Molecular biology of sporadic vestibular schwannomas including genetic and epigenetic alterations

Introduction: Vestibular schwannomas (VS) are benign tumors developing from the myelin-producing Schwann cells, which surround the vestibular branches of the auditory nerve. The vast majority occur sporadically and a small proportion are associated with neurofibromatosis type 2 (NF2). Most sVS are slow-growing neoplasms; however some have a cystic structure, show more rapid growth, cause more frequently paralysis of the facial nerve, and brainstem compression. The molecular hallmark of both sporadic and NF-2 associated VS is the inactivation of the tumor-suppressor gene NF2, also called merlin gene. Purpose: The paper presents the current knowledge on the molecular biology of VS, including: information on genetic and epigenetic aberrations, changes in gene expression and specific microRNA expression profiles.