Research Progress on Tumor Microenvironment in Neurofibromatosis Type 2 Related Vestibular Schwannoma
LI Yuchen
TAO Bingyan
ZHANG Jun
Abstract：Neurofibromatosis type 2(NF2)is a rare autosomal dominant disease.The NF2 gene is located in the tumor suppressor gene on chromosome 22q12.2.Systemic multiple tumor syndrome is caused by mutation of this gene.Bilateral vestibular schwannoma(BVS)is the most significant feature of NF2.Compared with sporadic vestibular schwannoma(VS),blood supply in NF2-BVS is relatively abundant and NF2 tumors are prone to recurrence,similar to low-grade malignant tumors.Therefore,similarities between NF2-BVS and sporadic VS need to be further studied.In addition,since NF2-BVS is mainly surgically treated and most patients require multiple operations,complications such as hearing loss are not uncommon with treatment outcomes and quality of life far less favorable than in sporadic VS.Ad-vances in studies on tumor microenvironment(TME)and tumor associated macrophages(TAMs)that promote tumor growth prompt a need to explore new treatment methods for NF2-BVS based on TME.This review provides a summary of characteristics of NF2-BVS and the deficiencies in current treatment methods,promotion and inhibition of tumor growth by tumor associated macrophages,the role of extracellular matrix(ECM)on tumor growth,research on VS extra-cellular matrix,and studies on the feasibility of ECN related nf2-bvs treatment methods.Innovative therapies for VS in relation to TME that exploit the differences in clinical manifestations between VS and BVS are also discussed.
Keywords：neurofibromatosis Type 2bilateral vestibular schwannomatumor microenvironmentExtracellular matrix
Publication Date：2023-12-28
Online Publishing Date：2025-08-15(First online date of this platform, not the publication date of the document)
Pages：7( 897-903 )