The remaining DICER1-associated ERMS of our cohort showed multiple missense mutations or homozygous hotspot mutations in DICER1. Interestingly, the DICER1 locus (14q) in the latter neoplasms of our cohort did not show any copy number alterations, particularly no deletion/microdeletion. Here, DICER1 is linked to embryonal rhabdomyosarcoma.