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Extrahepatic Bile Duct Neuroendocrine Carcinoma

MedGen UID:
474773
•Concept ID:
C3273140
•
Neoplastic Process
Synonyms: Extrahepatic Bile Duct G3; Extrahepatic Bile Duct High Grade Neuroendocrine Carcinoma; Extrahepatic Bile Duct NEC; Extrahepatic Bile Duct Poorly Differentiated Endocrine Carcinoma

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the extrahepatic bile ducts. The mitotic count is more than 20 per 10 HPF. According to the size of the malignant cells, the prominence of the nucleoli, and the amount of cytoplasm, it is classified either as small or large cell neuroendocrine carcinoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrahepatic Bile Duct Neuroendocrine Carcinoma

Professional guidelines

PubMed

Clinicopathological Findings and Treatment Outcomes of Patients with Primary Hepatobiliary Neuroendocrine Neoplasms: A Retrospective Single-institution Analysis.
Komiyama S, Okusaka T, Maruki Y, Ohba A, Nagashio Y, Kondo S, Hijioka S, Morizane C, Ueno H, Sukeda A, Mizui T, Takamoto T, Nara S, Ban D, Esaki M, Hiraoka N, Shimada K
Intern Med 2024 Apr 1;63(7):891-901. Epub 2023 Aug 23 doi: 10.2169/internalmedicine.2016-23. PMID: 37612088Free PMC Article
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Ampullary Mixed Adenoneuroendocrine Carcinoma: Surprise Histology, Familiar Management.
Mahansaria SS, Agrawal N, Arora A, Bihari C, Appukuttan M, Chattopadhyay TK
Int J Surg Pathol 2017 Oct;25(7):585-591. Epub 2017 May 29 doi: 10.1177/1066896917712454. PMID: 28552015

Recent clinical studies

Etiology

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Comparison of Clinicopathological Features of Biliary Neuroendocrine Carcinoma with Adenocarcinoma.
Shiihara M, Higuchi R, Furukawa T, Yazawa T, Uemura S, Izumo W, Yamamoto M
Dig Surg 2021;38(1):30-37. Epub 2020 Jun 22 doi: 10.1159/000508443. PMID: 32570243
Neuroendocrine neoplasms of the extrahepatic bile duct: radiologic and clinical characteristics.
Hong N, Kim HJ, Byun JH, Kim SY, Kim KW, Kim JH, Hong SM
Abdom Imaging 2015 Jan;40(1):181-91. doi: 10.1007/s00261-014-0191-0. PMID: 25008023
Liver transplantation for hepatic tumors: a systematic review.
Ravaioli M, Ercolani G, Neri F, Cescon M, Stacchini G, Del Gaudio M, Cucchetti A, Pinna AD
World J Gastroenterol 2014 May 14;20(18):5345-52. doi: 10.3748/wjg.v20.i18.5345. PMID: 24833864Free PMC Article

Diagnosis

Neuroendocrine Carcinoma of the Extrahepatic Bile Ducts: A Case Report.
Jevdokimov D, Tauvena E, Jevdokimova N, Vilisova S, Kriviča O, Briede I, Tone T, Staka A, Gerina-Berzina A, Ozolins A, Pukitis A
Am J Case Rep 2023 Jun 21;24:e939239. doi: 10.12659/AJCR.939239. PMID: 37340629Free PMC Article
Small-cell Neuroendocrine Carcinoma of the Extrahepatic Bile Duct: A Rare Case Report.
Han B, Seo YJ, Oh GH, You GR, Ma KY, Kim KH, Noh MG, Joo YE
Korean J Gastroenterol 2023 Mar 25;81(3):121-124. doi: 10.4166/kjg.2023.005. PMID: 36960694
Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Neuroendocrine carcinoma diagnosis from bile duct cytological specimens: A retrospective single-center study.
Ishida M, Okano K, Sandoh K, Ito H, Ikeura T, Mitsuyama T, Miyoshi H, Shimatani M, Takaoka M, Okazaki K, Tsuta K
Diagn Cytopathol 2020 Feb;48(2):154-158. Epub 2019 Nov 7 doi: 10.1002/dc.24334. PMID: 31697402
Neuroendocrine carcinoma in the extrahepatic biliary tract: A case report and literature review.
Zhang L, Wan D, Bao L, Chen Q, Xie H, Xu S, Lin S
Medicine (Baltimore) 2018 Jul;97(29):e11487. doi: 10.1097/MD.0000000000011487. PMID: 30024526Free PMC Article

Therapy

Bronchobiliary fistula: a rare complication after pancreaticoduodenectomy.
Odufalu FD, Zubairu J, Silverman W
BMJ Case Rep 2018 Sep 4;2018 doi: 10.1136/bcr-2017-221895. PMID: 30181397Free PMC Article
A case of neuroendocrine carcinoma in the hepatic hilar lymph nodes concomitant with an adenocarcinoma of the gallbladder.
Okada H, Uchida Y, Matsuzaki N, Goto T, Nishimura S, Kurita A, Nishimura T, Yazumi S, Terajima H
World J Surg Oncol 2016 Nov 14;14(1):284. doi: 10.1186/s12957-016-1039-6. PMID: 27842605Free PMC Article
Liver transplantation for hepatic tumors: a systematic review.
Ravaioli M, Ercolani G, Neri F, Cescon M, Stacchini G, Del Gaudio M, Cucchetti A, Pinna AD
World J Gastroenterol 2014 May 14;20(18):5345-52. doi: 10.3748/wjg.v20.i18.5345. PMID: 24833864Free PMC Article
Surgical technique and results of total mesopancreas excision (TMpE) in pancreatic tumors.
Adham M, Singhirunnusorn J
Eur J Surg Oncol 2012 Apr;38(4):340-5. Epub 2012 Jan 20 doi: 10.1016/j.ejso.2011.12.015. PMID: 22264964
Interventional endoscopy, neoadjuvant therapy and the gastroenterologist.
Snady H
Hematol Oncol Clin North Am 2002 Feb;16(1):53-79. doi: 10.1016/s0889-8588(01)00010-7. PMID: 12063829

Prognosis

Management of Primary Hepatopancreatobiliary and Ampulla Large Cell Neuroendocrine Carcinoma.
Zhu J, Xiao W, Li Y
J Laparoendosc Adv Surg Tech A 2022 Jun;32(6):639-645. Epub 2021 Oct 11 doi: 10.1089/lap.2021.0482. PMID: 34637632
Neuroendocrine carcinoma of the common bile duct associated with congenital bile duct dilatation: a case report.
Kiya Y, Nagakawa Y, Takishita C, Osakabe H, Nishino H, Akashi M, Yamaguchi H, Nagao T, Oono R, Katsumata K, Tsuchida A
BMC Gastroenterol 2021 Jun 12;21(1):257. doi: 10.1186/s12876-021-01777-7. PMID: 34118881Free PMC Article
Comparison of Clinicopathological Features of Biliary Neuroendocrine Carcinoma with Adenocarcinoma.
Shiihara M, Higuchi R, Furukawa T, Yazawa T, Uemura S, Izumo W, Yamamoto M
Dig Surg 2021;38(1):30-37. Epub 2020 Jun 22 doi: 10.1159/000508443. PMID: 32570243
Neuroendocrine carcinoma in the extrahepatic biliary tract: A case report and literature review.
Zhang L, Wan D, Bao L, Chen Q, Xie H, Xu S, Lin S
Medicine (Baltimore) 2018 Jul;97(29):e11487. doi: 10.1097/MD.0000000000011487. PMID: 30024526Free PMC Article
Neuroendocrine carcinoma of the extrahepatic bile duct: A case report.
Oshiro Y, Gen R, Hashimoto S, Oda T, Sato T, Ohkohchi N
World J Gastroenterol 2016 Aug 14;22(30):6960-4. doi: 10.3748/wjg.v22.i30.6960. PMID: 27570432Free PMC Article

Clinical prediction guides

Microsatellite instability in pancreatic and ampullary carcinomas: histology, molecular pathology, and clinical implications.
Luchini C, Scarpa A
Hum Pathol 2023 Feb;132:176-182. Epub 2022 Jun 14 doi: 10.1016/j.humpath.2022.06.009. PMID: 35714836
Hepatoid carcinoma and related entities of the extrahepatic bile duct: A clinicopathological study of four cases.
Okura K, Esaki M, Nara S, Ban D, Takamoto T, Shimada K, Hiraoka N
Pathol Int 2022 Jun;72(6):332-342. Epub 2022 Apr 26 doi: 10.1111/pin.13226. PMID: 35472251
Neuroendocrine carcinoma of the common bile duct associated with congenital bile duct dilatation: a case report.
Kiya Y, Nagakawa Y, Takishita C, Osakabe H, Nishino H, Akashi M, Yamaguchi H, Nagao T, Oono R, Katsumata K, Tsuchida A
BMC Gastroenterol 2021 Jun 12;21(1):257. doi: 10.1186/s12876-021-01777-7. PMID: 34118881Free PMC Article
Neuroendocrine carcinoma in the extrahepatic biliary tract: A case report and literature review.
Zhang L, Wan D, Bao L, Chen Q, Xie H, Xu S, Lin S
Medicine (Baltimore) 2018 Jul;97(29):e11487. doi: 10.1097/MD.0000000000011487. PMID: 30024526Free PMC Article
Neuroendocrine neoplasms of the extrahepatic bile duct: radiologic and clinical characteristics.
Hong N, Kim HJ, Byun JH, Kim SY, Kim KW, Kim JH, Hong SM
Abdom Imaging 2015 Jan;40(1):181-91. doi: 10.1007/s00261-014-0191-0. PMID: 25008023

Recent systematic reviews

Management and outcomes of mixed adenoneuroendocrine carcinoma of the ampulla of Vater: A systematic review and pooled analysis of 56 patients.
Ziogas IA, Rallis KS, Tasoudis PT, Moris D, Schulick RD, Del Chiaro M
Eur J Surg Oncol 2023 Apr;49(4):682-687. Epub 2023 Jan 9 doi: 10.1016/j.ejso.2023.01.005. PMID: 36646615
Somatostatinoma of the Ampulla of Vater: A Systematic Review.
Giannakodimos I, Giannakodimos A, Ziogou A, Frountzas M, Kritikos N, Vlachos K, Toutouzas KG, Schizas D
J Gastrointestin Liver Dis 2022 Dec 17;31(4):459-466. doi: 10.15403/jgld-4383. PMID: 36535044
Liver transplantation for hepatic tumors: a systematic review.
Ravaioli M, Ercolani G, Neri F, Cescon M, Stacchini G, Del Gaudio M, Cucchetti A, Pinna AD
World J Gastroenterol 2014 May 14;20(18):5345-52. doi: 10.3748/wjg.v20.i18.5345. PMID: 24833864Free PMC Article

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