Hereditary intraductal papillary mucinous neoplasm of the pancreas

Intraductal papillary mucinous neoplasm (IPMN) is a rare pancreatic tumor defined as intraductal mucin-producting neoplasm with tall, columnar, mucin-containing epithelium. IPMN have already been described in association with inherited genetic disorder including familial adenomatous polyposis and Peutz-Jeghers syndrome. However, there is no reported description of familial history of IPMN. We reported in this case-report IPMN in the first-degree relatives without familial history of colorectal polyposis or previous extra-pancreatic cancer. The rarety of IPMN suggests that the coexistence of this tumor in two first-degree relatives is probably due to a genetic inherited factor that remains to be elucidated.