A recent study on a patient-derived pleuropulmonary blastoma model with DICER1 hotspot mutations found that it was greatly sensitive to RNA pol I inhibitors such as CX-5461 (pidnarulex) (93), in accordance with our proposed model of Dicer’s tumorigenicity being caused by its nucleolar function rather than miRNA dysregulation (94). Here, DICER1 is linked to blastoma.