GNAS and pachyonychia congenita: Notably, recent researches highlighted that the IPMN oncogenetic process is regulated by two different pathways: the first, linked to GNAS mutations, involves intestinal IPMN progressing to colloid adenocarcinomas (a PC variant reach in extracellular mucin), and the second, driven by KRAS mutations, is typical of pancreatobiliary IPMN and leads to conventional PC [20,21].