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Sarcoma. 2012;2012:695603. doi: 10.1155/2012/695603. Epub 2012 Mar 11.

Notch, wnt, and hedgehog pathways in rhabdomyosarcoma: from single pathways to an integrated network.

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  • 1Laboratori de Recerca Translacional en el Càncer Pediàtric, Hospital Universitari Vall d'Hebron, Universitat Autònoma de Barcelona, Pg Vall d'Hebron 119-129 08035, Spain.


Rhabdomyosarcoma (RMS) is the most common type of soft tissue sarcoma in children. Regarding histopathological criteria, RMS can be divided into 2 main subtypes: embryonal and alveolar. These subtypes differ considerably in their clinical phenotype and molecular features. Abnormal regulation or mutation of signalling pathways that regulate normal embryonic development such as Notch, Hedgehog, and Wnt is a recurrent feature in tumorigenesis. Herein, the general features of each of the three pathways, their implication in cancer and particularly in RMS are reviewed. Finally, the cross-talking among these three pathways and the possibility of better understanding of the horizontal communication among them, leading to the development of more potent therapeutic approaches, are discussed.

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