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Malignant Hepatobiliary Neoplasm

MedGen UID:: 167699
•Concept ID:: C0854176
•: Neoplastic Process

Synonyms:

Malignant Hepato-Biliary Neoplasm; Malignant Liver and Biliary Neoplasm; Malignant Liver and Biliary System Neoplasm

Definition

A malignant neoplasm that affects the liver parenchyma, bile ducts, and gallbladder. Representative examples include hepatocellular carcinoma, intrahepatic and extrahepatic cholangiocarcinoma, and gallbladder carcinoma. [from NCI]

Term Hierarchy

GTR
MeSH

CClinical test, RResearch test, OOMIM, GGeneReviews, VClinVar

CROGVMalignant Hepatobiliary Neoplasm

Pathological process
- Disease
  - Disorder by Site
    - Disorder of gastrointestinal tract
      - Hepatobiliary disorder
        Hepatobiliary neoplasm
        Malignant Hepatobiliary Neoplasm
        Biliary Tract Carcinoma
        Bile duct carcinoma
        Bile duct adenocarcinoma
        Extrahepatic bile duct carcinoma
        Metastatic Bile Duct Carcinoma
        Recurrent Bile Duct Carcinoma
        Refractory Bile Duct Carcinoma
        Unresectable Bile Duct Carcinoma
        Gallbladder carcinoma
        Gallbladder adenocarcinoma
        Gallbladder adenosquamous carcinoma
        Gallbladder Cancer by AJCC v6 Stage
        Gallbladder Cancer by AJCC v7 Stage
        Gallbladder Cancer by AJCC v8 Stage
        Gallbladder Carcinosarcoma
        Gallbladder Mixed Adenoneuroendocrine Carcinoma
        Gallbladder Neuroendocrine Carcinoma
        Gallbladder squamous cell carcinoma
        Incidental Gallbladder Carcinoma
        Localized Gallbladder Carcinoma
        Metastatic Gallbladder Carcinoma
        Recurrent Gallbladder Carcinoma
        Refractory Gallbladder Carcinoma
        Undifferentiated gallbladder carcinoma
        Unresectable Gallbladder Carcinoma
        High-Grade Biliary Intraepithelial Neoplasia
        Metastatic Biliary Tract Carcinoma
        Advanced Biliary Tract Carcinoma
        Locally Advanced Biliary Tract Carcinoma
        Recurrent Biliary Tract Carcinoma
        Refractory Biliary Tract Carcinoma
        Unresectable Biliary Tract Carcinoma
        Gallbladder cancer
        Gallbladder lymphoma
        Gallbladder melanoma
        Gallbladder sarcoma
        Gallbladder angiosarcoma
        Gallbladder Kaposi Sarcoma
        Gallbladder leiomyosarcoma
        Gallbladder rhabdomyosarcoma
        Localized Malignant Gallbladder Neoplasm
        Metastatic Malignant Neoplasm in the Gallbladder
        Malignant tumor of extrahepatic bile duct
        Extrahepatic Bile Duct Lymphoma
        Extrahepatic bile duct sarcoma
        Extrahepatic Bile Duct Kaposi Sarcoma
        Extrahepatic bile duct leiomyosarcoma
        Extrahepatic bile duct rhabdomyosarcoma
        Metastatic Malignant Neoplasm in the Extrahepatic Bile Ducts
        Metastatic Malignant Neoplasm in the Liver
        Metastatic Carcinoma in the Liver
        Breast Carcinoma Metastatic in the Liver
        Colorectal Carcinoma Metastatic in the Liver
        Lung Carcinoma Metastatic in the Liver
        Uveal Melanoma Metastatic in the Liver
        Primary Malignant Liver Neoplasm
        Carcinoma of liver and intrahepatic biliary tract
        Adenocarcinoma of the liver and intrahepatic biliary tract
        Adult Liver Carcinoma
        Biliary cystadenocarcinoma
        Cholangiocarcinoma
        Combined hepatocellular carcinoma and cholangiocarcinoma
        Fibrolamellar hepatocellular carcinoma
        Hepatocellular carcinoma
        Intrahepatic Bile Duct Cancer by AJCC v8 Stage
        Intrahepatic cholangiocarcinoma
        Liver adenosquamous carcinoma
        Liver Carcinosarcoma
        Liver Mixed Adenoneuroendocrine Carcinoma
        Liver neuroendocrine carcinoma
        Liver Undifferentiated Carcinoma
        Metastatic Liver Carcinoma
        Recurrent Liver Carcinoma
        Refractory Liver Carcinoma
        Resectable Liver and Intrahepatic Bile Duct Carcinoma
        Squamous cell carcinoma of liver and intrahepatic biliary tract
        Stage I Liver Cancer
        Stage II Liver Cancer
        Stage III Liver Cancer
        Stage IV Liver Cancer
        Undifferentiated carcinoma of liver and intrahepatic biliary tract
        Unresectable Liver and Intrahepatic Bile Duct Carcinoma
        Childhood Malignant Liver Neoplasm
        Calcifying nested epithelial stromal tumor of the liver
        Extrarenal Rhabdoid Tumor of the Liver
        Hepatoblastoma
        Hepatocellular Malignant Neoplasm, Not Otherwise Specified
        Pediatric hepatocellular carcinoma
        Recurrent Childhood Malignant Liver Neoplasm
        Malignant Liver Non-Epithelial Neoplasm
        Liver Epithelioid Hemangioendothelioma
        Liver lymphoma
        Liver sarcoma
        Liver Yolk Sac Tumor
        Resectable Malignant Liver Neoplasm
        Resectable Hepatoblastoma
        Unresectable Malignant Liver Neoplasm
        Unresectable Hepatoblastoma

Professional guidelines

PubMed

International evidence-based Kyoto guidelines for the management of intraductal papillary mucinous neoplasm of the pancreas.

Ohtsuka T, Fernandez-Del Castillo C, Furukawa T, Hijioka S, Jang JY, Lennon AM, Miyasaka Y, Ohno E, Salvia R, Wolfgang CL, Wood LD
Pancreatology 2024 Mar;24(2):255-270. Epub 2023 Dec 28 doi: 10.1016/j.pan.2023.12.009. PMID: 38182527

Pancreatic cancer: A review of clinical diagnosis, epidemiology, treatment and outcomes.

McGuigan A, Kelly P, Turkington RC, Jones C, Coleman HG, McCain RS
World J Gastroenterol 2018 Nov 21;24(43):4846-4861. doi: 10.3748/wjg.v24.i43.4846. PMID: 30487695 Free PMC Article

Revisions of international consensus Fukuoka guidelines for the management of IPMN of the pancreas.

Tanaka M, Fernández-Del Castillo C, Kamisawa T, Jang JY, Levy P, Ohtsuka T, Salvia R, Shimizu Y, Tada M, Wolfgang CL
Pancreatology 2017 Sep-Oct;17(5):738-753. Epub 2017 Jul 13 doi: 10.1016/j.pan.2017.07.007. PMID: 28735806

See all (1402)

Recent clinical studies

Etiology

Imaging of Hepatobiliary Cancer.

Carney BW, Larson MC, Corwin MT, Lamba R
Curr Probl Cancer 2023 Apr;47(2):100964. Epub 2023 Jun 4 doi: 10.1016/j.currproblcancer.2023.100964. PMID: 37321910

Surgical Treatments of Hepatobiliary Cancers.

Gunasekaran G, Bekki Y, Lourdusamy V, Schwartz M
Hepatology 2021 Jan;73 Suppl 1:128-136. Epub 2020 Nov 26 doi: 10.1002/hep.31325. PMID: 32438491

Extracellular Vesicles in Hepatobiliary Malignancies.

Lapitz A, Arbelaiz A, Olaizola P, Aranburu A, Bujanda L, Perugorria MJ, Banales JM
Front Immunol 2018;9:2270. Epub 2018 Oct 12 doi: 10.3389/fimmu.2018.02270. PMID: 30369925 Free PMC Article

Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.

Norton JA, Foster DS, Blumgart LH, Poultsides GA, Visser BC, Fraker DL, Alexander HR, Jensen RT
JAMA Surg 2018 Mar 1;153(3):e175083. doi: 10.1001/jamasurg.2017.5083. PMID: 29365025 Free PMC Article

The clinical significance of elevated levels of serum CA 19-9.

Pavai S, Yap SF
Med J Malaysia 2003 Dec;58(5):667-72. PMID: 15190651

See all (11717)

Diagnosis

Advances in targeted therapy of cholangiocarcinoma.

Li Y, Yu J, Zhang Y, Peng C, Song Y, Liu S
Ann Med 2024 Dec;56(1):2310196. Epub 2024 Feb 15 doi: 10.1080/07853890.2024.2310196. PMID: 38359439 Free PMC Article

Liquid biopsy techniques and pancreatic cancer: diagnosis, monitoring, and evaluation.

Wang K, Wang X, Pan Q, Zhao B
Mol Cancer 2023 Oct 6;22(1):167. doi: 10.1186/s12943-023-01870-3. PMID: 37803304 Free PMC Article

Extracellular Vesicles in Hepatobiliary Malignancies.

Lapitz A, Arbelaiz A, Olaizola P, Aranburu A, Bujanda L, Perugorria MJ, Banales JM
Front Immunol 2018;9:2270. Epub 2018 Oct 12 doi: 10.3389/fimmu.2018.02270. PMID: 30369925 Free PMC Article

Incidence and Prognosis of Primary Gastrinomas in the Hepatobiliary Tract.

Norton JA, Foster DS, Blumgart LH, Poultsides GA, Visser BC, Fraker DL, Alexander HR, Jensen RT
JAMA Surg 2018 Mar 1;153(3):e175083. doi: 10.1001/jamasurg.2017.5083. PMID: 29365025 Free PMC Article

The clinical significance of elevated levels of serum CA 19-9.

Pavai S, Yap SF
Med J Malaysia 2003 Dec;58(5):667-72. PMID: 15190651

See all (8448)

Therapy

Lynch Syndrome and MSI-H Cancers: From Mechanisms to "Off-The-Shelf" Cancer Vaccines.

Roudko V, Cimen Bozkus C, Greenbaum B, Lucas A, Samstein R, Bhardwaj N
Front Immunol 2021;12:757804. Epub 2021 Sep 24 doi: 10.3389/fimmu.2021.757804. PMID: 34630437 Free PMC Article

Targeting cancer-promoting inflammation - have anti-inflammatory therapies come of age?

Hou J, Karin M, Sun B
Nat Rev Clin Oncol 2021 May;18(5):261-279. Epub 2021 Jan 19 doi: 10.1038/s41571-020-00459-9. PMID: 33469195 Free PMC Article

Systemic therapies for intrahepatic cholangiocarcinoma.

Kelley RK, Bridgewater J, Gores GJ, Zhu AX
J Hepatol 2020 Feb;72(2):353-363. doi: 10.1016/j.jhep.2019.10.009. PMID: 31954497

Surgery for cholangiocarcinoma.

Cillo U, Fondevila C, Donadon M, Gringeri E, Mocchegiani F, Schlitt HJ, Ijzermans JNM, Vivarelli M, Zieniewicz K, Olde Damink SWM, Groot Koerkamp B
Liver Int 2019 May;39 Suppl 1(Suppl Suppl 1):143-155. doi: 10.1111/liv.14089. PMID: 30843343 Free PMC Article

Immune checkpoint therapy in liver cancer.

Xu F, Jin T, Zhu Y, Dai C
J Exp Clin Cancer Res 2018 May 29;37(1):110. doi: 10.1186/s13046-018-0777-4. PMID: 29843754 Free PMC Article

See all (6402)

Prognosis

Gut microbiome is associated with the clinical response to anti-PD-1 based immunotherapy in hepatobiliary cancers.

Mao J, Wang D, Long J, Yang X, Lin J, Song Y, Xie F, Xun Z, Wang Y, Wang Y, Li Y, Sun H, Xue J, Song Y, Zuo B, Zhang J, Bian J, Zhang T, Yang X, Zhang L, Sang X, Zhao H
J Immunother Cancer 2021 Dec;9(12) doi: 10.1136/jitc-2021-003334. PMID: 34873013 Free PMC Article

Liver transplantation in hepatocellular carcinoma after tumour downstaging (XXL): a randomised, controlled, phase 2b/3 trial.

Mazzaferro V, Citterio D, Bhoori S, Bongini M, Miceli R, De Carlis L, Colledan M, Salizzoni M, Romagnoli R, Antonelli B, Vivarelli M, Tisone G, Rossi M, Gruttadauria S, Di Sandro S, De Carlis R, Lucà MG, De Giorgio M, Mirabella S, Belli L, Fagiuoli S, Martini S, Iavarone M, Svegliati Baroni G, Angelico M, Ginanni Corradini S, Volpes R, Mariani L, Regalia E, Flores M, Droz Dit Busset M, Sposito C
Lancet Oncol 2020 Jul;21(7):947-956. doi: 10.1016/S1470-2045(20)30224-2. PMID: 32615109

Pancreatic cancer: A review of clinical diagnosis, epidemiology, treatment and outcomes.

McGuigan A, Kelly P, Turkington RC, Jones C, Coleman HG, McCain RS
World J Gastroenterol 2018 Nov 21;24(43):4846-4861. doi: 10.3748/wjg.v24.i43.4846. PMID: 30487695 Free PMC Article

Epidemiology of cholangiocarcinoma.

Bergquist A, von Seth E
Best Pract Res Clin Gastroenterol 2015 Apr;29(2):221-32. Epub 2015 Feb 16 doi: 10.1016/j.bpg.2015.02.003. PMID: 25966423

Pathogenesis, diagnosis, and management of cholangiocarcinoma.

Ilyas SI, Gores GJ
Gastroenterology 2013 Dec;145(6):1215-29. Epub 2013 Oct 15 doi: 10.1053/j.gastro.2013.10.013. PMID: 24140396 Free PMC Article

See all (11642)

Clinical prediction guides

Development and Validation of a Model Including Distinct Vascular Patterns to Estimate Survival in Hepatocellular Carcinoma.

Lin WP, Xing KL, Fu JC, Ling YH, Li SH, Yu WS, Zhang YF, Zhong C, Wang JH, Chen ZY, Lu LH, Wei W, Guo RP
JAMA Netw Open 2021 Sep 1;4(9):e2125055. doi: 10.1001/jamanetworkopen.2021.25055. PMID: 34515782 Free PMC Article

Coming of a precision era of the staging systems for intrahepatic cholangiocarcinoma?

Cheng Z, Lei Z, Shen F
Cancer Lett 2019 Sep 28;460:10-17. Epub 2019 Jun 15 doi: 10.1016/j.canlet.2019.114426. PMID: 31212000

Extracellular Vesicles in Hepatobiliary Malignancies.

Lapitz A, Arbelaiz A, Olaizola P, Aranburu A, Bujanda L, Perugorria MJ, Banales JM
Front Immunol 2018;9:2270. Epub 2018 Oct 12 doi: 10.3389/fimmu.2018.02270. PMID: 30369925 Free PMC Article

Diagnosis of cholangiocarcinoma in primary sclerosing cholangitis.

Taghavi SA, Eshraghian A, Niknam R, Sivandzadeh GR, Bagheri Lankarani K
Expert Rev Gastroenterol Hepatol 2018 Jun;12(6):575-584. Epub 2018 May 21 doi: 10.1080/17474124.2018.1473761. PMID: 29781738

Clinical score for predicting recurrence after hepatic resection for metastatic colorectal cancer: analysis of 1001 consecutive cases.

Fong Y, Fortner J, Sun RL, Brennan MF, Blumgart LH
Ann Surg 1999 Sep;230(3):309-18; discussion 318-21. doi: 10.1097/00000658-199909000-00004. PMID: 10493478 Free PMC Article

See all (8770)

Recent systematic reviews

Circular RNA in cholangiocarcinoma: A systematic review and bibliometric analysis.

Zhou Z, Chen C, Han B, Wang Y, Liu Y, Liu Q, Xu X, Yin Y, Sun B
Pathol Res Pract 2023 Sep;249:154755. Epub 2023 Aug 9 doi: 10.1016/j.prp.2023.154755. PMID: 37651837

FOLFIRINOX or Gemcitabine-based Chemotherapy for Borderline Resectable and Locally Advanced Pancreatic Cancer: A Multi-institutional, Patient-Level, Meta-analysis and Systematic Review.

Eshmuminov D, Aminjonov B, Palm RF, Malleo G, Schmocker RK, Abdallah R, Yoo C, Shaib WL, Schneider MA, Rangelova E, Choi YJ, Kim H, Rose JB, Patel S, Wilson GC, Maloney S, Timmermann L, Sahora K, Rössler F, Lopez-Lopez V, Boyer E, Maggino L, Malinka T, Park JY, Katz MHG, Prakash L, Ahmad SA, Helton S, Jang JY, Hoffe SE, Salvia R, Taieb J, He J, Clavien PA, Held U, Lehmann K
Ann Surg Oncol 2023 Jul;30(7):4417-4428. Epub 2023 Apr 5 doi: 10.1245/s10434-023-13353-2. PMID: 37020094 Free PMC Article

The Impact of Prehabilitation on Patient Outcomes in Hepatobiliary, Colorectal, and Upper Gastrointestinal Cancer Surgery: A PRISMA-Accordant Meta-analysis.

Lambert JE, Hayes LD, Keegan TJ, Subar DA, Gaffney CJ
Ann Surg 2021 Jul 1;274(1):70-77. doi: 10.1097/SLA.0000000000004527. PMID: 33201129

Hepatotoxicity From Immune Checkpoint Inhibitors: A Systematic Review and Management Recommendation.

Peeraphatdit TB, Wang J, Odenwald MA, Hu S, Hart J, Charlton MR
Hepatology 2020 Jul;72(1):315-329. doi: 10.1002/hep.31227. PMID: 32167613

A systematic review of safety and efficacy of hepatopancreatoduodenectomy for biliary and gallbladder cancers.

Zhou Y, Zhang Z, Wu L, Li B
HPB (Oxford) 2016 Jan;18(1):1-6. Epub 2015 Nov 30 doi: 10.1016/j.hpb.2015.07.008. PMID: 26776844 Free PMC Article

See all (708)