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

INTRODUCTION: Mucinous 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 CASE: This report describes the first case of a pancreatic MCN with no ovarian-type stroma in a patient with Turner syndrome (TS). DISCUSSION: The 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. CONCLUSION: The 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.