title: Familial intraductal papillary mucinous neoplasms of the pancreas.
abstract: The prevalence of intraductal papillary mucinous neoplasms in patients with a high risk of pancreatic adenocarcinoma was estimated to be 15%. However, a familial form of intraductal papillary mucinous neoplasms was never described.
  
  
  METHODS
  Three families (8 patients) with intraductal papillary mucinous neoplasms familial forms were described. Diagnosis was made according to radiological criteria and was confirmed by pathological data. Genetic predisposing factors of pancreatic cancer were searched for.
  
  
  RESULTS
  Symptoms related to intraductal papillary mucinous neoplasms were recurrent acute pancreatitis (n=3) or fortuitous discovery (n=5). Number of cystic lesions was ≤3 (n=4) or >3 (n=4). Intraductal papillary mucinous neoplasms involved branch ducts (n=7) or both main pancreatic duct and branch duct (n=1). Severe and moderate dysplasia was found on surgical specimens. No genetic alteration was found (BRCA2, p16 or CDKN2A genes).
  
  
  CONCLUSIONS
  A familial form of intraductal papillary mucinous neoplasms was found in three families. No pancreatic cancer was found in relatives but an attentive survey has to be proposed.
authors.affiliation: Pôle des Maladies de l'Appareil Digestif, Service de Gastroentérologie - Pancréatologie, Hôpital Beaujon, APHP, Université Denis Diderot-Paris VII, 100, boulevard du général Leclerc, 92118 Clichy, France. vinciane.rebours@bjn.aphp.fr
authors.forename: Vinciane
authors.initials: V
authors.lastname: Rebours
authors.forename: Anne
authors.initials: A
authors.lastname: Couvelard
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Peyroux
authors.forename: Alain
authors.initials: A
authors.lastname: Sauvanet
authors.forename: Pascal
authors.initials: P
authors.lastname: Hammel
authors.forename: Philippe
authors.initials: P
authors.lastname: Ruszniewski
authors.forename: Philippe
authors.initials: P
authors.lastname: Lévy
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D000231:Adenocarcinoma, Papillary / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D000328:Adult; D000368:Aged; D021441:Carcinoma, Pancreatic Ductal / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D015331:Cohort Studies; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D010195:Pancreatitis / Q000209:etiology; D010375:Pedigree