Pancreatic mucinous cystic tumor in Turner syndrome: How a tumor bends to a genetic disease

INTRODUCTIONMucinous cystic neoplasms (MCN) are uncommon tumors of the pancreatic corpus/tail occurring mostly in middle-aged women, with a variable clinico-biological behavior. On histology, MCNs concurrently show an epithelial mucosecreting component with ovarian-type stromal cells.PRESENTATION OF CASEThis report describes the first case of a pancreatic MCN with no ovarian-type stroma in a patient with Turner syndrome (TS).DISCUSSIONThe mesenchymal component of MCN presumably results from the intra-pancreatic entrapment of ovarian stroma during embryogenesis. In our case, the absence of such stromal component may relate to the “dysgenetic” changes in the ovary involved in TS.CONCLUSIONThe present case of primary pancreatic MCN arising in a TS-patient triggers some original speculation on the morphogenesis of pancreatic MCN, also expanding the current clinico-pathological knowledge of this extremely rare entity.