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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

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
HepPar-1 and Arginase-1 Immunohistochemistry in Adenocarcinoma of the Small Intestine and Ampullary Region.
Lagana S, Hsiao S, Bao F, Sepulveda A, Moreira R, Lefkowitch J, Remotti H
Arch Pathol Lab Med 2015 Jun;139(6):791-5. doi: 10.5858/arpa.2013-0249-OA. PMID: 26030248
Intrahepatic cholangiocarcinoma: new insights in pathology.
Sempoux C, Jibara G, Ward SC, Fan C, Qin L, Roayaie S, Fiel MI, Schwartz M, Thung SN
Semin Liver Dis 2011 Feb;31(1):49-60. Epub 2011 Feb 22 doi: 10.1055/s-0031-1272839. PMID: 21344350
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

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
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
Intrahepatic cholangiocarcinoma: new insights in pathology.
Sempoux C, Jibara G, Ward SC, Fan C, Qin L, Roayaie S, Fiel MI, Schwartz M, Thung SN
Semin Liver Dis 2011 Feb;31(1):49-60. Epub 2011 Feb 22 doi: 10.1055/s-0031-1272839. PMID: 21344350
Cholangiocarcinoma.
Patel T
Nat Clin Pract Gastroenterol Hepatol 2006 Jan;3(1):33-42. doi: 10.1038/ncpgasthep0389. PMID: 16397610

Therapy

Differential in vitro effects of targeted therapeutics in primary human liver cancer: importance for combined liver cancer.
Malik IA, Rajput M, Werner R, Fey D, Salehzadeh N, von Arnim CAF, Wilting J
BMC Cancer 2022 Nov 19;22(1):1193. doi: 10.1186/s12885-022-10247-6. PMID: 36402986Free PMC Article
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
Bile duct obstruction in a patient treated with nivolumab as second-line chemotherapy for advanced non-small-cell lung cancer: a case report.
Kashima J, Okuma Y, Shimizuguchi R, Chiba K
Cancer Immunol Immunother 2018 Jan;67(1):61-65. Epub 2017 Sep 14 doi: 10.1007/s00262-017-2062-3. PMID: 28913619Free PMC Article
Photodynamic therapy and Klatskin tumour: an overview.
Rauws EA
Scand J Gastroenterol Suppl 2006;(243):135-8. doi: 10.1080/00365520600664441. PMID: 16782632

Prognosis

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
Intrahepatic cholangiocarcinoma: new insights in pathology.
Sempoux C, Jibara G, Ward SC, Fan C, Qin L, Roayaie S, Fiel MI, Schwartz M, Thung SN
Semin Liver Dis 2011 Feb;31(1):49-60. Epub 2011 Feb 22 doi: 10.1055/s-0031-1272839. PMID: 21344350
Periampullary carcinomas.
Filiz G, Yerci O, Adim SB, Gurel S, Dolar E, Memik F
Hepatogastroenterology 2007 Jun;54(76):1247-9. PMID: 17629080
Carcinoid of the ampulla of Vater.
Hartel M, Wente MN, Sido B, Friess H, Büchler MW
J Gastroenterol Hepatol 2005 May;20(5):676-81. doi: 10.1111/j.1440-1746.2005.03744.x. PMID: 15853978

Clinical prediction guides

Differential in vitro effects of targeted therapeutics in primary human liver cancer: importance for combined liver cancer.
Malik IA, Rajput M, Werner R, Fey D, Salehzadeh N, von Arnim CAF, Wilting J
BMC Cancer 2022 Nov 19;22(1):1193. doi: 10.1186/s12885-022-10247-6. PMID: 36402986Free 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
Periampullary carcinomas.
Filiz G, Yerci O, Adim SB, Gurel S, Dolar E, Memik F
Hepatogastroenterology 2007 Jun;54(76):1247-9. PMID: 17629080
Choledochocele associated with superficial spreading cancer with cholesterolosis of the bile duct.
Horaguchi J, Fujita N, Noda Y, Kobayashi G, Ito K, Takasawa O, Obana T, Endo T, Nakahara K, Honda H
J Gastroenterol 2007 Apr;42(4):318-24. Epub 2007 Apr 26 doi: 10.1007/s00535-006-1994-8. PMID: 17464462

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