Targeting the extracellular matrix for NF1-associated neurofibroma treatment  

作　　者：Chunhui Jiang 

机构地区：[1]NHC Key Laboratory of Cell Transplantation,First Affiliated Hospital of Harbin Medical University,23 Youzheng Street,Harbin 150001,Heilongjiang,China

出　　处：《Chinese Journal of Plastic and Reconstructive Surgery》2024年第2期87-93,共7页

摘　　要：Neurofibromatosis type 1(NF1)is one of the most common genetic disorders that predisposes patients to benign and malignant tumors of the peripheral nervous system.Plexiform and cutaneous neurofibromas are NF1-associated benign tumors.Despite their benign nature,they can cause tremendous morbidity in patients with NF1.Therapeutic drug options are limited to the MEK inhibitor,selumetinib,which is the only approved drug for pediatric patients with plexiform neurofibromas.Antifibrotic strategies have substantial therapeutic potential for NF1-associated neurofibromas.This review discusses the fibrotic features of plexiform and cutaneous neurofi-bromas focusing on the pathological composition of the extracellular matrix.It also highlights the core pathways implicated in the biochemical and biophysical regulation of the extracellular matrix remodeling in tumor imitation and progression.Finally,this review provides a brief outlook on how exploring novel vulnerabilities residing in the aberrant extracellular matrix and their underlying pathways can benefit the treatment of NF1-associated neurofibromas.

关 键 词：Neurofibromatosis type 1 Cutaneous neurofibroma Plexiform neurofibroma FIBROSIS Extracellular matrix Basement membrane 

分 类 号：R739.4[医药卫生—肿瘤]