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Adenocarcinoma of liver and intrahepatic biliary tract

MedGen UID:
1675981
Concept ID:
C5192429
Neoplastic Process
Synonyms: Adenocarcinoma of the liver and IBT; Adenocarcinoma of the liver and intrahepatic biliary tract
SNOMED CT: Adenocarcinoma of liver and intrahepatic biliary tract (787091002)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Orphanet: ORPHA424943

Definition

A very rare hepatic and biliary tract neoplasm with a growth pattern resembling that found in hepatocellular carcinomas and cholangiocarcinomas but presenting atypical histological and immunohistochemical features (such as trabecular, organoid, microcystic and/or blastemal-like architecture and inhibin A, cytokeratin 7 and/or cytokeratin 19 positivity) that do not allow a formal diagnosis of the more common aforementioned liver cancers. Patients may present abdominal distension and pain, a palpable abdominal mass and elevated liver enzymes. [from SNOMEDCT_US]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdenocarcinoma of liver and intrahepatic biliary tract

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
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
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

Recent clinical studies

Etiology

Vogel A, Bridgewater J, Edeline J, Kelley RK, Klümpen HJ, Malka D, Primrose JN, Rimassa L, Stenzinger A, Valle JW, Ducreux M; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2023 Feb;34(2):127-140. Epub 2022 Nov 10 doi: 10.1016/j.annonc.2022.10.506. PMID: 36372281
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
Brindley PJ, Bachini M, Ilyas SI, Khan SA, Loukas A, Sirica AE, Teh BT, Wongkham S, Gores GJ
Nat Rev Dis Primers 2021 Sep 9;7(1):65. doi: 10.1038/s41572-021-00300-2. PMID: 34504109Free PMC Article
Sarcognato S, Sacchi D, Fassan M, Fabris L, Cadamuro M, Zanus G, Cataldo I, Capelli P, Baciorri F, Cacciatore M, Guido M
Pathologica 2021 Jun;113(3):158-169. doi: 10.32074/1591-951X-252. PMID: 34294934Free PMC Article
Soares KC, Jarnagin WR
Ann Surg Oncol 2021 Aug;28(8):4158-4170. Epub 2021 Apr 7 doi: 10.1245/s10434-021-09871-6. PMID: 33829358Free PMC Article

Diagnosis

Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
Lamarca A, Edeline J, Goyal L
ESMO Open 2022 Feb;7(1):100378. Epub 2022 Jan 13 doi: 10.1016/j.esmoop.2021.100378. PMID: 35032765Free PMC Article
Brindley PJ, Bachini M, Ilyas SI, Khan SA, Loukas A, Sirica AE, Teh BT, Wongkham S, Gores GJ
Nat Rev Dis Primers 2021 Sep 9;7(1):65. doi: 10.1038/s41572-021-00300-2. PMID: 34504109Free PMC Article
Sarcognato S, Sacchi D, Fassan M, Fabris L, Cadamuro M, Zanus G, Cataldo I, Capelli P, Baciorri F, Cacciatore M, Guido M
Pathologica 2021 Jun;113(3):158-169. doi: 10.32074/1591-951X-252. PMID: 34294934Free PMC Article
Blechacz B, Komuta M, Roskams T, Gores GJ
Nat Rev Gastroenterol Hepatol 2011 Aug 2;8(9):512-22. doi: 10.1038/nrgastro.2011.131. PMID: 21808282Free PMC Article

Therapy

Merters J, Lamarca A
J Hepatol 2023 Mar;78(3):652-657. Epub 2022 Nov 15 doi: 10.1016/j.jhep.2022.11.005. PMID: 36400328
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
Vogel A, Segatto O, Stenzinger A, Saborowski A
Annu Rev Med 2023 Jan 27;74:293-306. Epub 2022 Sep 28 doi: 10.1146/annurev-med-042921-024707. PMID: 36170665
Soares KC, Jarnagin WR
Ann Surg Oncol 2021 Aug;28(8):4158-4170. Epub 2021 Apr 7 doi: 10.1245/s10434-021-09871-6. PMID: 33829358Free PMC Article
Ma L, Hernandez MO, Zhao Y, Mehta M, Tran B, Kelly M, Rae Z, Hernandez JM, Davis JL, Martin SP, Kleiner DE, Hewitt SM, Ylaya K, Wood BJ, Greten TF, Wang XW
Cancer Cell 2019 Oct 14;36(4):418-430.e6. Epub 2019 Oct 3 doi: 10.1016/j.ccell.2019.08.007. PMID: 31588021Free PMC Article

Prognosis

Vogel A, Bridgewater J, Edeline J, Kelley RK, Klümpen HJ, Malka D, Primrose JN, Rimassa L, Stenzinger A, Valle JW, Ducreux M; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2023 Feb;34(2):127-140. Epub 2022 Nov 10 doi: 10.1016/j.annonc.2022.10.506. PMID: 36372281
Elvevi A, Laffusa A, Scaravaglio M, Rossi RE, Longarini R, Stagno AM, Cristoferi L, Ciaccio A, Cortinovis DL, Invernizzi P, Massironi S
Ann Hepatol 2022 Sep-Oct;27(5):100737. Epub 2022 Jul 7 doi: 10.1016/j.aohep.2022.100737. PMID: 35809836
Khan SA, Tavolari S, Brandi G
Liver Int 2019 May;39 Suppl 1:19-31. Epub 2019 Mar 24 doi: 10.1111/liv.14095. PMID: 30851228
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
Blechacz B, Komuta M, Roskams T, Gores GJ
Nat Rev Gastroenterol Hepatol 2011 Aug 2;8(9):512-22. doi: 10.1038/nrgastro.2011.131. PMID: 21808282Free PMC Article

Clinical prediction guides

Shang T, Jiang T, Lu T, Wang H, Cui X, Pan Y, Xu M, Pei M, Ding Z, Feng X, Lin Y, Li X, Tan Y, Feng F, Dong H, Wang H, Dong L
Front Immunol 2023;14:1166497. Epub 2023 May 10 doi: 10.3389/fimmu.2023.1166497. PMID: 37234171Free PMC Article
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
Alvisi G, Termanini A, Soldani C, Portale F, Carriero R, Pilipow K, Costa G, Polidoro M, Franceschini B, Malenica I, Puccio S, Lise V, Galletti G, Zanon V, Colombo FS, De Simone G, Tufano M, Aghemo A, Di Tommaso L, Peano C, Cibella J, Iannacone M, Roychoudhuri R, Manzo T, Donadon M, Torzilli G, Kunderfranco P, Di Mitri D, Lugli E, Lleo A
J Hepatol 2022 Nov;77(5):1359-1372. Epub 2022 Jun 20 doi: 10.1016/j.jhep.2022.05.043. PMID: 35738508
Prokopič M, Beuers U
Hepatol Int 2021 Feb;15(1):6-20. Epub 2020 Dec 30 doi: 10.1007/s12072-020-10118-x. PMID: 33377990Free PMC Article
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

Recent systematic reviews

Yeow M, Fong KY, Zhao JJ, Tan AYH, Koh YX, Kam JH, Goh BKP, Pawlik TM, Kabir T
HPB (Oxford) 2024 Feb;26(2):161-170. Epub 2023 Nov 20 doi: 10.1016/j.hpb.2023.11.007. PMID: 38071187
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
Gao YX, Yang TW, Yin JM, Yang PX, Kou BX, Chai MY, Liu XN, Chen DX
Int J Oncol 2020 Jul;57(1):54-66. Epub 2020 Apr 1 doi: 10.3892/ijo.2020.5035. PMID: 32236573
Clements O, Eliahoo J, Kim JU, Taylor-Robinson SD, Khan SA
J Hepatol 2020 Jan;72(1):95-103. Epub 2019 Sep 16 doi: 10.1016/j.jhep.2019.09.007. PMID: 31536748
Abbas S, Sandroussi C
HPB (Oxford) 2013 Jul;15(7):492-503. Epub 2013 Jan 8 doi: 10.1111/j.1477-2574.2012.00616.x. PMID: 23750491Free PMC Article

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