Recent revelations of hotspot tumors linked to DICER1 in both PPB and PPB-FTDS patients who possess hereditary DICER1 mutations offer compelling support for the two-hit theory of cancer development (Heravi-Moussavi et al. 2012; Wu et al. 2013; Tomiak et al. 2014; Kock et al. 2013; Pugh et al. 2014). Here, DICER1 is linked to cancer.