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Alveolar rhabdomyosarcoma (ARMS) is a subtype of rhabdomyosarcoma and usually occurs in childhood and adolescence. ARMS is characterized by its aggressive behavior and poor prognosis. To improve the unfavorable prognosis, new therapeutic developments and the establishment of methods for precise prognostic prediction are required.
View Article and Find Full Text PDFAlveolar rhabdomyosarcoma-associated proteins PAX3/FOXO1A and PAX7/FOXO1A suppress the transcriptional activity of MyoD-target genes in muscle stem cells.
Oncogene
January 2013
King's College London, Randall Division of Cell and Molecular Biophysics, London, UK.
Rhabdomyosarcoma (RMS) is the commonest soft-tissue sarcoma in childhood and is characterized by expression of myogenic proteins, including the transcription factors MyoD and myogenin. There are two main subgroups, embryonal RMS and alveolar RMS (ARMS). Most ARMS are associated with chromosomal translocations that have breakpoints in introns of either PAX3 or PAX7, and FOXO1A.
View Article and Find Full Text PDFAKT and PAX3-FKHR cooperation enforces myogenic differentiation blockade in alveolar rhabdomyosarcoma cell.
Cell Cycle
March 2012
Department of Cell Stress Biology, Roswell Park Cancer Institute, Buffalo, NY, USA.
The chimeric PAX3-FKHR transcription factor is present in a majority of alveolar rhabdomyosarcoma (ARMS), an aggressive skeletal muscle cancer of childhood. PAX3-FKHR-mediated aberrant myogenic gene expression resulting in escape from terminal differentiation program is believed to contribute in ARMS development. In skeletal muscle differentiation, activation of AKT pathway leads to myogenic gene activation and terminal differentiation.
View Article and Find Full Text PDFPax7-FKHR transcriptional activity is enhanced by transcriptionally repressed MyoD.
J Cell Biochem
May 2011
Facultad de Ciencias Biológicas, Departamento Biología Celular y Molecular, Pontificia Universidad Católica de Chile, Alameda 340, Santiago, Chile.
Alveolar rhabdomyosarcoma (ARMS) are characterized by the expression of chimeric transcription factors Pax3-FKHR and Pax7-FKHR, due to chromosomal translocations fusing PAX3 or PAX7 with the FKHR gene. Although ARMS exhibits a muscle lineage phenotype, the cells evade terminal differentiation despite expressing the potent myogenic transcriptional regulator MyoD. Here we show that while Pax7-FKHR inhibits MyoD-dependent transcription, MyoD enhances Pax7-FKHR activity in myogenic cell cultures.
View Article and Find Full Text PDFTesticular fusocellular rhabdomyosarcoma as a metastasis of elbow sclerosing rhabdomyosarcoma: A clinicopathologic, immunohistochemical and molecular study of one case.
Diagn Pathol
August 2010
Department of Pathology, Hospital General Universitario de Valencia, University of Valencia, Spain.
Sclerosing rhabdomyosarcoma (SRMS) is an infrequent variant of rhabdomyosarcoma characterized by extensive intercellular hyaline fibrosis. We report the case of a 37 year-old male with a 9 x 6 cm SRMS on the right elbow. Histologically, the tumor showed an abundant extracellular hyaline matrix with extratumoral vascular emboli and microscopic foci of fusocellular embryonal rhabdomyosarcoma (FRMS) separated by a fibrotic band from the sclerosing areas.
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