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Rhabdomyosarcomas: an overview on the experimental animal models.


ABSTRACT: Rhabdomyosarcomas (RMS) are aggressive childhood soft-tissue malignancies deriving from mesenchymal progenitors that are committed to muscle-specific lineages. Despite the histopathological signatures associated with three main histological variants, termed embryonal, alveolar and pleomorphic, a plethora of genetic and molecular changes are recognized in RMS. Over the years, exposure to carcinogens or ionizing radiations and gene-targeting approaches in vivo have greatly contributed to disclose some of the mechanisms underlying RMS onset. In this review, we describe the principal distinct features associated with RMS variants and focus on the current available experimental animal models to point out the molecular determinants cooperating with RMS development and progression.

SUBMITTER: Zanola A 

PROVIDER: S-EPMC3823208 | biostudies-literature | 2012 Jul

REPOSITORIES: biostudies-literature

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Rhabdomyosarcomas: an overview on the experimental animal models.

Zanola Alessandra A   Rossi Stefania S   Faggi Fiorella F   Monti Eugenio E   Fanzani Alessandro A  

Journal of cellular and molecular medicine 20120701 7


Rhabdomyosarcomas (RMS) are aggressive childhood soft-tissue malignancies deriving from mesenchymal progenitors that are committed to muscle-specific lineages. Despite the histopathological signatures associated with three main histological variants, termed embryonal, alveolar and pleomorphic, a plethora of genetic and molecular changes are recognized in RMS. Over the years, exposure to carcinogens or ionizing radiations and gene-targeting approaches in vivo have greatly contributed to disclose  ...[more]

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