Spinal cord hemangioblastomas in von Hippel–Lindau disease

Abstract

Background. 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. Methods. The authors conducted a literature review and summation of available articles on spinal cord hemangioblastomas associated with VHL. Results. The embryological origins, epidemiology, natural history, surgical outcomes, nonsurgical treatments, and future directions in spinal cord hemangioblastomas are discussed. Conclusions. Hemangioblastomas in VHL are optimally managed with a multidisciplinary approach that includes surgical resection of symptomatic lesions. Novel treatments are gaining traction, but must be studied further for efficacy and safety.