Dicer1 Syndrome, also known as pleuro-pulmonary blastoma familial tumor susceptibility syndrome, is related to pleuropulmonary blastoma and tumor predisposition syndrome 1. An important gene associated with Dicer1 Syndrome is DICER1 (Dicer 1, Ribonuclease III), and among its related pathways/superpathways are Fragile X syndrome and Primary ovarian insufficiency. The drugs Vincristine and Dactinomycin have been mentioned in the context of this disorder. Affiliated tissues include thyroid and lung, and related phenotypes are Increased proliferation and Decreased viability in pancreas lineage