DICER1 and pleuropulmonary blastoma: A study led by one of the authors (DAH) identified germline loss-of-function DICER1 mutations affecting the RNase IIIb domain in affected families with pleuropulmonary blastoma (PPB)9, a rare dysembryonic lung malignancy of childhood which was not the only manifestation of this familial tumor predisposition syndrome; germline and somatic DICER1 mutations were subsequently identified in several other familial associated tumors in several extrapulmonary sites (Table 1).