Benign brain tumors and tumors associated with phakomatoses.

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

SUMMARY: 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.