Craniospinal Hemangioblastomas

von Hippel-Lindau disease (VHL) is an autosomal dominant neoplasia syndrome that predisposes patients to visceral and central nervous system lesions. VHL-associated central nervous system lesions include hemangioblastomas and endolymphatic sac tumors. Craniospinal hemangioblastomas are the most common tumors in VHL. While they are benign tumors, they are the most common cause of morbidity in VHL. These tumors grow in a saltatory pattern (characterized by periods of growth and quiescence). Surgery is the treatment of choice for symptomatic tumors. We describe the natural history, associated clinical findings, and management.