title: Muscular dystrophies share pathogenetic mechanisms with muscle sarcomas.
abstract: Several lines of recent evidence have opened a new debate on the mechanisms underlying the genesis of rhabdomyosarcoma, a pediatric soft tissue tumor with a widespread expression of muscle-specific markers. In particular, it is increasingly evident that the loss of skeletal muscle integrity observed in some mouse models of muscular dystrophy can favor rhabdomyosarcoma formation. This is especially true in old age. Here, we review these experimental findings and focus on the main molecular and cellular events that can dictate the tumorigenic process in dystrophic muscle, such as the loss of structural or regulatory proteins with tumor suppressor activity, the impaired DNA damage response due to oxidative stress, the chronic inflammation and the conflicting signals arising within the degenerated muscle niche.
authors.affiliation: Department of Molecular and Translational Medicine and Interuniversity Institute of Myology (IIM), University of Brescia, Viale Europa 11, Brescia, 25123, Italy. Electronic address: fanzani@med.unibs.it.
authors.forename: Alessandro
authors.initials: A
authors.lastname: Fanzani
authors.forename: Eugenio
authors.initials: E
authors.lastname: Monti
authors.forename: Rosario
authors.initials: R
authors.lastname: Donato
authors.forename: Guglielmo
authors.initials: G
authors.lastname: Sorci
mesh_terms: D000818:Animals; D002648:Child; D004249:DNA Damage / Q000235:genetics*; D015972:Gene Expression Regulation, Neoplastic; D006801:Humans; D051379:Mice; D018482:Muscle, Skeletal / Q000378:metabolism / Q000503:physiopathology; D009136:Muscular Dystrophies / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology; D018384:Oxidative Stress; D012208:Rhabdomyosarcoma / Q000209:etiology / Q000235:genetics* / Q000503:physiopathology
keywords: aging; inflammation; muscle niche; muscular dystrophy; oxidative stress; rhabdomyosarcoma