Continuous angiogenesis inhibition in the treatment for von Hippel–Lindau-related hemangioblastomas of retina and spinal cord

Abstract

Hemangioblastomas of central nervous system are rare and indolent. Twenty-five percent of cases are in association with von Hippel–Lindau disease. Surgery is the standard therapy but un-resectable or recurrent cases need radiation or systemic therapy. Defective von Hippel–Lindau tumor suppressor gene leads to vascular endothelial growth factor overexpression and enhance angiogenesis. Here we report a 19-year-old male, diagnosed at pediatric age, who had retinal and spinal cord hemangioblastomas. He was treated 34 months with bevacizumab, afterwards 12 months with thalidomide and tertiary therapy with pazopanib for 9 months which still goes on. In case of need, radiation and surgical procedures were performed. Vascular endothelial growth factor inhibition continuity is a good therapeutic option, which improves outcomes of von Hippel–Lindau-related hemangioblastomas.