Modeling pleomorphic rhabdomyosarcoma in mice

Here we used Ptch1+/-/ETV7TG/+/- mice with enhanced incidence of RMS to generate a model of pleomorphic RMS driven by haploinsufficiency of the lysosomal sialidase Neu1. These tumors share features and location with human RMS. We compared fourteen tumor samples taken from Neu1+/−/Ptch1+/−/ETV7 TG+/− (NPE) mice with seven tumor samples from Ptch1+/−/ETV7 TG+/− (PE) mice using the Mouse ClariomS array.