Hemangioblastoma
Hemangioblastoma
Updated: 10/20/2020
© Jun Wang, MD, PhD
General features
- Benign
- Intracranial tumor
- Associated with von Hippel-Lindau syndrome
- Sporadic cases with somatic VHL mutation
Clinical presentations
- Most common location: cerebellum
- Can be seen in spinal cord, meninges
- Symptoms associated with location due to mass effect
- Multiple tumors in Von Hippel-Lindau syndrome patients
Pathogenesis
- Loss of VHL promotes increased production of vascular endothelial growth factor and erythropoietin
Morphological features
- Reddish neoplasm
- Packed capillary type vascular proliferation
- Neoplastic stromal cells
Genetic abnormalities
- VHL mutation
Management
- Surgery
- Radiation
- Antiangiogenic therapy
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