This constrained proximity could also explain why the presumed germline variant in patient 2 leads to a phenotype similar to the one described in the two patients with GLOW syndrome associated with mosaic DICER1 mutation in the RNase IIIb domain, suggesting a genotype–phenotype relationship with missense mutations in RNase III. This evidence concerns the gene DROSHA and global developmental delay - lung cysts - overgrowth - Wilms tumor syndrome.