The finding of DICER1 syndrome-associated neoplasms such as PPB, cystic nephroma, SLCT, TFND and DTC in children or adolescents, cERMS, gynandroblastoma, and pituitary blastoma have a high specificity for germline alterations of the DICER1 gene or DICER1 syndrome. This evidence concerns the gene DICER1 and DICER1-related tumor predisposition.