A recent report of biallelic DICER1 mutations in an ovarian fibrosarcoma from a 9-year-old patient demonstrated a germline single base insertion in the DICER1 gene, causing a frameshift and premature stop codon as well as a second point mutation within the tumor that resulted in a substitution at amino acid position 1813 within the RNase IIIb domain (p.E1813G) [94]. Here, DICER1 is linked to neoplasm.