Spinal cord hemangioblastomas in von Hippel-Lindau disease

Abstract

Abstract von Hippel-Lindau disease (VHL) is an autosomal dominant familial neoplasia syndrome. The most common manifestation of VHL is central nervous system hemangioblastomas. VHL patients will often develop multiple hemangioblastomas along their craniospinal axis over their lifetime. Spinal cord hemangioblastomas account for nearly half of all nervous system hemangioblastomas in VHL. Because of the protean and multiple nature of hemangioblastomas in VHL, these tumors are optimally managed with a multidisciplinary approach that includes surgical resection of symptomatic lesions.