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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

Comprehensive Bioinformatic Investigation of TP53 Dysregulation in Diverse Cancer Landscapes.
Khan R, Pari B, Puszynski K
Genes (Basel) 2024 Apr 30;15(5) doi: 10.3390/genes15050577. PMID: 38790205Free PMC Article
Systematic pan-cancer analyses of the potential function of the Golgi scaffold protein PAQR3.
Ling ZN, Hong LL, Wu J, Ling ZQ
Sci Rep 2024 Feb 6;14(1):3030. doi: 10.1038/s41598-024-53489-z. PMID: 38321173Free PMC Article
Clonorchis sinensis infection amplifies hepatocellular carcinoma stemness, predicting unfavorable prognosis.
Lin Q, Tang Z, Qin Y, Deng X, Wei C, Liu F, Pan X, Liu D, Zhan T, Fang M
PLoS Negl Trop Dis 2024 Jan;18(1):e0011906. Epub 2024 Jan 29 doi: 10.1371/journal.pntd.0011906. PMID: 38285640Free 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
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

Diagnosis

Systematic pan-cancer analyses of the potential function of the Golgi scaffold protein PAQR3.
Ling ZN, Hong LL, Wu J, Ling ZQ
Sci Rep 2024 Feb 6;14(1):3030. doi: 10.1038/s41598-024-53489-z. PMID: 38321173Free PMC Article
Enteroblastic cholangiocarcinoma: An uncommon, underrecognized subtype of bile duct cancer.
Chun J, Moore M, Kelly P, Kanzawa M, Itoh T, Hong SM, Zen Y
Hum Pathol 2024 Feb;144:46-52. Epub 2024 Jan 30 doi: 10.1016/j.humpath.2024.01.011. PMID: 38301963
N-cadherin: A diagnostic marker to help discriminate primary liver carcinomas from extrahepatic carcinomas.
Gerber TS, Ridder DA, Goeppert B, Brobeil A, Stenzel P, Zimmer S, Jäkel J, Metzig MO, Schwab R, Martin SZ, Kiss A, Bergmann F, Schirmacher P, Galle PR, Lang H, Roth W, Straub BK
Int J Cancer 2024 May 15;154(10):1857-1868. Epub 2024 Jan 11 doi: 10.1002/ijc.34836. PMID: 38212892
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

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

Comprehensive Bioinformatic Investigation of TP53 Dysregulation in Diverse Cancer Landscapes.
Khan R, Pari B, Puszynski K
Genes (Basel) 2024 Apr 30;15(5) doi: 10.3390/genes15050577. PMID: 38790205Free 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
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

Clinical prediction guides

Comprehensive Bioinformatic Investigation of TP53 Dysregulation in Diverse Cancer Landscapes.
Khan R, Pari B, Puszynski K
Genes (Basel) 2024 Apr 30;15(5) doi: 10.3390/genes15050577. PMID: 38790205Free 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
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
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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