Purely extradural spinal nerve root hemangioblastomas

Abstract

Spinal nerve root hemangioblastomas present mostly as intradural-extradurally. Purely extradural spinal nerve root hemangioblastoma is a very rare entity. In this study, we aimed to analyze epidemiological perspectives of purely extradural spinal nerve root hemangioblastomas presented in English medical literature in addition to our own exemplary case. PubMed/MEDLINE was searched using the terms ``hemangioblastoma,{''} ``extradural,{''} ``spinal,{''} and ``nerve root.{''} Demographical variables of age, gender, concomitant presence of von Hippel-Lindau (VHL) disease

spinal imaging and/or intraoperative findings for tumor location were surveyed from retrieved articles. There are 38 patients with purely extradural spinal nerve root hemangioblastoma. The median age is 45 years (range = 24-72 years). Female: male ratio is 0.6. Spinal levels for purely extradural spinal nerve root hemangioblastomas, in order of decreasing frequency, are thoracic (48.6\%), cervical (13.5\%), lumbar (13.5\%), lumbosacral (10.8\%), sacral (8.1\%), and thoracolumbar (5.4\%). Concomitant presence of VHL disease is 45\%. Purely extradural spinal nerve root hemangioblastomas are very rare and can be confused with other more common extradural spinal cord tumors. Concomitant presence of VHL disease is observed in less than half of the patients with purely extradural spinal nerve root hemangioblastomas. Surgery is the first-line treatment in these tumors.