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Marshall AD, Grosveld GC. Alveolar rhabdomyosarcoma - The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis. Skelet Muscle. 2012;2(1):25doi: 10.1186/2044-5040-2-25.
Marshall, A. D., & Grosveld, G. C. (2012). Alveolar rhabdomyosarcoma - The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis. Skeletal muscle, 2(1), 25. https://doi.org/10.1186/2044-5040-2-25
Marshall, Amy D, and Grosveld, Gerard C. "Alveolar rhabdomyosarcoma - The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis." Skeletal muscle vol. 2,1 (2012): 25. doi: https://doi.org/10.1186/2044-5040-2-25
Marshall AD, Grosveld GC. Alveolar rhabdomyosarcoma - The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis. Skelet Muscle. 2012 Dec 03;2(1):25. doi: 10.1186/2044-5040-2-25. PMID: 23206814; PMCID: PMC3564712.
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Skelet Muscle. 2012 Dec 03;2(1):25. doi: 10.1186/2044-5040-2-25.

Alveolar rhabdomyosarcoma - The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis.

Skeletal muscle

Amy D Marshall, Gerard C Grosveld

Affiliations

  1. Department of Genetics, St Jude Children's Research Hospital, Memphis, TN, 38105, USA. [email protected].

PMID: 23206814 PMCID: PMC3564712 DOI: 10.1186/2044-5040-2-25

Abstract

Rhabdomyosarcoma is a soft tissue sarcoma arising from cells of a mesenchymal or skeletal muscle lineage. Alveolar rhabdomyosarcoma (ARMS) is more aggressive than the more common embryonal (ERMS) subtype. ARMS is more prone to metastasis and carries a poorer prognosis. In contrast to ERMS, the majority of ARMS tumors carry one of several characteristic chromosomal translocations, such as t(2;13)(q35;q14), which results in the expression of a PAX3-FOXO1 fusion transcription factor. In this review we discuss the genes that cooperate with PAX3-FOXO1, as well as the target genes of the fusion transcription factor that contribute to various aspects of ARMS tumorigenesis. The characterization of these pathways will lead to a better understanding of ARMS tumorigenesis and will allow the design of novel targeted therapies that will lead to better treatment for this aggressive pediatric tumor.

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