EWSR1::BEND2 gene fusions characterize a majority of ACTH-producing neuroendocrine neoplasms of the pancreas: A follow-up study and brief review of the recent literature

Abstract ACTH-producing pancreatic neuroendocrine neoplasms (PanNENs) are a rare aggressive subset of panNETs associated with clinical symptoms of ectopic Cushing syndrome. Until recently, their molecular pathogenesis remained poorly understood. Following our prior study highlighting high frequency of gene fusions (mostly EWSR1::BEND2 ) in them, we herein document our experience with 5 new tumors occurring in 4 women (one of unspecified sex) aged 36 to 79 (median, 45). All presented with clinica