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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]

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
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
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. Electronic address: easloffice@easloffice.eu; 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
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
Orcutt ST, Anaya DA
Cancer Control 2018 Jan-Mar;25(1):1073274817744621. doi: 10.1177/1073274817744621. PMID: 29327594Free PMC Article
Massarweh NN, El-Serag HB
Cancer Control 2017 Jul-Sep;24(3):1073274817729245. doi: 10.1177/1073274817729245. PMID: 28975830Free PMC Article

Diagnosis

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
Khan AS, Dageforde LA
Surg Clin North Am 2019 Apr;99(2):315-335. Epub 2019 Feb 10 doi: 10.1016/j.suc.2018.12.004. PMID: 30846037
Lee AJ, Chun YS
Chin Clin Oncol 2018 Oct;7(5):52. Epub 2018 Jul 12 doi: 10.21037/cco.2018.07.03. PMID: 30180751
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

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
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
Cillo U, Fondevila C, Donadon M, Gringeri E, Mocchegiani F, Schlitt HJ, Ijzermans JNM, Vivarelli M, Zieniewicz K, Olde Damink SWM, Groot Koerkamp B
Liver Int 2019 May;39 Suppl 1(Suppl Suppl 1):143-155. doi: 10.1111/liv.14089. PMID: 30843343Free PMC Article

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
Rumgay H, Ferlay J, de Martel C, Georges D, Ibrahim AS, Zheng R, Wei W, Lemmens VEPP, Soerjomataram I
Eur J Cancer 2022 Jan;161:108-118. Epub 2021 Dec 20 doi: 10.1016/j.ejca.2021.11.023. PMID: 34942552
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
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
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

Clinical prediction guides

Zhou SN, Jv DW, Meng XF, Zhang JJ, Liu C, Wu ZY, Hong N, Lu YY, Zhang N
Ann Med 2023 Dec;55(1):215-223. doi: 10.1080/07853890.2022.2160008. PMID: 36576390Free PMC Article
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
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

Schartz DA, Porter M, Schartz E, Kallas J, Gupta A, Butani D, Cantos A
J Vasc Interv Radiol 2022 Jun;33(6):679-686. Epub 2022 Feb 25 doi: 10.1016/j.jvir.2022.02.016. PMID: 35219834
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
Edeline J, Lamarca A, McNamara MG, Jacobs T, Hubner RA, Palmer D, Groot Koerkamp B, Johnson P, Guiu B, Valle JW
Cancer Treat Rev 2021 Sep;99:102258. Epub 2021 Jul 7 doi: 10.1016/j.ctrv.2021.102258. PMID: 34252720
Ziogas IA, Giannis D, Economopoulos KP, Hayat MH, Montenovo MI, Matsuoka LK, Alexopoulos SP
Transplantation 2021 Oct 1;105(10):2263-2271. doi: 10.1097/TP.0000000000003539. PMID: 33196623
Mavros MN, Economopoulos KP, Alexiou VG, Pawlik TM
JAMA Surg 2014 Jun;149(6):565-74. doi: 10.1001/jamasurg.2013.5137. PMID: 24718873

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

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