Cylindromatosis (CYLD) negatively regulates the NF-κB signaling pathway and it is identified to be a tumor suppressor involved in familial CYLD (Hayden and Ghosh, 2008; Harhaj and Dixit, 2012; Heideker and Wertz, 2015; Ohtake et al., 2016). This evidence concerns the gene NFKB1 and familial cylindromatosis.