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Intrahepatic cholangiocarcinoma

MedGen UID:
87521
Concept ID:
C0345905
Neoplastic Process
Synonyms: Cholangiocarcinoma, Intrahepatic; Cholangiocarcinomas, Intrahepatic; Intrahepatic Cholangiocarcinoma; Intrahepatic Cholangiocarcinomas
SNOMED CT: Intrahepatic bile duct carcinoma (109842005); Cholangiocarcinoma of intrahepatic biliary tract (1260050006); Intrahepatic cholangiocarcinoma (1260050006)
 
Monarch Initiative: MONDO:0003210

Definition

A carcinoma that arises from the intrahepatic bile duct epithelium in any site of the intrahepatic biliary tree. Grossly, the malignant lesions are solid, nodular, and grayish. Morphologically, the vast majority of cases are adenocarcinomas. Signs and symptoms include malaise, weight loss, right upper quadrant abdominal pain, and night sweats. Early detection is difficult and the prognosis is generally poor. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • Intrahepatic cholangiocarcinoma

Professional guidelines

PubMed

Moris D, Palta M, Kim C, Allen PJ, Morse MA, Lidsky ME
CA Cancer J Clin 2023 Mar;73(2):198-222. Epub 2022 Oct 19 doi: 10.3322/caac.21759. PMID: 36260350
Bridgewater J, Galle PR, Khan SA, Llovet JM, Park JW, Patel T, Pawlik TM, Gores GJ
J Hepatol 2014 Jun;60(6):1268-89. Epub 2014 Mar 27 doi: 10.1016/j.jhep.2014.01.021. PMID: 24681130
Ilyas SI, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396Free PMC Article

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Biliary Tract Cancers, 2024

Recent clinical studies

Etiology

European Association for the Study of the Liver
J Hepatol 2023 Jul;79(1):181-208. Epub 2023 Apr 20 doi: 10.1016/j.jhep.2023.03.010. PMID: 37084797
Valle JW, Kelley RK, Nervi B, Oh DY, Zhu AX
Lancet 2021 Jan 30;397(10272):428-444. doi: 10.1016/S0140-6736(21)00153-7. PMID: 33516341
Brandi G, Tavolari S
Cells 2020 Feb 12;9(2) doi: 10.3390/cells9020421. PMID: 32059499Free PMC Article
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
Massarweh NN, El-Serag HB
Cancer Control 2017 Jul-Sep;24(3):1073274817729245. doi: 10.1177/1073274817729245. PMID: 28975830Free PMC Article

Diagnosis

Harrison JM, Visser BC
Surg Clin North Am 2024 Dec;104(6):1281-1293. Epub 2024 May 10 doi: 10.1016/j.suc.2024.04.003. PMID: 39448128
Valle JW, Kelley RK, Nervi B, Oh DY, Zhu AX
Lancet 2021 Jan 30;397(10272):428-444. doi: 10.1016/S0140-6736(21)00153-7. PMID: 33516341
Mejia JC, Pasko J
Surg Clin North Am 2020 Jun;100(3):535-549. Epub 2020 Apr 16 doi: 10.1016/j.suc.2020.02.013. PMID: 32402299
Bridgewater J, Galle PR, Khan SA, Llovet JM, Park JW, Patel T, Pawlik TM, Gores GJ
J Hepatol 2014 Jun;60(6):1268-89. Epub 2014 Mar 27 doi: 10.1016/j.jhep.2014.01.021. PMID: 24681130
Brown KM, Parmar AD, Geller DA
Surg Oncol Clin N Am 2014 Apr;23(2):231-46. doi: 10.1016/j.soc.2013.10.004. PMID: 24560108Free PMC Article

Therapy

Goyal L, Meric-Bernstam F, Hollebecque A, Valle JW, Morizane C, Karasic TB, Abrams TA, Furuse J, Kelley RK, Cassier PA, Klümpen HJ, Chang HM, Chen LT, Tabernero J, Oh DY, Mahipal A, Moehler M, Mitchell EP, Komatsu Y, Masuda K, Ahn D, Epstein RS, Halim AB, Fu Y, Salimi T, Wacheck V, He Y, Liu M, Benhadji KA, Bridgewater JA; FOENIX-CCA2 Study Investigators
N Engl J Med 2023 Jan 19;388(3):228-239. doi: 10.1056/NEJMoa2206834. PMID: 36652354
Merters J, Lamarca A
J Hepatol 2023 Mar;78(3):652-657. Epub 2022 Nov 15 doi: 10.1016/j.jhep.2022.11.005. PMID: 36400328
Moris D, Palta M, Kim C, Allen PJ, Morse MA, Lidsky ME
CA Cancer J Clin 2023 Mar;73(2):198-222. Epub 2022 Oct 19 doi: 10.3322/caac.21759. PMID: 36260350
Mejia JC, Pasko J
Surg Clin North Am 2020 Jun;100(3):535-549. Epub 2020 Apr 16 doi: 10.1016/j.suc.2020.02.013. PMID: 32402299
Kelley RK, Bridgewater J, Gores GJ, Zhu AX
J Hepatol 2020 Feb;72(2):353-363. doi: 10.1016/j.jhep.2019.10.009. PMID: 31954497

Prognosis

Rumgay H, Arnold M, Ferlay J, Lesi O, Cabasag CJ, Vignat J, Laversanne M, McGlynn KA, Soerjomataram I
J Hepatol 2022 Dec;77(6):1598-1606. Epub 2022 Oct 5 doi: 10.1016/j.jhep.2022.08.021. PMID: 36208844Free PMC Article
Kelley RK, Bridgewater J, Gores GJ, Zhu AX
J Hepatol 2020 Feb;72(2):353-363. doi: 10.1016/j.jhep.2019.10.009. PMID: 31954497
Zamora-Valdes D, Heimbach JK
Gastroenterol Clin North Am 2018 Jun;47(2):267-280. doi: 10.1016/j.gtc.2018.01.002. PMID: 29735023
Ilyas SI, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396Free PMC Article
Poultsides GA, Zhu AX, Choti MA, Pawlik TM
Surg Clin North Am 2010 Aug;90(4):817-37. doi: 10.1016/j.suc.2010.04.011. PMID: 20637950

Clinical prediction guides

Ding GY, Ma JQ, Yun JP, Chen X, Ling Y, Zhang S, Shi JY, Chang YQ, Ji Y, Wang XY, Tan WM, Yuan KF, Yan B, Zhang XM, Liang F, Zhou J, Fan J, Zeng Y, Cai MY, Gao Q
J Hepatol 2022 Mar;76(3):608-618. Epub 2021 Nov 15 doi: 10.1016/j.jhep.2021.10.030. PMID: 34793865
Brandi G, Tavolari S
Cells 2020 Feb 12;9(2) doi: 10.3390/cells9020421. PMID: 32059499Free PMC Article
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
Xue R, Chen L, Zhang C, Fujita M, Li R, Yan SM, Ong CK, Liao X, Gao Q, Sasagawa S, Li Y, Wang J, Guo H, Huang QT, Zhong Q, Tan J, Qi L, Gong W, Hong Z, Li M, Zhao J, Peng T, Lu Y, Lim KHT, Boot A, Ono A, Chayama K, Zhang Z, Rozen SG, Teh BT, Wang XW, Nakagawa H, Zeng MS, Bai F, Zhang N
Cancer Cell 2019 Jun 10;35(6):932-947.e8. Epub 2019 May 23 doi: 10.1016/j.ccell.2019.04.007. PMID: 31130341Free PMC Article
Padia SA
Tech Vasc Interv Radiol 2015 Dec;18(4):227-35. Epub 2015 Jul 15 doi: 10.1053/j.tvir.2015.07.006. PMID: 26615163

Recent systematic reviews

Yeow M, Fong KY, Zhao JJ, Tan AYH, Koh YX, Kam JH, Goh BKP, Pawlik TM, Kabir T
HPB (Oxford) 2024 Feb;26(2):161-170. Epub 2023 Nov 20 doi: 10.1016/j.hpb.2023.11.007. PMID: 38071187
Wang L, Wen X, Zhuang L, Fang K, Shen J
J Gastrointestin Liver Dis 2022 Jun 12;31(2):215-222. doi: 10.15403/jgld-4207. PMID: 35574621
Apisarnthanarax S, Barry A, Cao M, Czito B, DeMatteo R, Drinane M, Hallemeier CL, Koay EJ, Lasley F, Meyer J, Owen D, Pursley J, Schaub SK, Smith G, Venepalli NK, Zibari G, Cardenes H
Pract Radiat Oncol 2022 Jan-Feb;12(1):28-51. Epub 2021 Oct 21 doi: 10.1016/j.prro.2021.09.004. PMID: 34688956
Fruscione M, Pickens RC, Baker EH, Martinie JB, Iannitti DA, Hwang JJ, Vrochides D
Curr Probl Cancer 2021 Feb;45(1):100614. Epub 2020 Jun 20 doi: 10.1016/j.currproblcancer.2020.100614. PMID: 32622478
Tsilidis KK, Kasimis JC, Lopez DS, Ntzani EE, Ioannidis JP
BMJ 2015 Jan 2;350:g7607. doi: 10.1136/bmj.g7607. PMID: 25555821

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    Clinical resources

    Practice guidelines

    • PubMed
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    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Curated

    • NCCN, 2024
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) Biliary Tract Cancers, 2024

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