Abstract:

Alveolar rhabdomyosarcoma（ARMS） is a highly invasive histological subtype of rhabdomyosarcoma（RMS），which has poor prognosis with unsatisfactory response to conventional treatment on account of early metastasis potential and frequent recurrence. In recent years，with the development of research，the biological characteristics of ARMS are better understood and biotargeted therapy，which is currently the hotspot and trend of research，has promising prospect to improve the prognosis of ARMS patients. Here we describe the most recent discoveries in the molecular and cellular biology of ARMS，including the generation and carcinogenicity of characteristic fusion genes，alterations in cell signaling pathways and potential targeted drugs tested in vitro or in vivo. This review will provide new ideas for the current treatment of ARMS.

Key words: alveolar rhabdomyosarcoma, molecular biology, signaling pathways, targeted therapy

摘要：

腺泡型横纹肌肉瘤（ARMS）是横纹肌肉瘤（RMS）的一种具有高度侵袭性的组织学亚型，临床上容易发生早期转移、复发，对常规治疗手段疗效差，预后不良。近年来随着研究的深入，人们对ARMS分子生物学特征的认识逐步加深，在此基础上进行的生物靶向治疗探索作为近年的研究热点和趋势将有望改善ARMS患者的预后。文章概述了近年ARMS在分子和细胞生物学领域的最新发现，包括特征性融合基因产生和致癌途径、细胞信号通路改变以及相关靶向药物的体内外试验现状，为目前ARMS的治疗提供新的思路。

关键词: 腺泡型横纹肌肉瘤, 分子生物学, 信号通路, 靶向治疗