title: DNA repair systems in rhabdomyosarcoma.
abstract: Rhabdomyosarcoma (RMS) represents the most common soft tissue sarcoma in children and adolescent population. There are two major histological subtypes, embryonal (ERMS) and alveolar (ARMS), differing in cytogenetic and morphological features. RMS pathogenesis remains controversial and several cellular mechanisms and pathways have been implicated. Application of intense chemo- and radio-therapy improves survival rates for RMS patients, but significant efficacy has not been proved as DNA damage induced-resistance frequently occurs. The present review is aimed at summarizing the current evidence on DNA repair systems, implications in RMS development, focusing on gene expression alterations and point mutations of genes encoding for DNA repair enzymes. Understanding of DNA repair systems involvement in RMS pathogenesis could diversify RMS patients and provide novel individualized therapeutic targets.
authors.affiliation: First Department of Pathology, Medical School, National and Kapodistrian University of Athens, Athens, Greece.
authors.forename: Panagiota G
authors.initials: PG
authors.lastname: Tsioli
authors.forename: Efstratios S
authors.initials: ES
authors.lastname: Patsouris
authors.forename: Constantinos
authors.initials: C
authors.lastname: Giaginis
authors.forename: Stamatios E
authors.initials: SE
authors.lastname: Theocharis
mesh_terms: D000293:Adolescent; D000818:Animals; D000970:Antineoplastic Agents / Q000737:chemistry; D002648:Child; D004260:DNA Repair; D004195:Disease Models, Animal; D019008:Drug Resistance, Neoplasm; D015972:Gene Expression Regulation, Neoplastic; D020022:Genetic Predisposition to Disease; D006801:Humans; D019656:Loss of Heterozygosity; D051379:Mice; D009154:Mutation; D011379:Prognosis; D011995:Recombination, Genetic; D012208:Rhabdomyosarcoma / Q000235:genetics* / Q000378:metabolism; D012509:Sarcoma / Q000235:genetics* / Q000378:metabolism; D015398:Signal Transduction; D016896:Treatment Outcome
chemical_list: D000970:Antineoplastic Agents