Intra-extradural dumbbell-shaped hemangioblastoma manifesting as subarachnoid hemorrhage in the cauda equina

Neurol Med Chir (Tokyo). 2012;52(9):659-65. doi: 10.2176/nmc.52.659.


A 56-year-old man presented with a rare intra-extradural dumbbell-shaped hemangioblastoma in the lumbar spine associated with von Hippel-Lindau (VHL) disease manifesting as subarachnoid hemorrhage. The tumor, which was exiting at the right L2-3 intervertebral foramen, was removed successfully by total facetectomy and posterior spinal fusion at the L2-3 segment. Nine years later, a recurrent tumor due to VHL was also totally removed with minimal neurological complications. Accurate diagnosis of this vascular-rich lesion is essential for developing an adequate surgical strategy. The dumbbell-shaped tumor requires total facetectomy and spinal reconstruction, and care should be taken to preserve the entire nerve root origin by only identifying the affected nerve fascicles at the origin, if possible. Postoperative adhesion must be minimized for second surgery in patients with genetic disease such as VHL, who are likely to suffer recurrence.

Publication types

  • Case Reports
  • Review

MeSH terms

  • Cauda Equina / pathology
  • Cauda Equina / surgery*
  • Cerebellar Neoplasms / genetics
  • Cerebellar Neoplasms / surgery
  • Combined Modality Therapy
  • Embolization, Therapeutic
  • Hemangioblastoma / blood supply
  • Hemangioblastoma / complications*
  • Hemangioblastoma / diagnosis
  • Hemangioblastoma / genetics
  • Hemangioblastoma / surgery
  • Hemangioblastoma / therapy
  • Humans
  • Lumbar Vertebrae / surgery
  • Male
  • Middle Aged
  • Neoplasm Recurrence, Local / surgery
  • Neoplasms, Second Primary / diagnosis
  • Neoplasms, Second Primary / genetics
  • Neoplasms, Second Primary / surgery
  • Polyradiculopathy / etiology
  • Spinal Cord Neoplasms / blood supply
  • Spinal Cord Neoplasms / complications*
  • Spinal Cord Neoplasms / diagnosis
  • Spinal Cord Neoplasms / genetics
  • Spinal Cord Neoplasms / surgery
  • Spinal Cord Neoplasms / therapy
  • Spinal Fusion
  • Subarachnoid Hemorrhage / etiology*
  • von Hippel-Lindau Disease