title: Intraductal papillary neoplasm of the bile duct accompanying biliary mixed adenoneuroendocrine carcinoma.
abstract: We present the first case of an intraductal papillary neoplasm of the bile duct (IPNB) accompanying a mixed adenoneuroendocrine carcinoma (MANEC). A 74-year-old woman presented with fever of unknown cause. Laboratory data revealed jaundice and liver injury. Contrast-enhanced computed tomography revealed a 20 mm polypoid tumor in the dilated distal bile duct, which exhibited early enhancement and papillary growth. Upper gastrointestinal endoscopy revealed mucus production from the papilla of Vater, characterized by its protruding and dilated orifice. Endoscopic ultrasonography visualized the polypoid tumor in the distal bile duct, but no invasive region was suggested by diagnostic imaging. Therefore, the initial diagnosis was IPNB. After endoscopic nasobiliary drainage, a pylorus-preserving pancreaticoduodenectomy was performed. Pathological examination of the resected bile duct revealed papillary proliferation of biliary-type cells with nuclear atypia, indicating pancreaticobiliary-type IPNB. In addition, solid portions comprised of tumor cells with characteristic salt-and-pepper nuclei were evident. Immunohistochemistry revealed expression of the neuroendocrine marker synaptophysin in this solid component, diagnosing it as a neuroendocrine tumor (NET). Furthermore, the MIB-1 proliferation index of NET was higher than that of IPNB, and microinvasion of the NET component was found, indicating neuroendocrine carcinoma (NET G3). This unique case of MANEC, comprising IPNB and NET, provides insight into the pathogenesis of biliary NET.
authors.affiliation: Department of Gastroenterologic Surgery, Graduate School of Medical Science, Kanazawa University, Kanazawa 920-8641, Japan. ionishi@kinbyou.hosp.go.jp
authors.forename: Ichiro
authors.initials: I
authors.lastname: Onishi
authors.forename: Hirohisa
authors.initials: H
authors.lastname: Kitagawa
authors.forename: Kenichi
authors.initials: K
authors.lastname: Harada
authors.forename: Syogo
authors.initials: S
authors.lastname: Maruzen
authors.forename: Seisyo
authors.initials: S
authors.lastname: Sakai
authors.forename: Isamu
authors.initials: I
authors.lastname: Makino
authors.forename: Hironori
authors.initials: H
authors.lastname: Hayashi
authors.forename: Hisatoshi
authors.initials: H
authors.lastname: Nakagawara
authors.forename: Hidehiro
authors.initials: H
authors.lastname: Tajima
authors.forename: Hiroyuki
authors.initials: H
authors.lastname: Takamura
authors.forename: Takashi
authors.initials: T
authors.lastname: Tani
authors.forename: Masato
authors.initials: M
authors.lastname: Kayahara
authors.forename: Hiroko
authors.initials: H
authors.lastname: Ikeda
authors.forename: Tetsuo
authors.initials: T
authors.lastname: Ohta
authors.forename: Yasuni
authors.initials: Y
authors.lastname: Nakanuma
mesh_terms: D000230:Adenocarcinoma / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D000368:Aged; D001650:Bile Duct Neoplasms / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D014408:Biomarkers, Tumor / Q000032:analysis; D001706:Biopsy; D018278:Carcinoma, Neuroendocrine / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D002291:Carcinoma, Papillary / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D004322:Drainage; D016099:Endoscopy, Gastrointestinal; D019160:Endosonography; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D018193:Neoplasms, Complex and Mixed / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D009378:Neoplasms, Multiple Primary / Q000737:chemistry / Q000473:pathology* / Q000601:surgery; D016577:Pancreaticoduodenectomy; D011237:Predictive Value of Tests; D014057:Tomography, X-Ray Computed
chemical_list: D014408:Biomarkers, Tumor
keywords: Bile duct; Intraductal papillary neoplasm of bile duct; Intraductal papillary neoplasm of the bile duct; Neuroendocrine tumor