In sporadic (i.e. non-familial) PNETs, inactivating mutations in MEN1, alpha thalassemia/mental retardation syndrome x-linked (ATRX) chromatin remodeller (ATRX), and death domain-associated protein (DAXX) are the most common, occurring in up to 40% of PNETs, and are involved in epigenetic regulation (Jiao et al. 2011, Thakker 2014, Chan et al. 2018) and inhibition of proliferative pathways (Jiao et al. 2011, Chamberlain et al. 2014). The gene discussed is DAXX; the disease is alpha thalassemia spectrum.