
authors.forename: Sungyoung
authors.initials: S
authors.lastname: Auh
authors.forename: Edward H
authors.initials: EH
authors.lastname: Oldfield
authors.forename: Russell R
authors.initials: RR
authors.lastname: Lonser
mesh_terms: D000293:Adolescent; D000328:Adult; D005260:Female; D005500:Follow-Up Studies; D018325:Hemangioblastoma / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D006801:Humans; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009460:Neurologic Examination; D019635:Neurosurgical Procedures / Q000379:methods; D011183:Postoperative Complications; D013116:Spinal Cord / Q000473:pathology / Q000601:surgery*; D013120:Spinal Cord Neoplasms / Q000209:etiology / Q000473:pathology / Q000601:surgery*; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult; D006623:von Hippel-Lindau Disease / Q000150:complications / Q000473:pathology / Q000601:surgery*