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

Author(s): Marshall Amy D | Grosveld Gerard C

Journal: Skeletal Muscle
ISSN 2044-5040

Volume: 2;
Issue: 1;
Start page: 25;
Date: 2012;
Original page

Keywords: Alveolar rhabdomyosarcoma | PAX3-FOXO1 | PAX7-FOXO1 | FGFR4 | CNR1 | IRIZIO | N-MYC | IGF2 | MET | CXCR4 | p53 | MDM2 | P-Cadherin | TFAP2B | miR17-92

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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