Current management and advances in the treatment of Vestibular Schwannomatosis

Abstract

Introduction: vestibular schwannomatosis (SV), also known as acoustic neurinoma or NF2, is a genetic mutation in the NF2 gene located on chromosome 22, characterized by loss of function of a tumor suppressor protein that has several names among them: Merlin, Neurofibromin 2 and schwannomin.  It is classified as a hereditary multiple neoplasm syndrome with an incidence of 1:25,000 to 1:40,000. Objectives: to determine the current management and progress in pharmacological treatment of vestibular Schwannomatosis Methods: a systematic search was carried out with the PRISMA 2020 statement of studies or research on VS, as search words were used the terms Schwannomatosis vestibular, Schwannoma vestibular, acoustic neuroma, published in the last 5 years. For the selection, observational studies, randomized and non-randomized clinical trials, systematic reviews and meta-analyses were used; studies with missing, unavailable data were excluded. Results: gamma-ray scalpel radiosurgery is a treatment that helps stabilize tumor growth and improves hearing.  Stereotactic radiosurgery for vestibular schwannoma has a high tumor control rate of 90.7%, excellent preservation of the facial nerve and low incidence of tinnitus and vertigo. Surgical management with a low failure rate can be used for tumor recurrence. Conclusions: treatment with monoclonal antibodies such as bevacizumab is a breakthrough in research, whose mechanism of action allows acting as an antiangiogenic that inhibits the vascularization of tumors by slowing their development. The main goal of treatment is to stop the growth of the tumor, which can improve symptoms such as hearing loss. Response to treatment will depend on tumor size, location and associated symptomatology. The surgical approach consists of microsurgery or stereotactic surgery with good results.