Skip to content

Spinal Haeangioblastoma

Summary

  • Rare, benign vascular tumours of the central nervous system
  • Most commonly occur in the cerebellum, but can affect the spinal cord
  • Often associated with von Hippel-Lindau (VHL) disease

Pathophysiology

  • Originate from mesenchymal cells of the capillary network
  • Composed of stromal cells and abundant capillaries
  • Tumour growth leads to cyst formation and oedema
  • VHL gene mutation implicated in pathogenesis

Demographics

  • Incidence: 1.5-2.1% of all spinal cord tumours
  • Peak age: 20-40 years
  • Male:Female ratio = 1.6:1
  • 20-30% associated with VHL disease

Diagnosis

  • Clinical presentation:
    • Gradual onset of neurological symptoms
    • Pain (radicular or local)
    • Sensory disturbances
    • Motor weakness
    • Gait abnormalities
  • Physical examination:
    • Hyperreflexia
    • Sensory level deficits
    • Motor weakness
  • Laboratory tests:
    • Genetic testing for VHL mutations
    • Elevated erythropoietin levels in some cases

Imaging

  • MRI:
    • T1-weighted: isointense to hypointense solid nodule
    • T2-weighted: hyperintense cystic component, isointense nodule
    • Contrast-enhanced T1: intense enhancement of solid nodule
    • Flow voids may be visible
  • Spinal angiography:
    • Hypervascular tumour blush
    • Enlarged feeding arteries
    • Early venous drainage

panels-1

  • 20-year-old patient presented with lower limb weakness and paraesthesia.
  • MRI showed an avidly enhancing nodule distal to a longitudinally extensive syrinx.

Treatment

  • Surgical resection:
    • Complete excision is the goal
    • Microsurgical techniques with intraoperative neurophysiological monitoring
  • Preoperative embolization:
    • May reduce intraoperative bleeding
    • Controversial due to potential complications
  • Radiation therapy:
    • Reserved for inoperable cases or residual tumour
    • Stereotactic radiosurgery for small, well-defined lesions
  • Follow-up:
    • Regular MRI surveillance
    • Screening for VHL-associated tumours in affected patients

Differential diagnosis

Differential Diagnosis Differentiating Feature
Spinal Astrocytoma Tends to be more infiltrative and less well-defined than hemangioblastomas
Spinal Cord Ependymoma Lacks cystic component typically seen in hemangioblastomas
Idiopathic syringomyelia Central fluid-filled cavity without an enhancing mural nodule
Spinal Schwannoma Typically enhances homogeneously, unlike the nodular enhancement of hemangioblastomas
Spinal Metastasis Often multiple lesions, whereas hemangioblastomas are usually solitary
Spinal Arteriovenous Malformation Flow voids on MRI, not typically seen in hemangioblastomas
Spinal Cavernoma Lacks the associated cyst seen in hemangioblastomas