%0 Journal Article
%T Alveolar rhabdomyosarcoma ¨C The molecular drivers of PAX3/7-FOXO1-induced tumorigenesis
%A Marshall Amy D
%A Grosveld Gerard C
%J Skeletal Muscle
%D 2012
%I BioMed Central
%R 10.1186/2044-5040-2-25
%X 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.
%K Alveolar rhabdomyosarcoma
%K PAX3-FOXO1
%K PAX7-FOXO1
%K FGFR4
%K CNR1
%K IRIZIO
%K N-MYC
%K IGF2
%K MET
%K CXCR4
%K p53
%K MDM2
%K P-Cadherin
%K TFAP2B
%K miR17-92
%U http://www.skeletalmusclejournal.com/content/2/1/25