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Undifferentiated carcinoma of liver and intrahepatic biliary tract

MedGen UID:
1656437
Concept ID:
C4749401
Neoplastic Process
Synonyms: Undifferentiated carcinoma of liver and IBT; undifferentiated carcinoma of liver and IBT; undifferentiated carcinoma of liver and intrahepatic biliary tract
SNOMED CT: Undifferentiated carcinoma of liver and intrahepatic biliary tract (770685009)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Monarch Initiative: MONDO:0018533
Orphanet: ORPHA424970

Definition

Undifferentiated carcinoma of liver and intrahepatic biliary tract is an extremely rare epithelial tumor of the liver and biliary tract which presents heterogenous histological findings and not yet fully defined clinicopathological characterisitcs. Patients usually present with nonspecific signs and symptoms, such as abdominal pain, nausea, vomiting, anorexia, weight loss and/or jaundice. Invasive growth, hight metastatic potential and a rapid clinical course are typically associated. [from ORDO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUndifferentiated carcinoma 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

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
Al-Rajabi R, Sun W
Curr Opin Gastroenterol 2021 Mar 1;37(2):105-111. doi: 10.1097/MOG.0000000000000715. PMID: 33507028

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

Pinter M, Scheiner B, Pinato DJ
Lancet Gastroenterol Hepatol 2023 Aug;8(8):760-770. Epub 2023 Jun 14 doi: 10.1016/S2468-1253(23)00147-4. PMID: 37327807
Ma J, Li J, Jin C, Yang J, Zheng C, Chen K, Xie Y, Yang Y, Bo Z, Wang J, Su Q, Wang J, Chen G, Wang Y
Liver Int 2023 Jan;43(1):221-233. Epub 2022 Nov 8 doi: 10.1111/liv.15466. PMID: 36300678
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
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

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
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
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

Zhou Y, Gu Q, Zhu L, Zhang S, Wu H, Pu X, Jiang C, Chen J
Cell Prolif 2023 Dec;56(12):e13513. Epub 2023 Jul 3 doi: 10.1111/cpr.13513. PMID: 37401015Free PMC Article
Liu S, Fan S, Wang Y, Chen R, Wang Z, Zhang Y, Jiang W, Chen Y, Xu X, Yu Y, Li C, Li X
BMC Cancer 2023 May 16;23(1):444. doi: 10.1186/s12885-023-10903-5. PMID: 37193981Free PMC Article
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
Nguyen MLT, Toan NL, Bozko M, Bui KC, Bozko P
Curr Cancer Drug Targets 2021;21(6):457-475. doi: 10.2174/1568009621666210204152028. PMID: 33563168
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

Hu YF, Hu HJ, Ma WJ, Jin YW, Li FY
Updates Surg 2023 Dec;75(8):2049-2061. Epub 2023 Nov 2 doi: 10.1007/s13304-023-01648-8. PMID: 37919559Free PMC Article
Asombang AW, Chishinga N, Mohamed MF, Nkhoma A, Chipaila J, Nsokolo B, Manda-Mapalo M, Montiero JFG, Banda L, Dua KS
BMC Gastroenterol 2023 Mar 11;23(1):66. doi: 10.1186/s12876-023-02687-6. PMID: 36906562Free PMC Article
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
Al-Adra DP, Gill RS, Axford SJ, Shi X, Kneteman N, Liau SS
Eur J Surg Oncol 2015 Jan;41(1):120-7. Epub 2014 Oct 7 doi: 10.1016/j.ejso.2014.09.007. PMID: 25449754Free PMC Article
Mavros MN, Economopoulos KP, Alexiou VG, Pawlik TM
JAMA Surg 2014 Jun;149(6):565-74. doi: 10.1001/jamasurg.2013.5137. PMID: 24718873

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