New insights into the origin and the genetic basis of rhabdomyosarcomas

Abstract

In this issue of Cancer Cell, Rubin et al. (2011) describe using various conditional mouse models to trace the developmental origin and genetic basis of rhabdomyosarcomas. Their work provides a genetic dissection underlying rhabdomyosarcomas development and unveils unexpected relationship between various soft-tissue tumor types.

Figure 1. The Developmental Origin and Mutational Profile of the Tumor Determine the Proportions of RMS

Different subpopulations of myogenic precursor cells give rise to RMS. While p53 deletion and Ptch1 haploinsufficiency are important players in cellular transformation and myodifferentiation potential of the resulting tumor, Rb1 deletion acts as a modifier of tumor phenotype in that context. Tumors (i.e., eRMSs) arising from different cells of origin exhibit the same gene expression profile as that of the activated muscle satellite cells.