MicroRNAs in rhabdomyosarcoma: pathogenetic implications and translational potentiality

• Main Authors: Giordano Antonio, Ciarapica Roberta, Rota Rossella, Miele Lucio, Locatelli Franco
• Format: Article
• Language: English
• Published: BMC 2011-09-01
• Series: Molecular Cancer
• Online Access: http://www.molecular-cancer.com/content/10/1/120

<p>Abstract</p> <p>There is growing evidence that interconnections among molecular pathways governing tissue differentiation are nodal points for malignant transformation. In this scenario, microRNAs appear as crucial players. This class of non-coding small regulatory RNA molecules controls developmental programs by modulating gene expression through post-transcriptional silencing of target mRNAs. During myogenesis, muscle-specific and ubiquitously-expressed microRNAs tightly control muscle tissue differentiation. In recent years, microRNAs have emerged as prominent players in cancer as well. Rhabdomyosarcoma is a pediatric skeletal muscle-derived soft-tissue sarcoma that originates from myogenic precursors arrested at different stages of differentiation and that continue to proliferate indefinitely. MicroRNAs involved in muscle cell fate determination appear down-regulated in rhabdomyosarcoma primary tumors and cell lines compared to their normal counterparts. More importantly, they behave as tumor suppressors in this malignancy, as their re-expression is sufficient to restore the differentiation capability of tumor cells and to prevent tumor growth <it>in vivo</it>. In addition, up-regulation of pro-oncogenic microRNAs has also been recently detected in rhabdomyosarcoma.</p> <p>In this review, we provide an overview of current knowledge on microRNAs de-regulation in rhabdomyosarcoma. Additionally, we examine the potential of microRNAs as prognostic and diagnostic markers in this soft-tissue sarcoma, and discuss possible therapeutic applications and challenges of a "microRNA therapy".</p>