Although low expression of DICER1 has been previously associated with worse prognosis in breast cancer [60] and ovarian tumors [61], the study of these nonepithelial ovarian tumors changed the paradigm as for the first time it was found that a hotspot genetic aberration in DICER1 can drive cancer through the combination of loss of one allele and a functionally deficient protein, this is an aberration of the classic two-hit hypothesis [59]. The gene discussed is DICER1; the disease is cancer.