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Adenocarcinoma of gallbladder and extrahepatic biliary tract

MedGen UID:
1633887
•Concept ID:
C4707555
•
Neoplastic Process
Synonyms: adenocarcinoma of gallbladder and EBT; adenocarcinoma of gallbladder and extrahepatic biliary tract; Adenocarcinoma of the gallbladder and EBT; adenocarcinoma of the gallbladder and EBT; adenocarcinoma of the gallbladder and extrahepatic biliary tract; Adenocarcinoma of the gallbladder and extrahepatic biliary tract
SNOMED CT: Adenocarcinoma of gallbladder and extrahepatic biliary tract (765741003)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018536
Orphanet: ORPHA424991

Definition

A rare epithelial carcinoma arising either in the gallbladder itself or from the epithelium lining the extrahepatic biliary tree, cystic duct and/or peribiliary gland. The disease has characteristics of nonspecific symptoms, such as abdominal pain, jaundice and vomiting and sometimes mimicking benign biliary diseases. Chronic biliary epithelial inflammation (for example primary sclerosing cholangitis, cholelithiasis, choledocholithiasis, liver fluke infestation) is a major risk factor. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdenocarcinoma of gallbladder and extrahepatic biliary tract

Professional guidelines

PubMed

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
The role of capecitabine in the management of tumors of the digestive system.
Gennatas C, Michalaki V, Gennatas S
Rev Recent Clin Trials 2009 Jan;4(1):1-11. doi: 10.2174/157488709787047576. PMID: 19149758
Surgical management of cholangiocarcinoma.
Jarnagin WR, Shoup M
Semin Liver Dis 2004 May;24(2):189-99. doi: 10.1055/s-2004-828895. PMID: 15192791

Recent clinical studies

Etiology

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
Trastuzumab plus FOLFOX for HER2-positive biliary tract cancer refractory to gemcitabine and cisplatin: a multi-institutional phase 2 trial of the Korean Cancer Study Group (KCSG-HB19-14).
Lee CK, Chon HJ, Cheon J, Lee MA, Im HS, Jang JS, Kim MH, Park S, Kang B, Hong M, Kim JW, Park HS, Kang MJ, Park YN, Choi HJ
Lancet Gastroenterol Hepatol 2023 Jan;8(1):56-65. Epub 2022 Oct 31 doi: 10.1016/S2468-1253(22)00335-1. PMID: 36328033
Targeting BRAF-Mutant Biliary Tract Cancer: Recent Advances and Future Challenges.
Rizzo A, Federico AD, Ricci AD, Frega G, Palloni A, Pagani R, Tavolari S, Marco MD, Brandi G
Cancer Control 2020 Jan-Dec;27(1):1073274820983013. doi: 10.1177/1073274820983013. PMID: 33356500Free PMC Article
Cholangiocarcinoma: update and future perspectives.
Gatto M, Bragazzi MC, Semeraro R, Napoli C, Gentile R, Torrice A, Gaudio E, Alvaro D
Dig Liver Dis 2010 Apr;42(4):253-60. Epub 2010 Jan 22 doi: 10.1016/j.dld.2009.12.008. PMID: 20097142
Surgical management of cholangiocarcinoma.
Jarnagin WR, Shoup M
Semin Liver Dis 2004 May;24(2):189-99. doi: 10.1055/s-2004-828895. PMID: 15192791

Diagnosis

Biliary Tract Cancer: Molecular Biology of Precursor Lesions.
Manzano-Núñez F, Prates Tiago Aguilar L, Sempoux C, Lemaigre FP
Semin Liver Dis 2023 Nov;43(4):472-484. Epub 2023 Nov 9 doi: 10.1055/a-2207-9834. PMID: 37944999
NCCN Guidelines® Insights: Biliary Tract Cancers, Version 2.2023.
Benson AB, D'Angelica MI, Abrams T, Abbott DE, Ahmed A, Anaya DA, Anders R, Are C, Bachini M, Binder D, Borad M, Bowlus C, Brown D, Burgoyne A, Castellanos J, Chahal P, Cloyd J, Covey AM, Glazer ES, Hawkins WG, Iyer R, Jacob R, Jennings L, Kelley RK, Kim R, Levine M, Palta M, Park JO, Raman S, Reddy S, Ronnekleiv-Kelly S, Sahai V, Singh G, Stein S, Turk A, Vauthey JN, Venook AP, Yopp A, McMillian N, Schonfeld R, Hochstetler C
J Natl Compr Canc Netw 2023 Jul;21(7):694-704. doi: 10.6004/jnccn.2023.0035. PMID: 37433432
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
Targeting BRAF-Mutant Biliary Tract Cancer: Recent Advances and Future Challenges.
Rizzo A, Federico AD, Ricci AD, Frega G, Palloni A, Pagani R, Tavolari S, Marco MD, Brandi G
Cancer Control 2020 Jan-Dec;27(1):1073274820983013. doi: 10.1177/1073274820983013. PMID: 33356500Free 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

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
Trastuzumab plus FOLFOX for HER2-positive biliary tract cancer refractory to gemcitabine and cisplatin: a multi-institutional phase 2 trial of the Korean Cancer Study Group (KCSG-HB19-14).
Lee CK, Chon HJ, Cheon J, Lee MA, Im HS, Jang JS, Kim MH, Park S, Kang B, Hong M, Kim JW, Park HS, Kang MJ, Park YN, Choi HJ
Lancet Gastroenterol Hepatol 2023 Jan;8(1):56-65. Epub 2022 Oct 31 doi: 10.1016/S2468-1253(22)00335-1. PMID: 36328033
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
Targeting BRAF-Mutant Biliary Tract Cancer: Recent Advances and Future Challenges.
Rizzo A, Federico AD, Ricci AD, Frega G, Palloni A, Pagani R, Tavolari S, Marco MD, Brandi G
Cancer Control 2020 Jan-Dec;27(1):1073274820983013. doi: 10.1177/1073274820983013. PMID: 33356500Free 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

Prognosis

Biliary Tract Cancer: Molecular Biology of Precursor Lesions.
Manzano-Núñez F, Prates Tiago Aguilar L, Sempoux C, Lemaigre FP
Semin Liver Dis 2023 Nov;43(4):472-484. Epub 2023 Nov 9 doi: 10.1055/a-2207-9834. PMID: 37944999
Targeting BRAF-Mutant Biliary Tract Cancer: Recent Advances and Future Challenges.
Rizzo A, Federico AD, Ricci AD, Frega G, Palloni A, Pagani R, Tavolari S, Marco MD, Brandi G
Cancer Control 2020 Jan-Dec;27(1):1073274820983013. doi: 10.1177/1073274820983013. PMID: 33356500Free PMC Article
Pathology of cholangiocellular carcinoma.
Hrudka J, Oliverius M, Gürlich R
Cas Lek Cesk 2019 Spring;158(2):57-63. PMID: 31109164
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
Imaging of malignancies of the biliary tract- an update.
Hennedige TP, Neo WT, Venkatesh SK
Cancer Imaging 2014 Apr 22;14(1):14. doi: 10.1186/1470-7330-14-14. PMID: 25608662Free PMC Article

Clinical prediction guides

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
Trastuzumab plus FOLFOX for HER2-positive biliary tract cancer refractory to gemcitabine and cisplatin: a multi-institutional phase 2 trial of the Korean Cancer Study Group (KCSG-HB19-14).
Lee CK, Chon HJ, Cheon J, Lee MA, Im HS, Jang JS, Kim MH, Park S, Kang B, Hong M, Kim JW, Park HS, Kang MJ, Park YN, Choi HJ
Lancet Gastroenterol Hepatol 2023 Jan;8(1):56-65. Epub 2022 Oct 31 doi: 10.1016/S2468-1253(22)00335-1. PMID: 36328033
Mutational spectrum and precision oncology for biliary tract carcinoma.
Lin J, Cao Y, Yang X, Li G, Shi Y, Wang D, Long J, Song Y, Mao J, Xie F, Bai Y, Zhang L, Yang X, Wan X, Wang A, Guan M, Zhao L, Hu K, Pan J, Huo L, Lu X, Mao Y, Sang X, Zhang H, Wang K, Wang X, Zhao H
Theranostics 2021;11(10):4585-4598. Epub 2021 Mar 4 doi: 10.7150/thno.56539. PMID: 33754015Free 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
Multiple tumors of the biliary tract.
Gertsch P, Thomas P, Baer H, Lerut J, Zimmermann A, Blumgart LH
Am J Surg 1990 Apr;159(4):386-8. doi: 10.1016/s0002-9610(05)81278-4. PMID: 2180336

Recent systematic reviews

Statins associate with lower risk of biliary tract cancers: A systematic review and meta-analysis.
Cheung KS, Yeung YWM, Wong WS, Li B, Seto WK, Leung WK
Cancer Med 2023 Jan;12(1):557-568. Epub 2022 Jun 13 doi: 10.1002/cam4.4942. PMID: 35698295Free PMC Article
Cholangiocarcinoma Attributed to Occupation: A Systematic Reviews.
Seeherunwong A, Chaiear N, Khuntikeo N, Ekpanyaskul C
Asian Pac J Cancer Prev 2022 Jun 1;23(6):1837-1845. doi: 10.31557/APJCP.2022.23.6.1837. PMID: 35763622Free PMC Article
Crossing borders: A systematic review with quantitative analysis of genetic mutations of carcinomas of the biliary tract.
Roos E, Soer EC, Klompmaker S, Meijer LL, Besselink MG, Giovannetti E, Heger M, Kazemier G, Klümpen HJ, Takkenberg RB, Wilmink H, Würdinger T, Dijk F, van Gulik TM, Verheij J, van de Vijver MJ
Crit Rev Oncol Hematol 2019 Aug;140:8-16. Epub 2019 May 24 doi: 10.1016/j.critrevonc.2019.05.011. PMID: 31158800
Gemcitabine-based chemotherapy for advanced biliary tract carcinomas.
Abdel-Rahman O, Elsayed Z, Elhalawani H
Cochrane Database Syst Rev 2018 Apr 6;4(4):CD011746. doi: 10.1002/14651858.CD011746.pub2. PMID: 29624208Free PMC Article

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