Hemangioblastomas in Von Hippel–Lindau Disease
- CNS hemangioblastoma is one of the most common manifestations of VHL disease.
- Typical sites: cerebellum (44%–72%), spinal cord (13%–59%), and medulla (5%). Supra-tentorial lesions are less common. Only 5%–30% of all cerebellar hemangioblastomas are attributed to VHL disease, whereas 80% of spinal cord hemangioblastomas occur with the disease.
- Highly vascular lesions that readily enhance with contrast material. They may be solid, cystic, hemorrhagic, or mixed. They are often cystic with a solid enhancing mural nodule.
- Diagnostic criteria for VHL disease include the following: (a) more than one CNS hemangioblastoma, (b) one CNS hemangioblastoma and visceral manifestations of VHL disease, and (c) any manifestation and a known family history of VHL disease.
Suggested Reading
Leung RS, Biswas SV, Duncan M, et al. Imaging Features of von Hippel-Lindau Disease. RadioGraphics 2008;28:65-79.
Conway JE, Chou D, Clatterbuck RE, et al. Hemangioblastomas of the Central Nervous System in von Hippel-Lindau Syndrome and Sporadic Disease. Neurosurgery 2001;48:55-62.