Molecular biology of sporadic vestibular schwannomas including genetic and epigenetic alterations

Abstract

Introduction: Vestibular schwannomas (VSs) are benign tumors, developing from neoplastic Schwann cells, that produce myelin sheath covering vestibular nerve fibers. The majority of these tumors are sporadic VS and very rarely they can be associated with neurofibromatosis type 2 (NF2). VSs are usually characterized with slow growth rate, however some of them appear to have a cystic structure, grow more rapidly, can cause facial nerve palsy and brainstem compression. The molecular hallmark of both sporadic and NF-2 associated VS is the inactivation of supresor gene NF-2, also called merlin gene. Aim: In this review we summarize the current knowledge on the molecular biology of VS, including genetic and epigenetic aberrations, altered gene expression and specific microRNA expression profiles.