Case of the Week
Section Editors: Matylda Machnowska1 and Anvita Pauranik2
1University of Toronto, Toronto, Ontario, Canada
2BC Children's Hospital, University of British Columbia, Vancouver, British Columbia, Canada
Sign up to receive an email alert when a new Case of the Week is posted.
June 29, 2009
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.
Current Issue
Case Collections
