Within the past decade, as whole-genome and exome sequencing techniques emerged, ATRX and DAXX mutations were reported in a variety of human tumours, namely various tumours of the central nervous system (CNS) (17% and < 1%, respectively) [13,14,15,16] and pancreatic neuroendocrine tumours (PanNETs) (10% and 20%, respectively) [17,18,19,20]. This evidence concerns the gene ATRX and neoplasm.