DICER1 and Sertoli-Leydig cell tumor: After more than three decades of this seminal observation16, numerous studies have confirmed the relationship between DICER1 variants in carriers and the development of a range of neoplasms and non-neoplastic conditions; these associations have served to clarify the molecular genetic nature of previously known pathologic entities such as cystic nephroma and Sertoli-Leydig cell tumor, but also to identify and characterize entities which were included among other apparently unrelated neoplastic processes or not recognized as familial associated pathology18.