title: Increased incidence of extrapancreatic neoplasms in patients with IPMN: Fact or fiction? A critical systematic review.
abstract: BACKGROUND
  To identify potential associations between intraductal papillary mucinous neoplasm of the pancreas (IPMN) and extrapancreatic neoplasms (EPN), a systematic review of the literature has been performed.
  
  
  METHODS
  A systematic search of Medline/Pubmed was performed according to the PRISMA guidelines for reporting systematic reviews and meta-analysis for the following search terms: "extrapancreatic", "non pancreatic", "additional pancreatic", "additional primary" and alternatively matched with "neoplasms/tumours/cancers/malignancies/lesions". The results obtained specifically for IPMN were examined one by one by two independent investigators for further data selection and extraction.
  
  
  RESULTS
  Fifteen studies were identified to be suitable and included for systematic review. Fourteen reported an elevated risk for extrapancreatic malignancy, particularly gastric and colon cancer, while the largest and only prospective study did not find any association. Most studies were retrospective with a weak level of evidence that was not substantially enhanced even by a recent multicentre case series.
  
  
  CONCLUSIONS
  The available data on this clinically relevant question remain inconclusive. Due to lacking evidence on extrapancreatic neoplasms in IPMN patients, only a standard surveillance can be advised.
authors.affiliation: Unit of General Surgery 2, Department of Surgery, IRCCS Policlinico San Matteo, Pavia, Italy.
authors.forename: Luigi
authors.initials: L
authors.lastname: Pugliese
authors.affiliation: Division of Gastroenterology, Department of Internal Medicine, Ege University, Izmir, Turkey.
authors.forename: Muharrem
authors.initials: M
authors.lastname: Keskin
authors.affiliation: Division of Epidemiology and Statistics, European Institute of Oncology, Milan, Italy.
authors.forename: Patrick
authors.initials: P
authors.lastname: Maisonneuve
authors.affiliation: Department of Surgery, Klinikum rechts der Isar, Technische Universität München, Munich, Germany.
authors.forename: Jan G
authors.initials: JG
authors.lastname: D'Haese
authors.affiliation: Department of Surgery, Pancreas Institute, Verona University Hospital, Verona, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Marchegiani
authors.affiliation: Department of Gastroenterology, Klinikum rechts der Isar, Technische Universität München, Munich, Germany.
authors.forename: Patrick
authors.initials: P
authors.lastname: Wenzel
authors.affiliation: Division of Surgery, CLINTEC, Karolinska Institutet at Karolinska University Hospital, Stockholm, Sweden.
authors.forename: Marco
authors.initials: M
authors.lastname: Del Chiaro
authors.affiliation: Department of Surgery, Klinikum rechts der Isar, Technische Universität München, Munich, Germany. Electronic address: gueralp.ceyhan@tum.de.
authors.forename: Güralp O
authors.initials: GO
authors.lastname: Ceyhan
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000175:diagnosis / Q000453:epidemiology*; D021441:Carcinoma, Pancreatic Ductal / Q000175:diagnosis / Q000453:epidemiology*; D002291:Carcinoma, Papillary / Q000175:diagnosis / Q000453:epidemiology*; D014943:Global Health; D006801:Humans; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000453:epidemiology*; D010190:Pancreatic Neoplasms / Q000175:diagnosis / Q000453:epidemiology*; D011379:Prognosis
keywords: Benign neoplasms; Extrapancreatic; IPMN; Malignant neoplasms; Surveillance; Systematic