title: 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.
authors.affiliation: Department of Neurosurgery, Vocational School of Health Services, Acıbadem University, Istanbul, Turkey.
authors.forename: Murat Hamit
authors.initials: MH
authors.lastname: Aytar
authors.affiliation: Department of Neurosurgery, Acıbadem University School of Medicine, Istanbul, Turkey.
authors.forename: Ulaş
authors.initials: U
authors.lastname: Yener
authors.affiliation: Department of Orthopedic Surgery-Spine Center, University of California at San Francisco, CA, USA.
authors.forename: Murat Şakir
authors.initials: MŞ
authors.lastname: Ekşi
authors.affiliation: Department of Neurosurgery, Acıbadem Maslak Hospital, Istanbul, Turkey.
authors.forename: Behram
authors.initials: B
authors.lastname: Kaya
authors.affiliation: Department of Neurosurgery, Acıbadem University School of Medicine, Istanbul, Turkey.
authors.forename: Serdar
authors.initials: S
authors.lastname: Özgen
authors.affiliation: Department of Pathology, Acıbadem University School of Medicine, Istanbul, Turkey.
authors.forename: Aydin
authors.initials: A
authors.lastname: Sav
authors.affiliation: Department of Orthopedics and Traumatology, Acıbadem University School of Medicine, Istanbul, Turkey.
authors.forename: Ahmet
authors.initials: A
authors.lastname: Alanay
keywords: Extradural; hemangioblastoma; spine; surgery; von Hippel–Lindau