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. This evidence concerns the gene DICER1 and neoplasm.