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Malignant tumor of extrahepatic bile duct

MedGen UID:
56304
•Concept ID:
C0153453
•
Neoplastic Process
Synonyms: Ca extrahepatic bile ducts; cancer of extrahepatic bile duct; Extrahepatic bile duct cancer; Malignant Extrahepatic Bile Duct Neoplasm; malignant extrahepatic bile duct neoplasm; Malignant Extrahepatic Bile Duct Tumor; malignant extrahepatic bile duct tumor; malignant extrahepatic bile duct tumour; Malignant Neoplasm of Extrahepatic Bile Duct; malignant neoplasm of extrahepatic bile duct; Malignant Neoplasm of the Extrahepatic Bile Duct; malignant neoplasm of the extrahepatic bile duct; Malignant Tumor of Extrahepatic Bile Duct; malignant tumor of extrahepatic bile duct; Malignant Tumor of the Extrahepatic Bile Duct; malignant tumor of the extrahepatic bile duct; malignant tumour of the extrahepatic bile duct
SNOMED CT: Ca extrahepatic bile ducts (363416002); Extrahepatic bile duct cancer (363416002); Malignant tumor of extrahepatic bile duct (363416002)
 
Monarch Initiative: MONDO:0021321

Definition

A primary or metastatic malignant neoplasm that affects the extrahepatic bile ducts. Representative examples include carcinoma and sarcoma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMalignant tumor of extrahepatic bile duct

Professional guidelines

PubMed

The Diagnosis and Treatment of Ampullary Carcinoma.
Walter D, Schnitzbauer AA, Schulze F, Trojan J
Dtsch Arztebl Int 2023 Oct 27;120(43):729-735. doi: 10.3238/arztebl.m2023.0195. PMID: 37656482Free PMC Article
Diagnosis and management of choledochal cysts.
Brown ZJ, Baghdadi A, Kamel I, Labiner HE, Hewitt DB, Pawlik TM
HPB (Oxford) 2023 Jan;25(1):14-25. Epub 2022 Oct 5 doi: 10.1016/j.hpb.2022.09.010. PMID: 36257874
Clinical treatment of cholangiocarcinoma: an updated comprehensive review.
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836

Recent clinical studies

Etiology

Diagnosis and management of choledochal cysts.
Brown ZJ, Baghdadi A, Kamel I, Labiner HE, Hewitt DB, Pawlik TM
HPB (Oxford) 2023 Jan;25(1):14-25. Epub 2022 Oct 5 doi: 10.1016/j.hpb.2022.09.010. PMID: 36257874
Clinical treatment of cholangiocarcinoma: an updated comprehensive review.
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis.
Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA
J Hepatol 2020 Jan;72(1):95-103. Epub 2019 Sep 16 doi: 10.1016/j.jhep.2019.09.007. PMID: 31536748
Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma.
Bertuccio P, Malvezzi M, Carioli G, Hashim D, Boffetta P, El-Serag HB, La Vecchia C, Negri E
J Hepatol 2019 Jul;71(1):104-114. Epub 2019 Mar 23 doi: 10.1016/j.jhep.2019.03.013. PMID: 30910538
Cancer of the gallbladder and extrahepatic bile ducts.
Cleary SP, Dawson LA, Knox JJ, Gallinger S
Curr Probl Surg 2007 Jul;44(7):396-482. doi: 10.1067/j.cpsurg.2007.04.006. PMID: 17693325

Diagnosis

Diagnosis and management of choledochal cysts.
Brown ZJ, Baghdadi A, Kamel I, Labiner HE, Hewitt DB, Pawlik TM
HPB (Oxford) 2023 Jan;25(1):14-25. Epub 2022 Oct 5 doi: 10.1016/j.hpb.2022.09.010. PMID: 36257874
Clinical treatment of cholangiocarcinoma: an updated comprehensive review.
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma.
Bertuccio P, Malvezzi M, Carioli G, Hashim D, Boffetta P, El-Serag HB, La Vecchia C, Negri E
J Hepatol 2019 Jul;71(1):104-114. Epub 2019 Mar 23 doi: 10.1016/j.jhep.2019.03.013. PMID: 30910538
Cholangiocarcinoma.
Krasinskas AM
Surg Pathol Clin 2018 Jun;11(2):403-429. doi: 10.1016/j.path.2018.02.005. PMID: 29751883
SWOG S0809: A Phase II Intergroup Trial of Adjuvant Capecitabine and Gemcitabine Followed by Radiotherapy and Concurrent Capecitabine in Extrahepatic Cholangiocarcinoma and Gallbladder Carcinoma.
Ben-Josef E, Guthrie KA, El-Khoueiry AB, Corless CL, Zalupski MM, Lowy AM, Thomas CR Jr, Alberts SR, Dawson LA, Micetich KC, Thomas MB, Siegel AB, Blanke CD
J Clin Oncol 2015 Aug 20;33(24):2617-22. Epub 2015 May 11 doi: 10.1200/JCO.2014.60.2219. PMID: 25964250Free PMC Article

Therapy

Revolutionizing anti-HER2 therapies for extrahepatic cholangiocarcinoma and gallbladder cancer: Current advancements and future perspectives.
Ten Haaft BH, Pedregal M, Prato J, Klümpen HJ, Moreno V, Lamarca A
Eur J Cancer 2024 Mar;199:113564. Epub 2024 Jan 20 doi: 10.1016/j.ejca.2024.113564. PMID: 38266541
Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis.
Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA
J Hepatol 2020 Jan;72(1):95-103. Epub 2019 Sep 16 doi: 10.1016/j.jhep.2019.09.007. PMID: 31536748
Preoperative Stenting for Benign and Malignant Periampullary Diseases: Unnecessary if Not Harmful.
Gholami S, Brennan MF
Surg Clin North Am 2018 Feb;98(1):37-47. doi: 10.1016/j.suc.2017.09.005. PMID: 29191276Free PMC Article
SWOG S0809: A Phase II Intergroup Trial of Adjuvant Capecitabine and Gemcitabine Followed by Radiotherapy and Concurrent Capecitabine in Extrahepatic Cholangiocarcinoma and Gallbladder Carcinoma.
Ben-Josef E, Guthrie KA, El-Khoueiry AB, Corless CL, Zalupski MM, Lowy AM, Thomas CR Jr, Alberts SR, Dawson LA, Micetich KC, Thomas MB, Siegel AB, Blanke CD
J Clin Oncol 2015 Aug 20;33(24):2617-22. Epub 2015 May 11 doi: 10.1200/JCO.2014.60.2219. PMID: 25964250Free PMC Article
Cancer of the gallbladder and extrahepatic bile ducts.
Cleary SP, Dawson LA, Knox JJ, Gallinger S
Curr Probl Surg 2007 Jul;44(7):396-482. doi: 10.1067/j.cpsurg.2007.04.006. PMID: 17693325

Prognosis

Clinical treatment of cholangiocarcinoma: an updated comprehensive review.
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis.
Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA
J Hepatol 2020 Jan;72(1):95-103. Epub 2019 Sep 16 doi: 10.1016/j.jhep.2019.09.007. PMID: 31536748
Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma.
Bertuccio P, Malvezzi M, Carioli G, Hashim D, Boffetta P, El-Serag HB, La Vecchia C, Negri E
J Hepatol 2019 Jul;71(1):104-114. Epub 2019 Mar 23 doi: 10.1016/j.jhep.2019.03.013. PMID: 30910538
Cholangiocarcinoma.
Krasinskas AM
Surg Pathol Clin 2018 Jun;11(2):403-429. doi: 10.1016/j.path.2018.02.005. PMID: 29751883
SWOG S0809: A Phase II Intergroup Trial of Adjuvant Capecitabine and Gemcitabine Followed by Radiotherapy and Concurrent Capecitabine in Extrahepatic Cholangiocarcinoma and Gallbladder Carcinoma.
Ben-Josef E, Guthrie KA, El-Khoueiry AB, Corless CL, Zalupski MM, Lowy AM, Thomas CR Jr, Alberts SR, Dawson LA, Micetich KC, Thomas MB, Siegel AB, Blanke CD
J Clin Oncol 2015 Aug 20;33(24):2617-22. Epub 2015 May 11 doi: 10.1200/JCO.2014.60.2219. PMID: 25964250Free PMC Article

Clinical prediction guides

PTEN deficiency induces an extrahepatic cholangitis-cholangiocarcinoma continuum via aurora kinase A in mice.
Yang Y, Wang J, Wan J, Cheng Q, Cheng Z, Zhou X, Wang O, Shi K, Wang L, Wang B, Zhu X, Chen J, Feng D, Liu Y, Jahan-Mihan Y, Haddock AN, Edenfield BH, Peng G, Hohenstein JD, McCabe CE, O'Brien DR, Wang C, Ilyas SI, Jiang L, Torbenson MS, Wang H, Nakhleh RE, Shi X, Wang Y, Bi Y, Gores GJ, Patel T, Ji B
J Hepatol 2024 Jul;81(1):120-134. Epub 2024 Feb 28 doi: 10.1016/j.jhep.2024.02.018. PMID: 38428643Free PMC Article
Revolutionizing anti-HER2 therapies for extrahepatic cholangiocarcinoma and gallbladder cancer: Current advancements and future perspectives.
Ten Haaft BH, Pedregal M, Prato J, Klümpen HJ, Moreno V, Lamarca A
Eur J Cancer 2024 Mar;199:113564. Epub 2024 Jan 20 doi: 10.1016/j.ejca.2024.113564. PMID: 38266541
Intrahepatic Cholangiocarcinoma.
El-Diwany R, Pawlik TM, Ejaz A
Surg Oncol Clin N Am 2019 Oct;28(4):587-599. doi: 10.1016/j.soc.2019.06.002. PMID: 31472907
Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma.
Bertuccio P, Malvezzi M, Carioli G, Hashim D, Boffetta P, El-Serag HB, La Vecchia C, Negri E
J Hepatol 2019 Jul;71(1):104-114. Epub 2019 Mar 23 doi: 10.1016/j.jhep.2019.03.013. PMID: 30910538
SWOG S0809: A Phase II Intergroup Trial of Adjuvant Capecitabine and Gemcitabine Followed by Radiotherapy and Concurrent Capecitabine in Extrahepatic Cholangiocarcinoma and Gallbladder Carcinoma.
Ben-Josef E, Guthrie KA, El-Khoueiry AB, Corless CL, Zalupski MM, Lowy AM, Thomas CR Jr, Alberts SR, Dawson LA, Micetich KC, Thomas MB, Siegel AB, Blanke CD
J Clin Oncol 2015 Aug 20;33(24):2617-22. Epub 2015 May 11 doi: 10.1200/JCO.2014.60.2219. PMID: 25964250Free PMC Article

Recent systematic reviews

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
Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis.
Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA
J Hepatol 2020 Jan;72(1):95-103. Epub 2019 Sep 16 doi: 10.1016/j.jhep.2019.09.007. PMID: 31536748
A systematic review of the anatomical findings of multiple gallbladders.
Darnis B, Mohkam K, Cauchy F, Cazauran JB, Bancel B, Rode A, Ducerf C, Lesurtel M, Mabrut JY
HPB (Oxford) 2018 Nov;20(11):985-991. Epub 2018 Jun 7 doi: 10.1016/j.hpb.2018.04.002. PMID: 29887260
Treatment and Prognosis for Patients With Intrahepatic Cholangiocarcinoma: Systematic Review and Meta-analysis.
Mavros MN, Economopoulos KP, Alexiou VG, Pawlik TM
JAMA Surg 2014 Jun;149(6):565-74. doi: 10.1001/jamasurg.2013.5137. PMID: 24718873
Chemoradiation and brachytherapy in extrahepatic bile duct carcinoma.
Mattiucci GC, Autorino R, D'Agostino GR, Deodato F, Macchia G, Perri V, Tringali A, Morganti AG, Mutignani M, Valentini V
Crit Rev Oncol Hematol 2014 Apr;90(1):58-67. Epub 2013 Nov 2 doi: 10.1016/j.critrevonc.2013.10.007. PMID: 24289902

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