There are >20 genes described to be mutated inNETs, with the most common being menin 1 (MEN1),death-domain-associated protein (DAXX) and ATRX chromatin remodeler(ATRX), which occur in 40–70% of sporadic G1–G3pancreatic NETs (PanNETs), and cyclin-dependent kinase inhibitor 1B(CDKN1B), which occurs in approximately 10% of sporadicG1–G3 small intestinal NETs (siNETs) (Jiaoet al. 2011, Francis et al. 2013, Di Domenico et al. 2017, Scarpa et al. 2017, van Riet et al. 2021). Here, DAXX is linked to small intestine neuroendocrine neoplasm.