Low-grade appendiceal mucinous neoplasms are predominantly driven by KRAS- and GNAS-associated mucinous programs, whereas progression toward invasive adenocarcinoma more commonly involves additional alterations affecting tumor suppressor and differentiation pathways, including TP53 and TGF-β/SMAD signaling [47,51]. This evidence concerns the gene TGFB1 and mucinous neoplasm.