Although this bi-allelic model is in line with the Knudson two-hit hypothesis indicating a bona fide tumor suppressor function, there are instances in for example cases of Wilms tumors in which DICER1 RNAse III mutations are seen without associated second hits (in terms of a trans mutation or loss of the remaining allele) [28]. The gene discussed is DICER1; the disease is Nephroblastoma.