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.

Address correspondence to Dr Thomas J. Kaley, Memorial Sloan Kettering Cancer Center, Department of Neurology, 1275 York Avenue, New York, NY 10065, [email protected].

Relationship Disclosure: Drs Daras and Kaley report no disclosures.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Daras and Kaley discuss the unlabeled use of chemotherapies and hydroxyurea in the treatment of sporadic benign brain tumors and those related to phakomatoses.