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Combined hepatocellular carcinoma and cholangiocarcinoma

MedGen UID:
113160
Concept ID:
C0221287
Neoplastic Process
Synonyms: Carcinoma of Liver and Intrahepatic Biliary Tract; Carcinoma of the Liver and Intrahepatic Biliary Tract; carcinoma of the liver and intrahepatic biliary tract; cHCC-CC; Cholangiohepatoma; Combined HCC-CC; combined hepatocellular cancer and cholangiocarcinoma (bile duct cancer); combined hepatocellular cancer and intrahepatic bile duct cancer (cholangiocarcinoma); Combined Hepatocellular Carcinoma and Cholangiocarcinoma; combined hepatocellular carcinoma and cholangiocarcinoma; Combined hepatocellular-cholangiocarcinoma; Hepatocholangiocarcinoma; Liver and Intrahepatic Biliary Tract Carcinoma; Mixed hepatocellular and bile duct carcinoma; Mixed Hepatocellular Cholangiocarcinoma; Mixed hepatocellular cholangiocarcinoma
SNOMED CT: Mixed hepatocellular and bile duct carcinoma (274902006); Hepatocholangiocarcinoma (274902006); Combined hepatocellular carcinoma and cholangiocarcinoma (274902006); Combined hepatocellular carcinoma and cholangiocarcinoma (52178006); Mixed hepatocellular and bile duct carcinoma (52178006); Hepatocholangiocarcinoma (52178006)
 
Monarch Initiative: MONDO:0044791
Orphanet: ORPHA529852

Definition

A rare hepatic tumor characterized by the presence of both hepatocytic and cholangiocytic differentiation within a primary liver carcinoma. The lesion commonly arises in the context of chronic liver disease (such as hepatitis B or C, or steatohepatitis) or exposure to a variety of exogenous agents. Patients may present with signs and symptoms related to the tumor, as well as to the underlying condition. Typical manifestations include right upper quadrant abdominal pain, weight loss, hepatosplenomegaly, jaundice, and ascites. The entity has been associated with a worse prognosis than hepatocellular carcinoma after resection. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCombined hepatocellular carcinoma and cholangiocarcinoma

Professional guidelines

PubMed

Weber SM, Ribero D, O'Reilly EM, Kokudo N, Miyazaki M, Pawlik TM
HPB (Oxford) 2015 Aug;17(8):669-80. doi: 10.1111/hpb.12441. PMID: 26172134Free PMC Article
Yin X, Zhang BH, Qiu SJ, Ren ZG, Zhou J, Chen XH, Zhou Y, Fan J
Ann Surg Oncol 2012 Sep;19(9):2869-76. Epub 2012 Mar 27 doi: 10.1245/s10434-012-2328-0. PMID: 22451237

Recent clinical studies

Etiology

Shen YT, Yue WW, Xu HX
Abdom Radiol (NY) 2023 Jun;48(6):2019-2037. Epub 2023 Mar 24 doi: 10.1007/s00261-023-03879-0. PMID: 36961531
Hou GM, Jiang C, Du JP, Yuan KF
Cancer Med 2022 Jan;11(2):317-331. Epub 2021 Dec 5 doi: 10.1002/cam4.4448. PMID: 34866356Free PMC Article
Wege H, Schulze K, von Felden J, Calderaro J, Reig M; rare liver tumors working group of the European Reference Network on Hepatological Diseases (ERN RARE-LIVER)
Eur J Med Genet 2021 Nov;64(11):104313. Epub 2021 Aug 18 doi: 10.1016/j.ejmg.2021.104313. PMID: 34418585
Wu CH, Yong CC, Liew EH, Tsang LL, Lazo M, Hsu HW, Ou HY, Yu CY, Chen TY, Huang TL, Concejero AM, Chen CL, Cheng YF
Transplant Proc 2016 May;48(4):1100-4. doi: 10.1016/j.transproceed.2015.11.029. PMID: 27320566
Yoon YI, Hwang S, Lee YJ, Kim KH, Ahn CS, Moon DB, Ha TY, Song GW, Jung DH, Lee JW, Hong SM, Yu ES, Lee SG
J Gastrointest Surg 2016 Feb;20(2):411-20. Epub 2015 Dec 1 doi: 10.1007/s11605-015-3045-3. PMID: 26628072

Diagnosis

Shen YT, Yue WW, Xu HX
Abdom Radiol (NY) 2023 Jun;48(6):2019-2037. Epub 2023 Mar 24 doi: 10.1007/s00261-023-03879-0. PMID: 36961531
Tang YY, Zhao YN, Zhang T, Chen ZY, Ma XL
World J Gastroenterol 2021 Nov 7;27(41):7173-7189. doi: 10.3748/wjg.v27.i41.7173. PMID: 34887636Free PMC Article
Wege H, Schulze K, von Felden J, Calderaro J, Reig M; rare liver tumors working group of the European Reference Network on Hepatological Diseases (ERN RARE-LIVER)
Eur J Med Genet 2021 Nov;64(11):104313. Epub 2021 Aug 18 doi: 10.1016/j.ejmg.2021.104313. PMID: 34418585
Wu CH, Yong CC, Liew EH, Tsang LL, Lazo M, Hsu HW, Ou HY, Yu CY, Chen TY, Huang TL, Concejero AM, Chen CL, Cheng YF
Transplant Proc 2016 May;48(4):1100-4. doi: 10.1016/j.transproceed.2015.11.029. PMID: 27320566
Yin X, Zhang BH, Qiu SJ, Ren ZG, Zhou J, Chen XH, Zhou Y, Fan J
Ann Surg Oncol 2012 Sep;19(9):2869-76. Epub 2012 Mar 27 doi: 10.1245/s10434-012-2328-0. PMID: 22451237

Therapy

Tang YY, Zhao YN, Zhang T, Chen ZY, Ma XL
World J Gastroenterol 2021 Nov 7;27(41):7173-7189. doi: 10.3748/wjg.v27.i41.7173. PMID: 34887636Free PMC Article
Liu WR, Tian MX, Tao CY, Tang Z, Zhou YF, Song SS, Jiang XF, Wang H, Zhou PY, Qu WF, Fang Y, Ding ZB, Zhou J, Fan J, Shi YH
BMC Cancer 2020 Jul 10;20(1):642. doi: 10.1186/s12885-020-07138-z. PMID: 32650743Free PMC Article
Weber SM, Ribero D, O'Reilly EM, Kokudo N, Miyazaki M, Pawlik TM
HPB (Oxford) 2015 Aug;17(8):669-80. doi: 10.1111/hpb.12441. PMID: 26172134Free PMC Article
Noh SJ, Lee HK, Yu HC, Moon WS
Clin Mol Hepatol 2012 Dec;18(4):416-9. Epub 2012 Dec 21 doi: 10.3350/cmh.2012.18.4.416. PMID: 23323259Free PMC Article
Kim JH, Yoon HK, Ko GY, Gwon DI, Jang CS, Song HY, Shin JH, Sung KB
Radiology 2010 Apr;255(1):270-7. doi: 10.1148/radiol.09091076. PMID: 20308463

Prognosis

Shen YT, Yue WW, Xu HX
Abdom Radiol (NY) 2023 Jun;48(6):2019-2037. Epub 2023 Mar 24 doi: 10.1007/s00261-023-03879-0. PMID: 36961531
Hou GM, Jiang C, Du JP, Yuan KF
Cancer Med 2022 Jan;11(2):317-331. Epub 2021 Dec 5 doi: 10.1002/cam4.4448. PMID: 34866356Free PMC Article
Jung DH, Hwang S, Hong SM, Chung YK, Song GW, Lee YJ, Kim KH, Ahn CS, Moon DB, Ha TY, Park GC, Lee HC, Lim YS, Yu ES, Lee SG
World J Surg 2017 May;41(5):1347-1357. doi: 10.1007/s00268-016-3837-y. PMID: 27896409
Sasaki M, Sato Y, Nakanuma Y
Histopathology 2017 Feb;70(3):423-434. Epub 2016 Nov 15 doi: 10.1111/his.13084. PMID: 27634656
Wu CH, Yong CC, Liew EH, Tsang LL, Lazo M, Hsu HW, Ou HY, Yu CY, Chen TY, Huang TL, Concejero AM, Chen CL, Cheng YF
Transplant Proc 2016 May;48(4):1100-4. doi: 10.1016/j.transproceed.2015.11.029. PMID: 27320566

Clinical prediction guides

Hou GM, Jiang C, Du JP, Yuan KF
Cancer Med 2022 Jan;11(2):317-331. Epub 2021 Dec 5 doi: 10.1002/cam4.4448. PMID: 34866356Free PMC Article
Jung DH, Hwang S, Hong SM, Chung YK, Song GW, Lee YJ, Kim KH, Ahn CS, Moon DB, Ha TY, Park GC, Lee HC, Lim YS, Yu ES, Lee SG
World J Surg 2017 May;41(5):1347-1357. doi: 10.1007/s00268-016-3837-y. PMID: 27896409
Sasaki M, Sato Y, Nakanuma Y
Histopathology 2017 Feb;70(3):423-434. Epub 2016 Nov 15 doi: 10.1111/his.13084. PMID: 27634656
Yoon YI, Hwang S, Lee YJ, Kim KH, Ahn CS, Moon DB, Ha TY, Song GW, Jung DH, Lee JW, Hong SM, Yu ES, Lee SG
J Gastrointest Surg 2016 Feb;20(2):411-20. Epub 2015 Dec 1 doi: 10.1007/s11605-015-3045-3. PMID: 26628072
Lee SD, Park SJ, Han SS, Kim SH, Kim YK, Lee SA, Ko YH, Hong EK
Hepatobiliary Pancreat Dis Int 2014 Dec;13(6):594-601. doi: 10.1016/s1499-3872(14)60275-7. PMID: 25475861

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