Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
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.

PubMed Disclaimer

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
See this image and copyright information in PMC

References

    1. Anderson MJ, Shelton GD, Cavenee WK, Arden KC. Embryonic expression of the tumor-associated PAX3-FKHR fusion protein interferes with the developmental functions of Pax3. Proc. Natl. Acad. Sci. USA. 2001;98:1589–1594. - PMC - PubMed
    1. Bartel F, Taylor AC, Taubert H, Harris LC. Novel mdm2 splice variants identified in pediatric rhabdomyosarcoma tumors and cell lines. Oncol. Res. 2001a;12:451–457. - PubMed
    1. Bartel F, Meye A, Wurl P, Kappler M, Bache M, Lautenschlager C, Grunbaum U, Schmidt H, Taubert H. Amplification of the MDM2 gene, but not expression of splice variants of MDM2 MRNA, is associated with prognosis in soft tissue sarcoma. Int. J. Cancer. 2001b;95:168–175. - PubMed
    1. Bartel F, Schulz J, Bohnke A, Blumke K, Kappler M, Bache M, Schmidt H, Wurl P, Taubert H, Hauptmann S. Significance of HDMX-S (or MDM4) mRNA splice variant overexpression and HDMX gene amplification on primary soft tissue sarcoma prognosis. Int. J. Cancer. 2005;117:469–475. - PubMed
    1. Bennicelli JL, Fredericks WJ, Wilson RB, Rauscher FJ, 3rd, Barr FG. Wild type PAX3 protein and the PAX3-FKHR fusion protein of alveolar rhabdomyosarcoma contain potent, structurally distinct transcriptional activation domains. Oncogene. 1995;11:119–130. - PubMed

Publication types