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Extrahepatic bile duct small cell adenocarcinoma

MedGen UID:
277648
•Concept ID:
C1335979
•
Neoplastic Process
Synonyms: extrahepatic bile duct small cell adenocarcinoma; Extrahepatic Bile Duct Small Cell Carcinoma; Extrahepatic Bile Duct Small Cell NEC; extrahepatic bile duct small cell NEC; Extrahepatic Bile Duct Small Cell Neuroendocrine Carcinoma; extrahepatic bile duct small cell neuroendocrine carcinoma; Oat cell carcinoma of extrahepatic bile duct; Oat cell carcinoma of the extrahepatic bile duct; Oat cell extrahepatic bile duct carcinoma; Small Cell Adenocarcinoma of Extrahepatic Bile Duct; small cell adenocarcinoma of extrahepatic bile duct; Small Cell Adenocarcinoma of the Extrahepatic Bile Duct; small cell adenocarcinoma of the extrahepatic bile duct; Small Cell Extrahepatic Bile Duct Carcinoma; small cell extrahepatic bile duct carcinoma; Small Cell Extrahepatic Bile Duct Neuroendocrine Carcinoma; small cell extrahepatic bile duct neuroendocrine carcinoma
 
Monarch Initiative: MONDO:0003708

Definition

An aggressive, high-grade and poorly differentiated carcinoma with neuroendocrine differentiation that arises from the extrahepatic bile ducts. It is characterized by the presence of malignant small cells. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVExtrahepatic bile duct small cell adenocarcinoma

Professional guidelines

PubMed

Nestin may be a candidate marker for differential diagnosis between small duct type and large duct type intrahepatic cholangiocarcinomas.
Sasaki M, Sato Y, Nakanuma Y
Pathol Res Pract 2024 Jan;253:155061. Epub 2023 Dec 22 doi: 10.1016/j.prp.2023.155061. PMID: 38154357
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
CD56 expression aids in the differential diagnosis of cholangiocarcinomas and benign cholangiocellular lesions.
Gütgemann I, Haas S, Berg JP, Zhou H, Büttner R, Fischer HP
Virchows Arch 2006 Apr;448(4):407-11. Epub 2006 Jan 13 doi: 10.1007/s00428-005-0145-6. PMID: 16411132

Recent clinical studies

Etiology

A Novel Approach to Quantify Heterogeneity of Intrahepatic Cholangiocarcinoma: The Hidden-Genome Classifier.
Song Y, Boerner T, Drill E, Shin P, Kumar S, Sigel C, Cercek A, Kemeny N, Abou-Alfa G, Iacobuzio-Donahue C, Cowzer D, Schultz N, Walch H, Balachandran V, Groot Koerkamp B, Kingham P, Soares K, Wei A, D'Angelica M, Drebin J, Chandwani R, Harding JJ, Jarnagin W
Clin Cancer Res 2024 Aug 15;30(16):3499-3511. doi: 10.1158/1078-0432.CCR-24-0657. PMID: 38864854Free PMC Article
Integrative analysis reveals early and distinct genetic and epigenetic changes in intraductal papillary and tubulopapillary cholangiocarcinogenesis.
Goeppert B, Stichel D, Toth R, Fritzsche S, Loeffler MA, Schlitter AM, Neumann O, Assenov Y, Vogel MN, Mehrabi A, Hoffmann K, Köhler B, Springfeld C, Weichenhan D, Plass C, Esposito I, Schirmacher P, von Deimling A, Roessler S
Gut 2022 Feb;71(2):391-401. Epub 2021 Jan 19 doi: 10.1136/gutjnl-2020-322983. PMID: 33468537Free PMC Article
Recent advances in the regulation of cholangiocarcinoma growth.
Francis H, Alpini G, DeMorrow S
Am J Physiol Gastrointest Liver Physiol 2010 Jul;299(1):G1-9. Epub 2010 Apr 29 doi: 10.1152/ajpgi.00114.2010. PMID: 20430870Free PMC Article
Cholangiocarcinoma.
Patel T
Nat Clin Pract Gastroenterol Hepatol 2006 Jan;3(1):33-42. doi: 10.1038/ncpgasthep0389. PMID: 16397610
Signet-ring cell carcinoma of the ampulla of Vater.
Li L, Chen QH, Sullivan JD, Breuer FU
Ann Clin Lab Sci 2004 Autumn;34(4):471-5. PMID: 15648791

Diagnosis

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
Integrative analysis reveals early and distinct genetic and epigenetic changes in intraductal papillary and tubulopapillary cholangiocarcinogenesis.
Goeppert B, Stichel D, Toth R, Fritzsche S, Loeffler MA, Schlitter AM, Neumann O, Assenov Y, Vogel MN, Mehrabi A, Hoffmann K, Köhler B, Springfeld C, Weichenhan D, Plass C, Esposito I, Schirmacher P, von Deimling A, Roessler S
Gut 2022 Feb;71(2):391-401. Epub 2021 Jan 19 doi: 10.1136/gutjnl-2020-322983. PMID: 33468537Free PMC Article
Omics-Based Platforms: Current Status and Potential Use for Cholangiocarcinoma.
Chang YC, Chen MH, Yeh CN, Hsiao M
Biomolecules 2020 Sep 28;10(10) doi: 10.3390/biom10101377. PMID: 32998289Free PMC Article
Immunoglobulin G4-related hepatobiliary disease.
Lee HE, Zhang L
Semin Diagn Pathol 2019 Nov;36(6):423-433. Epub 2019 Jul 24 doi: 10.1053/j.semdp.2019.07.007. PMID: 31358425
Cholangiocarcinoma.
Patel T
Nat Clin Pract Gastroenterol Hepatol 2006 Jan;3(1):33-42. doi: 10.1038/ncpgasthep0389. PMID: 16397610

Therapy

Molecular Characterization of Biliary Tract Cancer Predicts Chemotherapy and Programmed Death 1/Programmed Death-Ligand 1 Blockade Responses.
Yoon JG, Kim MH, Jang M, Kim H, Hwang HK, Kang CM, Lee WJ, Kang B, Lee CK, Lee MG, Chung HC, Choi HJ, Park YN
Hepatology 2021 Oct;74(4):1914-1931. Epub 2021 Jul 29 doi: 10.1002/hep.31862. PMID: 33884649
Omics-Based Platforms: Current Status and Potential Use for Cholangiocarcinoma.
Chang YC, Chen MH, Yeh CN, Hsiao M
Biomolecules 2020 Sep 28;10(10) doi: 10.3390/biom10101377. PMID: 32998289Free PMC Article
Peroral cholangioscopy of nivolumab-related (induced) ulcerative cholangitis in a patient with non-small cell lung cancer.
Kuraoka N, Hara K, Terai S, Yatabe Y, Horio Y
Endoscopy 2018 Sep;50(9):E259-E261. Epub 2018 Jul 3 doi: 10.1055/a-0640-2392. PMID: 29969801
Two-stage operation for synchronous triple primary cancer of the esophagus, stomach, and ampulla of Vater: report of a case.
Fukaya M, Abe T, Yokoyama Y, Itatsu K, Nagino M
Surg Today 2014 May;44(5):967-71. Epub 2013 Mar 17 doi: 10.1007/s00595-013-0549-x. PMID: 23504004
Photodynamic therapy and Klatskin tumour: an overview.
Rauws EA
Scand J Gastroenterol Suppl 2006;(243):135-8. doi: 10.1080/00365520600664441. PMID: 16782632

Prognosis

Lymphotoxin beta-activated LTBR/NIK/RELB axis drives proliferation in cholangiocarcinoma.
Xu K, Kessler A, Nichetti F, Hoffmeister-Wittmann P, Scherr AL, Nader L, Kelmendi E, Schmitt N, Schwab M, García-Beccaria M, Sobol B, Nieto OA, Isele H, Gärtner U, Vaquero-Siguero N, Volk J, Korell F, Mock A, Heide D, Ramadori P, Lenoir B, Albrecht T, Hüllein J, Jäger D, Fröhling S, Springfeld C, Jackstadt R, Heikenwälder M, Dill MT, Roessler S, Goeppert B, Köhler BC
Liver Int 2024 Nov;44(11):2950-2963. Epub 2024 Aug 20 doi: 10.1111/liv.16069. PMID: 39164890
A Novel Approach to Quantify Heterogeneity of Intrahepatic Cholangiocarcinoma: The Hidden-Genome Classifier.
Song Y, Boerner T, Drill E, Shin P, Kumar S, Sigel C, Cercek A, Kemeny N, Abou-Alfa G, Iacobuzio-Donahue C, Cowzer D, Schultz N, Walch H, Balachandran V, Groot Koerkamp B, Kingham P, Soares K, Wei A, D'Angelica M, Drebin J, Chandwani R, Harding JJ, Jarnagin W
Clin Cancer Res 2024 Aug 15;30(16):3499-3511. doi: 10.1158/1078-0432.CCR-24-0657. PMID: 38864854Free PMC Article
Proteogenomic characterization of cholangiocarcinoma.
Deng M, Ran P, Chen L, Wang Y, Yu Z, Cai K, Feng J, Qin Z, Yin Y, Tan S, Liu Y, Xu C, Shi G, Ji Y, Zhao JY, Zhou J, Fan J, Hou Y, Ding C
Hepatology 2023 Feb 1;77(2):411-429. Epub 2022 Jul 5 doi: 10.1002/hep.32624. PMID: 35716043Free PMC Article
Omics-Based Platforms: Current Status and Potential Use for Cholangiocarcinoma.
Chang YC, Chen MH, Yeh CN, Hsiao M
Biomolecules 2020 Sep 28;10(10) doi: 10.3390/biom10101377. PMID: 32998289Free PMC Article
Signet-ring cell carcinoma of the ampulla of Vater.
Li L, Chen QH, Sullivan JD, Breuer FU
Ann Clin Lab Sci 2004 Autumn;34(4):471-5. PMID: 15648791

Clinical prediction guides

A Novel Approach to Quantify Heterogeneity of Intrahepatic Cholangiocarcinoma: The Hidden-Genome Classifier.
Song Y, Boerner T, Drill E, Shin P, Kumar S, Sigel C, Cercek A, Kemeny N, Abou-Alfa G, Iacobuzio-Donahue C, Cowzer D, Schultz N, Walch H, Balachandran V, Groot Koerkamp B, Kingham P, Soares K, Wei A, D'Angelica M, Drebin J, Chandwani R, Harding JJ, Jarnagin W
Clin Cancer Res 2024 Aug 15;30(16):3499-3511. doi: 10.1158/1078-0432.CCR-24-0657. PMID: 38864854Free PMC Article
Decreasing Albumin mRNA Expression in Cholangiocarcinomas along the Bile Duct Tree.
Albertini E, Chillotti S, Monti G, Malvi D, Deserti M, Degiovanni A, Palloni A, Tavolari S, Brandi G, D'Errico A, Vasuri F
Pathobiology 2024;91(5):338-344. Epub 2024 May 19 doi: 10.1159/000538706. PMID: 38763129Free 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
PD-L1 expression in extrahepatic cholangiocarcinoma.
Walter D, Herrmann E, Schnitzbauer AA, Zeuzem S, Hansmann ML, Peveling-Oberhag J, Hartmann S
Histopathology 2017 Sep;71(3):383-392. Epub 2017 Jun 23 doi: 10.1111/his.13238. PMID: 28419539
Duodenal and ampullary carcinoid tumors. A report of 12 cases with pathological characteristics, polypeptide content and relation to the MEN I syndrome and von Recklinghausen's disease (neurofibromatosis).
Stamm B, Hedinger CE, Saremaslani P
Virchows Arch A Pathol Anat Histopathol 1986;408(5):475-89. doi: 10.1007/BF00705301. PMID: 2869609

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