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Summary  The scenario of paediatric acute myeloid leukaemia ( AML ), particularly non‐Down syndrome acute megakaryoblastic leukaemia (non‐ DS ‐ AMKL ), has been recently revolutionized by the advent of large‐scale, genomic sequencing technologies. In this changing landscape, a significantly relevant discovery has been represented by the identification of the   CBFA 2T3 ‐  GLIS 2  fusion gene, which is the result of a cryptic inversion of chromosome 16. It is the most frequent chimeric oncogene identified to date in non‐ DS ‐ AMKL , although it seems not to be exclusively restricted to the French‐American‐British M7 subgroup. The   CBFA 2T3 ‐  GLIS 2  fusion gene characterizes a subtype of leukaemia that is specific to paediatrics, having never been identified in adults. It characterizes an extremely aggressive leukaemia, as the presence of this fusion is associated with a grim outcome in almost all of the case series reported, with overall survival rates ranging between 15% and 30%. Although the molecular basis that underlies this leukaemia subtype is still far from being completely elucidated, unique functional properties induced by   CBFA 2T3‐ GLIS 2  in the leukaemogenesis driving process have been recently identified. We here review the peculiarities of   CBFA 2T3 ‐  GLIS 2 ‐positive  AML , describing its intriguing clinical and biological behaviour and providing some challenging targeting opportunities.

CBFA2T3‐GLIS2‐positive acute myeloid leukaemia. A peculiar paediatric entity