title: Intraductal papillary mucinous neoplasms of the pancreas with concurrent pancreatic and periampullary neoplasms.
abstract: BACKGROUND
  Intraductal papillary mucinous neoplasms (IPMN) have been reported to be associated with concurrent, distinct pancreatic ductal adenocarcinoma (con-PDAC) in about 8% (range, 4-10%) of resected branch duct (BD) lesions. In addition, other pancreatic and ampullary tumors are occasionally diagnosed with IPMN in patients undergoing pancreatic surgery. The objective of this study is to describe the prevalence, clinicopathologic characteristics and prognosis of IPMN with concurrent pancreatic and ampullary neoplasms, especially con-PDAC.
  
  
  METHODS
  The combined databases of pancreatic resections from the Massachusetts General Hospital and the Negrar Hospital, Italy, were analyzed for patients who had been diagnosed with IPMN and concurrent pancreatic or ampullary neoplasms.
  
  
  RESULTS
  2762 patients underwent pancreatic surgery from January 2000 to December 2012. Sixteen percent (n = 441) had pathologically confirmed IPMN and 11% of these (n = 50) had a different distinct synchronous pancreatic neoplasm. The majority of these, 62%, were con-PDAC, followed by neuroendocrine neoplasms (10%) and ampullary carcinoma (10%). Less frequently, mucinous (6%) as well as serous cystic neoplasms (6%), adenosquamous carcinoma (4%) and distal bile duct cancer (2%) were diagnosed. Among all patients with synchronous neoplasms, 66% harbored BD-IPMN, 28% combined IPMN and 6% main duct IPMN. Abdominal pain and/or jaundice were the leading symptoms in half of patients.
  
  
  CONCLUSIONS
  IPMN, mainly BD-IPMN, are associated with con-PDAC in about 7% of patients and account for 62% of all concurrent pancreatic/ampullary neoplasms. Other synchronous neoplasms may be found sporadically with IPMN without a suspected association.
authors.affiliation: Department of Surgery, Massachusetts General Hospital, Boston, USA; Department of Pathology, Massachusetts General Hospital, Boston, USA.
authors.forename: K
authors.initials: K
authors.lastname: Sahora
authors.affiliation: Department of Surgery, Ospedale Sacro Cuore Don Calabria, Negrar (VR), Italy; Department of Pathology, Ospedale Sacro Cuore Don Calabria, Negrar (VR), Italy.
authors.forename: S
authors.initials: S
authors.lastname: Crippa
authors.affiliation: Department of Pathology, Ospedale Sacro Cuore Don Calabria, Negrar (VR), Italy.
authors.forename: G
authors.initials: G
authors.lastname: Zamboni
authors.affiliation: Department of Surgery, Massachusetts General Hospital, Boston, USA; Department of Pathology, Massachusetts General Hospital, Boston, USA.
authors.forename: C
authors.initials: C
authors.lastname: Ferrone
authors.affiliation: Department of Surgery, Massachusetts General Hospital, Boston, USA; Department of Pathology, Massachusetts General Hospital, Boston, USA.
authors.forename: A L
authors.initials: AL
authors.lastname: Warshaw
authors.affiliation: Department of Surgery, Massachusetts General Hospital, Boston, USA; Department of Pathology, Massachusetts General Hospital, Boston, USA.
authors.forename: K
authors.initials: K
authors.lastname: Lillemoe
authors.affiliation: Department of Pathology, Massachusetts General Hospital, Boston, USA.
authors.forename: M
authors.initials: M
authors.lastname: Mino-Kenudson
authors.affiliation: Department of Surgery, Ospedale Sacro Cuore Don Calabria, Negrar (VR), Italy; Department of Pathology, Ospedale Sacro Cuore Don Calabria, Negrar (VR), Italy.
authors.forename: M
authors.initials: M
authors.lastname: Falconi
authors.affiliation: Department of Surgery, Massachusetts General Hospital, Boston, USA; Department of Pathology, Massachusetts General Hospital, Boston, USA. Electronic address: cfernandez@partners.org.
authors.forename: C
authors.initials: C
authors.lastname: Fernandez-del Castillo
mesh_terms: D015746:Abdominal Pain / Q000209:etiology; D002288:Adenocarcinoma, Mucinous / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D000368:Aged; D000369:Aged, 80 and over; D014670:Ampulla of Vater; D018196:Carcinoma, Adenosquamous / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D021441:Carcinoma, Pancreatic Ductal / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D017024:Chemotherapy, Adjuvant; D003138:Common Bile Duct Neoplasms / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D033162:Incidental Findings; D007565:Jaundice / Q000209:etiology; D008297:Male; D008875:Middle Aged; D009378:Neoplasms, Multiple Primary / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D018358:Neuroendocrine Tumors / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000453:epidemiology / Q000473:pathology* / Q000601:surgery; D015995:Prevalence; D011379:Prognosis; D015996:Survival Rate
keywords: Concurrent neoplasm; Intraductal papillary mucinous noeplasm; Pancreas; Pancreatic cancer