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Clear Cell Intrahepatic Cholangiocarcinoma

MedGen UID:
317517
•Concept ID:
C1707406
•
Neoplastic Process

Definition

A morphologic variant of intrahepatic cholangiocarcinoma composed of malignant glandular epithelium, an abundant fibrous stroma, and the presence of clear cells. Clinical symptoms include abdominal pain, weight loss and malaise. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClear Cell Intrahepatic Cholangiocarcinoma

Professional guidelines

PubMed

Survey of activated kinase proteins reveals potential targets for cholangiocarcinoma treatment.
Dokduang H, Juntana S, Techasen A, Namwat N, Yongvanit P, Khuntikeo N, Riggins GJ, Loilome W
Tumour Biol 2013 Dec;34(6):3519-28. Epub 2013 Jun 29 doi: 10.1007/s13277-013-0930-9. PMID: 23812726
A systematic review of photodynamic therapy in the treatment of pre-cancerous skin conditions, Barrett's oesophagus and cancers of the biliary tract, brain, head and neck, lung, oesophagus and skin.
Fayter D, Corbett M, Heirs M, Fox D, Eastwood A
Health Technol Assess 2010 Jul;14(37):1-288. doi: 10.3310/hta14370. PMID: 20663420
CD56 expression aids in the differential diagnosis of cholangiocarcinomas and benign cholangiocellular lesions.
Gütgemann I, Haas S, Berg JP, Zhou H, Büttner R, Fischer HP
Virchows Arch 2006 Apr;448(4):407-11. Epub 2006 Jan 13 doi: 10.1007/s00428-005-0145-6. PMID: 16411132

Recent clinical studies

Etiology

Extrahepatic cancers and chronic HCV infection.
Pol S, Vallet-Pichard A, Hermine O
Nat Rev Gastroenterol Hepatol 2018 May;15(5):283-290. Epub 2018 Jan 17 doi: 10.1038/nrgastro.2017.172. PMID: 29339810
Hepatolithiasis and intrahepatic cholangiocarcinoma: A review.
Kim HJ, Kim JS, Joo MK, Lee BJ, Kim JH, Yeon JE, Park JJ, Byun KS, Bak YT
World J Gastroenterol 2015 Dec 28;21(48):13418-31. doi: 10.3748/wjg.v21.i48.13418. PMID: 26730152Free PMC Article
Emerging roles of Notch signaling in liver disease.
Geisler F, Strazzabosco M
Hepatology 2015 Jan;61(1):382-92. Epub 2014 Sep 19 doi: 10.1002/hep.27268. PMID: 24930574Free PMC Article
Histopathology of hepatocellular carcinoma.
Schlageter M, Terracciano LM, D'Angelo S, Sorrentino P
World J Gastroenterol 2014 Nov 21;20(43):15955-64. doi: 10.3748/wjg.v20.i43.15955. PMID: 25473149Free PMC Article
Cholangiocarcinoma.
Patel T
Nat Clin Pract Gastroenterol Hepatol 2006 Jan;3(1):33-42. doi: 10.1038/ncpgasthep0389. PMID: 16397610

Diagnosis

Update on hepatocellular carcinoma: Pathologists' review.
El Jabbour T, Lagana SM, Lee H
World J Gastroenterol 2019 Apr 14;25(14):1653-1665. doi: 10.3748/wjg.v25.i14.1653. PMID: 31011252Free PMC Article
Hepatolithiasis and intrahepatic cholangiocarcinoma: A review.
Kim HJ, Kim JS, Joo MK, Lee BJ, Kim JH, Yeon JE, Park JJ, Byun KS, Bak YT
World J Gastroenterol 2015 Dec 28;21(48):13418-31. doi: 10.3748/wjg.v21.i48.13418. PMID: 26730152Free PMC Article
Annexin A4 and cancer.
Wei B, Guo C, Liu S, Sun MZ
Clin Chim Acta 2015 Jul 20;447:72-8. Epub 2015 Jun 3 doi: 10.1016/j.cca.2015.05.016. PMID: 26048190
Histopathology of hepatocellular carcinoma.
Schlageter M, Terracciano LM, D'Angelo S, Sorrentino P
World J Gastroenterol 2014 Nov 21;20(43):15955-64. doi: 10.3748/wjg.v20.i43.15955. PMID: 25473149Free PMC Article
Cholangiocarcinoma.
Patel T
Nat Clin Pract Gastroenterol Hepatol 2006 Jan;3(1):33-42. doi: 10.1038/ncpgasthep0389. PMID: 16397610

Therapy

Anti-PD-1 in Combination With Trametinib Suppresses Tumor Growth and Improves Survival of Intrahepatic Cholangiocarcinoma in Mice.
Wabitsch S, Tandon M, Ruf B, Zhang Q, McCallen JD, McVey JC, Ma C, Green BL, Diggs LP, Heinrich B, Greten TF
Cell Mol Gastroenterol Hepatol 2021;12(3):1166-1178. Epub 2021 May 23 doi: 10.1016/j.jcmgh.2021.05.011. PMID: 34033968Free PMC Article
Perihilar clear cell cholangiocarcinoma: an often-diagnostic challenge.
Marzouk O, Lakshmaiah S, Anjarwalla S
BMJ Case Rep 2020 Dec 22;13(12) doi: 10.1136/bcr-2020-237683. PMID: 33370986Free PMC Article
The cancer driver genes IDH1/2, JARID1C/ KDM5C, and UTX/ KDM6A: crosstalk between histone demethylation and hypoxic reprogramming in cancer metabolism.
Chang S, Yim S, Park H
Exp Mol Med 2019 Jun 20;51(6):1-17. doi: 10.1038/s12276-019-0230-6. PMID: 31221981Free PMC Article
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.
Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J
N Engl J Med 2015 Aug 20;373(8):726-36. doi: 10.1056/NEJMoa1502309. PMID: 26287849Free PMC Article
Tumor hypercalcemia and "ectopic hyperparathyroidism".
Skrabanek P, McPartlin J, Powell D
Medicine (Baltimore) 1980 Jul;59(4):262-82. doi: 10.1097/00005792-198007000-00003. PMID: 6993843

Prognosis

CircZNF215 promotes tumor growth and metastasis through inactivation of the PTEN/AKT pathway in intrahepatic cholangiocarcinoma.
Liao W, Du J, Li L, Wu X, Chen X, Feng Q, Xu L, Chen X, Liao M, Huang J, Yuan K, Zeng Y
J Exp Clin Cancer Res 2023 May 18;42(1):125. doi: 10.1186/s13046-023-02699-w. PMID: 37198696Free PMC Article
The Role of IgG4 in Autoimmunity and Rheumatic Diseases.
Maslinska M, Dmowska-Chalaba J, Jakubaszek M
Front Immunol 2021;12:787422. Epub 2022 Jan 25 doi: 10.3389/fimmu.2021.787422. PMID: 35145508Free PMC Article
The cancer driver genes IDH1/2, JARID1C/ KDM5C, and UTX/ KDM6A: crosstalk between histone demethylation and hypoxic reprogramming in cancer metabolism.
Chang S, Yim S, Park H
Exp Mol Med 2019 Jun 20;51(6):1-17. doi: 10.1038/s12276-019-0230-6. PMID: 31221981Free PMC Article
Update on hepatocellular carcinoma: Pathologists' review.
El Jabbour T, Lagana SM, Lee H
World J Gastroenterol 2019 Apr 14;25(14):1653-1665. doi: 10.3748/wjg.v25.i14.1653. PMID: 31011252Free PMC Article
Annexin A4 and cancer.
Wei B, Guo C, Liu S, Sun MZ
Clin Chim Acta 2015 Jul 20;447:72-8. Epub 2015 Jun 3 doi: 10.1016/j.cca.2015.05.016. PMID: 26048190

Clinical prediction guides

CircZNF215 promotes tumor growth and metastasis through inactivation of the PTEN/AKT pathway in intrahepatic cholangiocarcinoma.
Liao W, Du J, Li L, Wu X, Chen X, Feng Q, Xu L, Chen X, Liao M, Huang J, Yuan K, Zeng Y
J Exp Clin Cancer Res 2023 May 18;42(1):125. doi: 10.1186/s13046-023-02699-w. PMID: 37198696Free PMC Article
Roles and mechanisms of BAP1 deubiquitinase in tumor suppression.
Masclef L, Ahmed O, Estavoyer B, Larrivée B, Labrecque N, Nijnik A, Affar EB
Cell Death Differ 2021 Feb;28(2):606-625. Epub 2021 Jan 18 doi: 10.1038/s41418-020-00709-4. PMID: 33462414Free PMC Article
The cancer driver genes IDH1/2, JARID1C/ KDM5C, and UTX/ KDM6A: crosstalk between histone demethylation and hypoxic reprogramming in cancer metabolism.
Chang S, Yim S, Park H
Exp Mol Med 2019 Jun 20;51(6):1-17. doi: 10.1038/s12276-019-0230-6. PMID: 31221981Free PMC Article
Update on hepatocellular carcinoma: Pathologists' review.
El Jabbour T, Lagana SM, Lee H
World J Gastroenterol 2019 Apr 14;25(14):1653-1665. doi: 10.3748/wjg.v25.i14.1653. PMID: 31011252Free PMC Article
Vemurafenib in Multiple Nonmelanoma Cancers with BRAF V600 Mutations.
Hyman DM, Puzanov I, Subbiah V, Faris JE, Chau I, Blay JY, Wolf J, Raje NS, Diamond EL, Hollebecque A, Gervais R, Elez-Fernandez ME, Italiano A, Hofheinz RD, Hidalgo M, Chan E, Schuler M, Lasserre SF, Makrutzki M, Sirzen F, Veronese ML, Tabernero J, Baselga J
N Engl J Med 2015 Aug 20;373(8):726-36. doi: 10.1056/NEJMoa1502309. PMID: 26287849Free PMC Article

Recent systematic reviews

Phenotypical and potential functional characteristics of different immune cells expressing CD28H/B7-H5 and their relationship with cancer prognosis.
Zhong C, Lang Q, Yu J, Wu S, Xu F, Tian Y
Clin Exp Immunol 2020 Apr;200(1):12-21. Epub 2020 Feb 4 doi: 10.1111/cei.13413. PMID: 31901178Free PMC Article
Possible association between hepatitis C virus and malignancies different from hepatocellular carcinoma: A systematic review.
Fiorino S, Bacchi-Reggiani L, de Biase D, Fornelli A, Masetti M, Tura A, Grizzi F, Zanello M, Mastrangelo L, Lombardi R, Acquaviva G, di Tommaso L, Bondi A, Visani M, Sabbatani S, Pontoriero L, Fabbri C, Cuppini A, Pession A, Jovine E
World J Gastroenterol 2015 Dec 7;21(45):12896-953. doi: 10.3748/wjg.v21.i45.12896. PMID: 26668515Free PMC Article
A systematic review of photodynamic therapy in the treatment of pre-cancerous skin conditions, Barrett's oesophagus and cancers of the biliary tract, brain, head and neck, lung, oesophagus and skin.
Fayter D, Corbett M, Heirs M, Fox D, Eastwood A
Health Technol Assess 2010 Jul;14(37):1-288. doi: 10.3310/hta14370. PMID: 20663420

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