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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

Advances in the treatment of intrahepatic cholangiocarcinoma: An overview of the current and future therapeutic landscape for clinicians.
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
Global burden of primary liver cancer in 2020 and predictions to 2040.
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
Pathogenesis, diagnosis, and management of cholangiocarcinoma.
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

Intraarterial Chemotherapy for Liver Metastases.
Connell LC, Kemeny NE
Surg Oncol Clin N Am 2021 Jan;30(1):143-158. Epub 2020 Oct 20 doi: 10.1016/j.soc.2020.08.005. PMID: 33220802Free PMC Article

Diagnosis

Long-Term Survivor of Intrahepatic Cholangiocarcinoma for over 18 Years: Case Study with Longitudinal Histo-molecular and Tumor Immune Microenvironment Characterization and Systematic Review of the Literature.
Mattiolo P, De Bellis M, Mafficini A, Fassan M, Bevere M, Ciulla C, Bersani S, Lawlor RT, Milella M, Scarpa A, Luchini C, Ruzzenente A
J Gastrointest Cancer 2024 Dec;55(4):1634-1646. Epub 2024 Sep 16 doi: 10.1007/s12029-024-01113-8. PMID: 39283582Free PMC Article

Therapy

Futibatinib for FGFR2-Rearranged Intrahepatic Cholangiocarcinoma.
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
Advances in the treatment of intrahepatic cholangiocarcinoma: An overview of the current and future therapeutic landscape for clinicians.
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
Primary Liver Cancers: Intrahepatic Cholangiocarcinoma and Hepatocellular Carcinoma.
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
Systemic therapies for intrahepatic cholangiocarcinoma.
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
Surgery for cholangiocarcinoma.
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

Global burden of primary liver cancer in 2020 and predictions to 2040.
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
Global, regional and national burden of primary liver cancer by subtype.
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
Systemic therapies for intrahepatic cholangiocarcinoma.
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
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
Pathogenesis, diagnosis, and management of cholangiocarcinoma.
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

Charting co-mutation patterns associated with actionable drivers in intrahepatic cholangiocarcinoma.
Kendre G, Murugesan K, Brummer T, Segatto O, Saborowski A, Vogel A
J Hepatol 2023 Mar;78(3):614-626. Epub 2022 Dec 15 doi: 10.1016/j.jhep.2022.11.030. PMID: 36528236
Distribution and density of tertiary lymphoid structures predict clinical outcome in intrahepatic cholangiocarcinoma.
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
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
Genomic and Transcriptomic Profiling of Combined Hepatocellular and Intrahepatic Cholangiocarcinoma Reveals Distinct Molecular Subtypes.
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
Intrahepatic Cholangiocarcinoma.
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

The present roles and future perspectives of Interleukin-6 in biliary tract cancer.
Zhou M, Na R, Lai S, Guo Y, Shi J, Nie J, Zhang S, Wang Y, Zheng T
Cytokine 2023 Sep;169:156271. Epub 2023 Jun 16 doi: 10.1016/j.cyto.2023.156271. PMID: 37331095
Major complications and mortality after resection of intrahepatic cholangiocarcinoma: A systematic review and meta-analysis.
van Keulen AM, Büttner S, Erdmann JI, Hagendoorn J, Hoogwater FJH, IJzermans JNM, Neumann UP, Polak WG, De Jonge J, Olthof PB, Koerkamp BG
Surgery 2023 Apr;173(4):973-982. Epub 2022 Dec 27 doi: 10.1016/j.surg.2022.11.027. PMID: 36577599
Transarterial Yttrium-90 Radioembolization for Unresectable Intrahepatic Cholangiocarcinoma: A Systematic Review and Meta-Analysis.
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
External Beam Radiation Therapy for Primary Liver Cancers: An ASTRO Clinical Practice Guideline.
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
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

Supplemental Content

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    Clinical resources

    Practice guidelines

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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

    Reviews

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