Although DROSHA/DGCR8/DICER1 mutations appear to be infrequent in adult tumors, a recent pan-cancer analysis using The Cancer Genome Atlas and MSK-IMPACT databases reported enrichment of hotspot mutations in DICER1 RNase IIIb and RNase IIIa domains in uterine cancers, including endometrial cancer and uterine sarcoma (63), (64). Here, DICER1 is linked to uterine corpus sarcoma.