title: Intraductal papillary mucinous carcinoma of the pancreas associated with pancreas divisum: a case report and review of the literature.
abstract: BACKGROUND
  Pancreas divisum, the most common congenital anomaly of the pancreas, is caused by failure of the fusion of the ventral and dorsal pancreatic duct systems during embryological development. Although various pancreatic tumors can occur in patients with pancreas divisum, intraductal papillary mucinous neoplasm is rare.
  
  
  METHODS
  A 77-year-old woman was referred to our hospital because she was incidentally found to have a cystic tumor in her pancreas at a regular health checkup. Contrast-enhanced abdominal computed tomography images demonstrated a cystic tumor in the head of the pancreas measuring 40 mm in diameter with slightly enhancing mural nodules within the cyst. Endoscopic retrograde pancreatography via the major duodenal papilla revealed a cystic tumor and a slightly dilated main pancreatic duct with an abrupt interruption at the head of the pancreas. The orifice of the major duodenal papilla was remarkably dilated and filled with an abundant extrusion of mucin, and the diagnosis based on pancreatic juice cytology was "highly suspicious for adenocarcinoma". Magnetic resonance cholangiopancreatography depicted a normal, non-dilated dorsal pancreatic duct throughout the pancreas. The patient underwent a pylorus-preserving pancreaticoduodenectomy under the diagnosis of intraductal papillary mucinous neoplasm with suspicion of malignancy arising in the ventral part of the pancreas divisum. A pancreatography via the major and minor duodenal papillae on the surgical specimen revealed that the ventral and dorsal pancreatic ducts were not connected, and the tumor originated in the ventral duct, i.e., the Wirsung's duct. Microscopically, the tumor was diagnosed as intraductal papillary mucinous carcinoma with microinvasion. In addition, marked fibrosis with acinar cell depletion was evident in the ventral pancreas, whereas no fibrotic change was noted in the dorsal pancreas.
  
  
  CONCLUSIONS
  Invasive ductal carcinomas of the pancreas associated with pancreas divisum usually arise from the dorsal pancreas, in which the occurrence of pancreatic cancer may link to underlying longstanding chronic pancreatitis in the dorsal pancreas; however, the histopathogenesis of intraductal papillary mucinous neoplasm in this anomaly is a critical issue that warrants further investigation in future.
authors.affiliation: Deparment of Surgery, Matsue Red Cross Hospital, 200 Horo-machi, Matsue, Shimane, 690-8506, Japan. nishiken1027@gmail.com.|Department of Digestive and General Surgery, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. nishiken1027@gmail.com.
authors.forename: Takeshi
authors.initials: T
authors.lastname: Nishi
authors.affiliation: Department of Digestive and General Surgery, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. batayan5@med.shimane-u.ac.jp.
authors.forename: Yasunari
authors.initials: Y
authors.lastname: Kawabata
authors.affiliation: Department of Organ Pathology, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. kanatomo@med.shimane-u.ac.jp.
authors.forename: Noriyoshi
authors.initials: N
authors.lastname: Ishikawa
authors.affiliation: Department of Organ Pathology, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. asuka@med.shimane-u.ac.jp.
authors.forename: Asuka
authors.initials: A
authors.lastname: Araki
authors.affiliation: Department of Digestive and General Surgery, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. yano@med.shimane-u.ac.jp.
authors.forename: Seiji
authors.initials: S
authors.lastname: Yano
authors.affiliation: Department of Organ Pathology, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. hm5995@med.shimane-u.ac.jp.
authors.forename: Riruke
authors.initials: R
authors.lastname: Maruyama
authors.affiliation: Department of Digestive and General Surgery, Shimane University Faculty of Medicine, 89-1 Enyacho, Izumo, Shimane, 693-8501, Japan. ytajima@med.shimane-u.ac.jp.
authors.forename: Yoshitsugu
authors.initials: Y
authors.lastname: Tajima
mesh_terms: D002288:Adenocarcinoma, Mucinous / Q000473:pathology* / Q000601:surgery; D000368:Aged; D021441:Carcinoma, Pancreatic Ductal / Q000473:pathology* / Q000601:surgery; D002291:Carcinoma, Papillary / Q000473:pathology* / Q000601:surgery; D005260:Female; D006801:Humans; D010179:Pancreas / Q000002:abnormalities* / Q000473:pathology* / Q000601:surgery; D010190:Pancreatic Neoplasms / Q000473:pathology* / Q000601:surgery; D016577:Pancreaticoduodenectomy