Benign brain tumors and tumors associated with phakomatoses

This article reviews the clinical features, diagnostic criteria, molecular genetics, and management of sporadic meningiomas and vestibular schwannomas and tumors associated with the major neurocutaneous disorders.

Recent advances in cancer genomics are now allowing sequencing of the genome of these tumors to identify oncogenic drivers and possible therapeutic targets.

Sporadic meningiomas and schwannomas, and tumors occurring as part of a neurocutaneous syndrome, have been the subjects of strong biological and therapeutic interest in the past few years; new and exciting therapies are either under investigation or will be in the upcoming years. This article takes an in-depth look at sporadic meningiomas and schwannomas, followed by a review of the phakomatoses and their associated tumors.