Suggested by MRI and confirmed by pathology (brain biopsy/resection)

Findings on Investigations:


  • Cerebellar, less commonly brainstem or spinal cord
  • Cyst with enhancing mural nodule

Other features:
Associated with von Hippel-Lindau syndrome, 3p25-26
FBC: high Hb & HCT
Pancreatic cysts, renal cyst
Retinal hemangioblastoma


Histology: Well circumscribed, cystic with nodule
Microscopically: 2 parts; prominent vascular capillaries & stromal cells. Stromal cells have vacuolated cytoplasm. Adjacent gliosis.
Immunohistochemistry: vimentin= focally positive, GFAP= weakly positive in some areas. Stromal cells negative for: factor VIII, EMA, neurofilament, keratin
Oil red O stain: foamy cells


Surgical resection with or without pre-operative embolization

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