Hemangioblastoma

• Benign neoplasm
• 80% found in cerebellum
  • Remainder are located in spinal cord > medulla > cerebrum
• Account for 10% of posterior fossa masses (vestibular schwannomas and metastases rule here.)
• Most often occurs in ages 30 to 40
  • M > F
• Relationship to von Hippel-Lindau disease
  • 20% occur in patients with von Hippel-Lindau disease (multiple lesions).
  • 35-60% of von Hippel-Lindau disease patients have hemangioblastomas
  • von Hippel-Lindau disease consists of
    • Retinal, spinal, cerebellar and medullary hemangioblastomas
    • Renal cysts and carcinomas
    • Pancreatic cysts
    • Pheochromocytomas
    • Papillary Cystadenoma of the epididymis
• Clinical findings
  • Headache
  • Ataxia
  • Nausea
  • Vomiting
  • Vertigo
  • Polycythemia caused by increased erythropoietin found in 40%.
  • Spinal lesions may present with subarachnoid hemorrhage
• Findings on CT and MRI:
  • Cystic lesion in the cerebellum with an avidly enhancing mural nodule (75%)
  • Purely solid enhancing lesion (10%)
  • Enhancing lesion with multiple cystic areas (15%)

MRI of brain shows a cystic lesion in the cerebellum with an enhancing nodule (post-Gadolinium)

• Findings on angiography:
  • Vascular nodule within an avascular mass
  • Serpentine vessels
• Treated with surgical removal of solid nodule
  • Cystic component is not neoplastic
• DDx:

o       Similar appearance to Juvenile pilocytic astrocytoma

§         But that is typically found in patients 5 to 15 years of age.