Hemangioblastomas of the central nervous system

Hemangioblastomas are rare tumors, accounting for around 2% of all CNS tumors. They can occur anywhere in the neuraxis, but are most common in the posterior fossa (80-90%), followed by the spinal cord (10-20%).Although histologically benign, hemangioblastomas can be associated with significant morbidity and mortality, due either to the mass effect of tumor progression, or to visceral localization in VHL disease, or to repeated neurological or visceral interventions.Over the last few decades, the management of hemangioblastomas has undergone major changes, both diagnostically and therapeutically.In this monograph, we propose to review the epidemiological, clinical, biological, radiological and evolutionary aspects of hemangioblastomas of the central nervous system, and to identify and discuss the various therapeutic modalities.