Dysregulated Myogenesis in Rhabdomyosarcoma

Curr Top Dev Biol. 2018:126:285-297. doi: 10.1016/bs.ctdb.2017.10.007. Epub 2017 Oct 31.

Abstract

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.

Keywords: Aarcoma; Differentiation; Myogenesis; Rhabdomyosarcoma; Skeletal muscle.

Publication types

  • Review

MeSH terms

  • Cell Differentiation / genetics
  • Gene Expression Regulation, Developmental*
  • Gene Expression Regulation, Neoplastic*
  • Humans
  • MicroRNAs / genetics
  • Models, Genetic
  • Muscle Development / genetics*
  • Muscle, Skeletal / metabolism
  • Muscle, Skeletal / pathology
  • Rhabdomyosarcoma / genetics*
  • Rhabdomyosarcoma / pathology
  • Signal Transduction / genetics

Substances

  • MicroRNAs