DICER1 and DICER1-related tumor predisposition: A body of evidence has indicated that deleterious germline mutations in the DICER1 gene are responsible for DICER1 syndrome, an inherited disorder characterized by an increased frequency of various types of malignant and benign tumors that occur predominantly in infants and young children, the most common and most characteristic of which is pleuropulmonary blastoma (54,55).