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Review
. 2012 Jan;27(1):13-22.
doi: 10.14670/HH-27.13.

Advances in pediatric rhabdomyosarcoma characterization and disease model development

D O'Brien  1 , A G Jacob, S J Qualman, D S Chandler
Affiliations
Review

Advances in pediatric rhabdomyosarcoma characterization and disease model development

D O'Brien et al. Histol Histopathol. 2012 Jan.

Abstract

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.

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Figures

Fig. 1
Fig. 1
Typical light microscopic appearance of pediatric rhabdomyosarcoma subtypes. A. Embryonal RMS (NOS). B. Spindle-cell variant of embryonal RMS. C. Botryoid variant of embryonal RMS. D. Alveolar RMS. E. Solid variant of alveolar RMS. F. Anaplastic RMS. Haematoxylin and eosin, × 100
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