Cord compression due to extradural thoracic nerve root hemangioblastoma

Hemangioblastomas (HGBs) are benign tumors that occur sporadically or as part of von Hippel-Lindau (VHL) disease. 6-8% of spinal HGBs are extramedullary in location, and basically referred to as HGB of the spinal nerve root. Purely extradural (ED) location is the rarest form of these tumors. We report a case of a non-VHL patient with large thoracic ED HGB, who presented with myelopathy due to cord compression. We emphasize the importance of correct pre-operative diagnosis as well as pre-operative embolization in order to reach the goal of complete tumor resection with minimal complications. We also review the relevant literature and summarize the few case reports of this unique tumor.