Distinct and overlapping sarcoma subtypes initiated from muscle stem and progenitor cells.

Journal Article (Journal Article)

Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma in children, whereas undifferentiated pleomorphic sarcoma (UPS) is one of the most common soft tissue sarcomas diagnosed in adults. To investigate the myogenic cell(s) of origin of these sarcomas, we used Pax7-CreER and MyoD-CreER mice to transform Pax7(+) and MyoD(+) myogenic progenitors by expressing oncogenic Kras(G12D) and deleting Trp53 in vivo. Pax7-CreER mice developed RMS and UPS, whereas MyoD-CreER mice developed UPS. Using gene set enrichment analysis, RMS and UPS each clustered specifically within their human counterparts. These results suggest that RMS and UPS have distinct and overlapping cells of origin within the muscle lineage. Taking them together, we have established mouse models of soft tissue sarcoma from muscle stem and progenitor cells.

Full Text

Duke Authors

Cited Authors

  • Blum, JM; Añó, L; Li, Z; Van Mater, D; Bennett, BD; Sachdeva, M; Lagutina, I; Zhang, M; Mito, JK; Dodd, LG; Cardona, DM; Dodd, RD; Williams, N; Ma, Y; Lepper, C; Linardic, CM; Mukherjee, S; Grosveld, GC; Fan, C-M; Kirsch, DG

Published Date

  • November 27, 2013

Published In

Volume / Issue

  • 5 / 4

Start / End Page

  • 933 - 940

PubMed ID

  • 24239359

Pubmed Central ID

  • PMC3893104

Electronic International Standard Serial Number (EISSN)

  • 2211-1247

Digital Object Identifier (DOI)

  • 10.1016/j.celrep.2013.10.020


  • eng

Conference Location

  • United States