HISTOLOGY AND HISTOPATHOLOGY

Cellular and Molecular Biology

 

Review

Advances in pediatric rhabdomyosarcoma characterization and disease model development

Dennis O’ Brien, Aishwarya G. Jacob, Stephen J. Qualman and Dawn S. Chandler

The Center for Childhood Cancer, Columbus Children’s Research Institute and the Department of Pediatrics, The Ohio State University, Columbus, Ohio, USA.

Offprint requests to: Dawn S. Chandler, Ph.D., Center for Childhood Cancer, WA5023, Children’s Research Institute, 700 Children’s Drive, Columbus, OH 43205, Ohio, USA. e-mail: chandler.135@osu.edu


Summary. Rhabdomyosarcoma (RMS), a form of soft tissue sarcoma, is one of the most common pediatric malignancies. A complex disease with at least three different subtypes, it is characterized by perturbations in a number of signaling pathways and genetic abnormalities. Extensive clinical studies have helped classify these tumors into high and low risk groups to facilitate different treatment regimens. Research into the etiology of the disease has helped uncover numerous potential therapeutic intervention points which can be tested on various animal models of RMS; both genetically modified models and tumor Xenograft models. Taken together, there has been a marked increase in the survival rate of RMS patients but the highly invasive, metastatic forms of the disease continue to baffle researchers. This review aims to highlight and summarize some of the most important developments in characterization and in vivo model generation for RMS research, in the last few decades
. Histol Histopathol 27, 13-22 (2012)

Key words: RMS subtypes, Sarcoma, Mouse models

DOI: 10.14670/HH-27.13