Molecular pathogenesis of desmoid tumor and advances in its treatment
10.12354/j.issn.1000-8179.2024.20240181
- VernacularTitle:硬纤维瘤的分子发病机制和治疗进展
- Author:
Wu HONGYU
1
;
Li ZHUOYU
;
Liu WEIFENG
Author Information
1. 北京大学第四临床医学院骨肿瘤科(北京市 100035);首都医科大学附属北京积水潭医院骨肿瘤科;国家骨科医学中心;北京积水潭骨科机器人工程研究中心有限公司
- Keywords:
desmoid tumor(DT);
molecular pathogenesis;
treatment advances;
treatment strategies;
nirogacestat
- From:
Chinese Journal of Clinical Oncology
2024;51(8):418-424
- CountryChina
- Language:Chinese
-
Abstract:
Desmoid tumors(DT)are rare,locally aggressive tumors that lack metastatic ability but tend to grow invasively and recur locally.This can severely impair patient function,thereby causing significant clinical burden and even death.Over the past 20 years,there has been a significant shift in the treatment philosophy for this disease.Conservative methods with active surveillance are being employed as the first-line of treatment as opposed to aggressive surgeries that were previously the norm.The range of treatment options has also expanded,re-vealing a trend towards multidisciplinary management.Studies have indicated the pathogenesis of DT to be linked to the dysregulation of Wnt and Notch signaling pathways.Several new drugs targeting these pathways,such as nirogacestat,are currently under development and are expected to be a part of future treatments for this disease.This article reviews the molecular pathogenesis and advances in the treat-ment of DT,providing a basis and direction for clinical treatment and future research on this disease.
