Recently, it has been reported that hotspot mutations in the RNAse IIIb domain are associated with dysregulation of specific miRNAs, leading to activation of the PI3K/AKT/mTOR pathway, and GLOW syndrome in concurrence with DICER1 syndrome may benefit from the use of PI3K/AKT/mTOR inhibitors such as rapamycin and TORIN-1 [18]. Here, MTOR is linked to DICER1-related tumor predisposition.