Solid haemangioblastomas of the CNS: a review of 17 consecutive cases

Neurosurg Rev. 2009 Jan;32(1):37-47; discussion 47-8. doi: 10.1007/s10143-008-0166-0. Epub 2008 Sep 20.

Abstract

A retrospective study on solid central nervous system haemangioblastomas was performed to characterize clinical features, treatment strategies and outcome in these rare lesions. Between 1993 and 2006 23, solid haemangioblastomas were surgically removed in 17 patients. Eight lesions were located within pons Varolii and medulla oblongata, six within the cerebellar hemispheres and three in the cerebellopontine angle. Three haemangioblastomas were located supratentorially and three within the spinal cord. All patients except two underwent pre-operative magnetic resonance imaging (MRI). Post-operative digital subtraction angiography and/or MRI was performed in all surviving patients. Except for spinal cord lesions, rather unsystematic clinical symptoms were observed. Twenty-two tumours could be resected completely. Two patients with brainstem lesions died within 10 weeks after surgery from infectious complications. Persistent new neurological deficits occurred in two patients. Three patients underwent radiosurgery prior to or following the surgical procedure. Solid haemangioblastomas represent a surgical challenge due to their arteriovenous malformation-like vascularisation and their frequent location in eloquent areas. Surgery is the therapy of choice. Circumferential dissection with devascularization and en bloc removal yields good functional results. A location within the brainstem carries the most unfavourable prognosis.

MeSH terms

  • Adult
  • Aged
  • Central Nervous System Neoplasms / diagnostic imaging
  • Central Nervous System Neoplasms / pathology*
  • Central Nervous System Neoplasms / surgery*
  • Cerebellar Neoplasms / diagnostic imaging
  • Cerebellar Neoplasms / pathology
  • Cerebellar Neoplasms / surgery
  • Cerebral Angiography
  • Combined Modality Therapy
  • Embolization, Therapeutic
  • Female
  • Hemangioblastoma / diagnostic imaging
  • Hemangioblastoma / pathology*
  • Hemangioblastoma / surgery*
  • Humans
  • Karnofsky Performance Status
  • Male
  • Middle Aged
  • Neurosurgical Procedures
  • Retrospective Studies
  • Spinal Cord Neoplasms / diagnostic imaging
  • Spinal Cord Neoplasms / pathology
  • Spinal Cord Neoplasms / surgery
  • Treatment Outcome
  • Young Adult