title: Well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst.
abstract: We encountered a rare case of a well-differentiated endocrine carcinoma originating from the bile duct in association with a congenital choledochal cyst (CCC). The patient is a 28-year-old woman referred to our clinic for pruritus. Laboratory data showed mild elevation of serum hepatobiliary enzymes. Computed tomography and magnetic resonance imaging demonstrated pancreatobiliary maljunction and a Todani type IV-A CCC from the inferior bile duct to the bilateral intrahepatic bile ducts. A solid tumor was detected in the middle portion of the common bile duct. Pancreatoduodenectomy and total extrahepatic bile duct resection was performed. Based on pathologic and immunohistochemical examinations, a diagnosis of well-differentiated endocrine carcinoma was made according to the World Health Organization criteria. To our knowledge, this is the third report of a neuroendocrine tumor originating from the bile duct in association with a CCC.
authors.affiliation: Organ Transplantation Gastroenterological and Hepatobiliary Surgery, Division of Clinical Medicine, Faculty of Medicine, Graduate School of University of Tsukuba, Tsukuba, Japan.
authors.forename: Kazuhiro
authors.initials: K
authors.lastname: Takahashi
authors.forename: Ryoko
authors.initials: R
authors.lastname: Sasaki
authors.forename: Yukio
authors.initials: Y
authors.lastname: Oshiro
authors.forename: Kiyoshi
authors.initials: K
authors.lastname: Fukunaga
authors.forename: Tatsuya
authors.initials: T
authors.lastname: Oda
authors.forename: Nobuhiro
authors.initials: N
authors.lastname: Ohkohchi
mesh_terms: D000328:Adult; D015529:Choledochal Cyst / Q000150:complications*; D003138:Common Bile Duct Neoplasms / Q000150:complications / Q000175:diagnosis*; D005260:Female; D006801:Humans; D018358:Neuroendocrine Tumors / Q000150:complications / Q000175:diagnosis*