Arthur G. James Comprehensive Cancer Center, The Ohio State University, Columbus, OH, United States; College of Medicine, The Ohio State University, Columbus, OH, United States.
Arthur G. James Comprehensive Cancer Center, The Ohio State University, Columbus, OH, United States; The Ohio State University, Columbus, OH, United States.
Curr Top Dev Biol. 2018;126:285-297. doi: 10.1016/bs.ctdb.2017.10.007. Epub 2017 Oct 31.
Rhabdomyosarcoma is a mesenchymal malignancy associated with the skeletal muscle lineage and is also the most common pediatric soft tissue cancer. Between the two pediatric subtypes, embryonal and alveolar rhabdomyosarcoma, the alveolar subtype is generally more aggressive and high-risk. Despite intensive multimodal therapy, patients with high-risk rhabdomyosarcoma continue to have poor prognosis. In this chapter we address the mechanisms underlying the dysregulation of myogenesis in rhabdomyosarcoma. We specifically focus on recently identified signaling pathways that function to inhibit myogenesis and how similar functions have been shown to overlap in rhabdomyosarcoma, potentially contributing to the disease.
横纹肌肉瘤是一种间叶性恶性肿瘤,与骨骼肌谱系相关,也是最常见的儿童软组织癌。在两种儿科亚型(胚胎性和肺泡性横纹肌肉瘤)中,肺泡性亚型通常更具侵袭性和高风险。尽管采用了强化的多模式治疗,但高危横纹肌肉瘤患者的预后仍然较差。在本章中,我们将探讨横纹肌肉瘤中肌生成失调的机制。我们特别关注最近发现的抑制肌生成的信号通路,以及这些信号通路在横纹肌肉瘤中的相似功能如何重叠,这可能是导致该疾病的原因之一。