横纹肌肉瘤是起源于骨骼肌的间叶源性肿瘤,常发生于15岁以下的儿童和青少年。由于不同类型横纹肌肉瘤的组织学特征、发病年龄、部位和预后各具差异,迄今为止横纹肌肉瘤的分子机制仍不明确。近年来,关于其分子机制的研究主要集中于关键基因和信号通路等方面,研究发现成纤维生长因子受体信号通路与横纹肌肉瘤的生长及转移密切相关,在多数上皮源性肿瘤中已证实上皮间叶转化促进了肿瘤增生与转移,因此笔者提出在横纹肌肉瘤中上皮间叶转化可能通过成纤维生长因子受体信号通路促进肿瘤增生和转移的假设,结合成纤维生长因子受体信号通路和上皮间叶转化来试着解释横纹肌肉瘤的发生、发展,以期为其治疗和预后评估提供一定的帮助。
Rabdomyosarcoma (RMS), common in the children and adolescence, is a mesenchymal malignancy derived from skeletal muscle. RMS can be classified into 3 main subtypes, more aggressive alveolar rhabdomyosarcoma (ARMS), more common, and better outcome embryonal rhabdomyosarcoma (ERMS) and the rare pleomorphic rhabdomyosarcoma (PRMS). The molecular mechanism for RMS is still unclear due to the different types of RMS with differences in histologic characteristic, age of onset, location, and prognosis. Recent studies are mainly focused on the key genes and signaling pathways in RMS. It has been found that fibroblast growth factor receptor (FGFR) signaling pathway is strongly associated with the proliferation and metastasis of RMS. More interestingly, it has been confirmed that epithelial-mesenchymal transition (EMT) promotes the proliferation and metastasis in lots of epithelial tumors. So we hypothesize that EMT promotes proliferation and metastasis via FGFR signaling pathway in RMS. In this article we tried to understand the development of RMS via EMT and FGFR signaling pathway. Hopefully, it can offer a new view for the treatment and prognosis assessment of RMS.