ERMSs arising elsewhere in the genitourinary tract have been associated with DICER1 syndrome; three cases with germline loss-of-function mutations have been described in the urinary bladder70, one case in the fallopian tube with a germline DICER1 mutation, two cases in the ovary with somatic DICER1 mutations72, and another case in the ovary with a germline DICER1 mutation in exon 8 and a somatic mutation73. This evidence concerns the gene DICER1 and DICER1-related tumor predisposition.