title: Intraductal papillary-mucinous neoplasm of the pancreas associated with polycystic liver and kidney disease.
abstract: A case of intraductal papillary-mucinous neoplasm (IPMN) of the pancreas associated with polycystic liver and kidney disease is reported. A 63-year-old man was admitted to hospital with heartburn and upper abdominal pain. CT indicated multiple cysts in the liver and bilateral kidneys, and also showed multiple cystic lesions in the pancreatic head. Pancreatoduodenectomy was performed, and a pathological diagnosis of intraductal papillary-mucinous adenoma of branch duct type of the pancreas was made. He died of renal failure and hepatic failure due to recurrent cholangitis 12 years after the operation. An autopsy confirmed multiple cysts in the liver and kidneys. Multiple hepatic cysts were lined by biliary-type epithelium with no connection to the bile ducts. Peribiliary cysts were observed, and histology of the liver indicated von Meyenburg complexes. The kidneys showed that the cysts originated from all segments of the renal tubule. The autopsy findings suggested that the patient had suffered from autosomal-dominant polycystic kidney disease (ADPKD), but he did not have a family history of ADPKD. This case demonstrates that IPMN of the pancreas can occur as an extrarenal complication in patients with polycystic liver and kidney disease.
authors.affiliation: Department of Human Pathology, Kanazawa University Graduate School of Medicine, Kanazawa, Japan.
authors.forename: Yasunori
authors.initials: Y
authors.lastname: Sato
authors.forename: Munenori
authors.initials: M
authors.lastname: Mukai
authors.forename: Motoko
authors.initials: M
authors.lastname: Sasaki
authors.forename: Azusa
authors.initials: A
authors.lastname: Kitao
authors.forename: Norihide
authors.initials: N
authors.lastname: Yoneda
authors.forename: Daisuke
authors.initials: D
authors.lastname: Kobayashi
authors.forename: Yoshiaki
authors.initials: Y
authors.lastname: Imamura
authors.forename: Yasuni
authors.initials: Y
authors.lastname: Nakanuma
mesh_terms: D000236:Adenoma / Q000150:complications* / Q000473:pathology / Q000601:surgery; D017809:Fatal Outcome; D006801:Humans; D008107:Liver Diseases / Q000150:complications* / Q000473:pathology; D008297:Male; D008875:Middle Aged; D010190:Pancreatic Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery; D016577:Pancreaticoduodenectomy; D016891:Polycystic Kidney, Autosomal Dominant / Q000150:complications* / Q000473:pathology; D014057:Tomography, X-Ray Computed