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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
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
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

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
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
Ampullary carcinoid tumour presenting with extrahepatic biliary obstruction.
Shafique MS, Fahim M, Shahbaz Bhatti ST, Khan JS, Khan MM
J Pak Med Assoc 2015 Aug;65(8):892-4. PMID: 26228340
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
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
Multiple, unique, and common p53 mutations in a thorotrast recipient with four primary cancers.
Iwamoto KS, Mizuno T, Kurata A, Masuzawa M, Mori T, Seyama T
Hum Pathol 1998 Apr;29(4):412-6. doi: 10.1016/s0046-8177(98)90126-5. PMID: 9563795

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
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
Ampullary carcinoid tumour presenting with extrahepatic biliary obstruction.
Shafique MS, Fahim M, Shahbaz Bhatti ST, Khan JS, Khan MM
J Pak Med Assoc 2015 Aug;65(8):892-4. PMID: 26228340
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
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
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
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
MK-1 expression in carcinoma of the ampulla of Vater as a predictor of improved prognosis after surgical resection.
Hamada Y, Nakayama Y, Mizoguchi M, Ikeda S, Kuroki M, Iwasaki H
Cancer Lett 2006 Nov 18;243(2):211-6. Epub 2006 Apr 17 doi: 10.1016/j.canlet.2005.11.037. PMID: 16616808

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