There is grade 3 evidence for the use of angiogenesis inhibitors as salvage systemic therapy in vHL and/or sporadic hemangioblastomas, for instance interferon-beta [16], VEGF-targeting tyrosine kinase inhibitors such as sunitinib [17] and semaxanib [18] and monoclonal antibodies such as bevacixumab [19]. This evidence concerns the gene VEGFA and hemangioblastoma.