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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

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
Hepatobiliary Cancers, Version 2.2021, NCCN Clinical Practice Guidelines in Oncology.
Benson AB, D'Angelica MI, Abbott DE, Anaya DA, Anders R, Are C, Bachini M, Borad M, Brown D, Burgoyne A, Chahal P, Chang DT, Cloyd J, Covey AM, Glazer ES, Goyal L, Hawkins WG, Iyer R, Jacob R, Kelley RK, Kim R, Levine M, Palta M, Park JO, Raman S, Reddy S, Sahai V, Schefter T, Singh G, Stein S, Vauthey JN, Venook AP, Yopp A, McMillian NR, Hochstetler C, Darlow SD
J Natl Compr Canc Netw 2021 May 1;19(5):541-565. doi: 10.6004/jnccn.2021.0022. PMID: 34030131

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
Epidemiology of cholangiocarcinoma.
Bergquist A, von Seth E
Best Pract Res Clin Gastroenterol 2015 Apr;29(2):221-32. Epub 2015 Feb 16 doi: 10.1016/j.bpg.2015.02.003. PMID: 25966423

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

Adjuvant gemcitabine plus cisplatin versus capecitabine in node-positive extrahepatic cholangiocarcinoma: the STAMP randomized trial.
Jeong H, Kim KP, Jeong JH, Hwang DW, Lee JH, Kim KH, Moon DB, Lee MA, Park SJ, Chon HJ, Park JH, Lee JS, Ryoo BY, Yoo C
Hepatology 2023 May 1;77(5):1540-1549. Epub 2023 Jan 3 doi: 10.1097/HEP.0000000000000046. PMID: 37070950
Molecular profiling and treatment pattern differences between intrahepatic and extrahepatic cholangiocarcinoma.
Spencer K, Pappas L, Baiev I, Maurer J, Bocobo AG, Zhang K, Jain A, De Armas AD, Reyes S, Le TM, Rahma OE, Stanton J, DeLeon TT, Roth M, Peters MLB, Zhu AX, Lennerz JK, Iafrate AJ, Boyhen K, VanCott C, Roberts LR, Lindsey S, Horick N, Goff LW, Mody K, Borad MJ, Shroff RT, Kelley RK, Javle MM, Goyal L
J Natl Cancer Inst 2023 Jul 6;115(7):870-880. doi: 10.1093/jnci/djad046. PMID: 37040087Free PMC Article
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
Cholangiocellular Carcinoma.
Vogel A, Saborowski A
Digestion 2017;95(3):181-185. Epub 2017 Mar 14 doi: 10.1159/000454763. PMID: 28288474
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

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

Management of primary sclerosing cholangitis and its complications: an algorithmic approach.
Prokopič M, Beuers U
Hepatol Int 2021 Feb;15(1):6-20. Epub 2020 Dec 30 doi: 10.1007/s12072-020-10118-x. PMID: 33377990Free PMC Article
Asbestos and Intrahepatic Cholangiocarcinoma.
Brandi G, Tavolari S
Cells 2020 Feb 12;9(2) doi: 10.3390/cells9020421. PMID: 32059499Free PMC Article
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

Cystic duct cyst in adults: a systematic review of the sixth entity.
Al Samaraee A, Bhattacharya V
Surg Today 2023 May;53(5):527-543. Epub 2022 Feb 6 doi: 10.1007/s00595-022-02461-3. PMID: 35124738
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
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
Review of the investigation and surgical management of resectable ampullary adenocarcinoma.
Askew J, Connor S
HPB (Oxford) 2013 Nov;15(11):829-38. Epub 2013 Jan 10 doi: 10.1111/hpb.12038. PMID: 23458317Free PMC Article

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