The DICER1 variant D1709N locates to the functionally important RNAse IIIb domain of the mature DICER1 protein, and this specific variant is reported as a somatic mutation in several DICER1 syndrome-associated tumor forms, including ovarian Sertoli-Leydig cell tumors, pleuropulmonary blastoma and single cases of pediatric thyroid cancer [19–22]. The gene discussed is DICER1; the disease is Ovarian Sertoli-Leydig Cell Tumor.