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.
Department of Digestive Surgery, Hôpital Saint-Antoine, 184, rue du faubourg Saint-Antoine, 75012 Paris, France. denostquentin@hotmail.fr
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D002288:Adenocarcinoma, Mucinous / Q000235:genetics*; D021441:Carcinoma, Pancreatic Ductal / Q000235:genetics*; D006801:Humans; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000235:genetics*