Original Publication Date: 1 December, 2015
Publication / Source: CNS Oncology
Authors: Denis Migliorini, Sven Haller, Doron Merkler et al.
Hemangioblastoma is a rare benign neoplasm, accounting for less than 2% of all primitive brain tumors. It may arise sporadically in a solitary form, or associated with Von Hippel–Lindau (VHL) disease with multiple tumors. Surgery is the mainstay treatment, but management is challenging in case of recurrent and/or multiple tumors. VHL protein is defective in both forms of hemangioblastoma, leading to the accumulation of hypoxia-inducible factor, stimulating angiogenesis via VEGF and PDGF mainly. Here, we report a 37-year-old woman’s case with recurrent and rapidly progressive VHL-associated hemangioblastomas, causing severe disability.