DICER1 and DICER1-related tumor predisposition: Most of the reported somatic mutations are concentrated to the RNase IIIb region (exon 24 and 25), which is in contrast to the germline DICER1 gene mutations that are found spread out across the gene, resulting in a phenotype predisposed for tumours such as pleuropulmonary blastoma, Sertoli-Leydig cell tumour, multinodular goitre and thyroid cancer, collectively called DICER1 syndrome (Hill et al. 2009, Slade et al. 2011, Foulkes et al. 2014).