CCA
MCID: CHL065
MIFTS: 73

Cholangiocarcinoma (CCA) malady

Liver diseases, Cancer diseases categories

Summaries for Cholangiocarcinoma

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63Wikipedia, 46OMIM, 32MalaCards
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Wikipedia:63 Cholangiocarcinoma is a form of cancer that is composed of mutated epithelial cells (or cells showing... more...

MalaCards: Cholangiocarcinoma, also known as adult primary cholangiocellular carcinoma, is related to hepatocellular carcinoma and hepatitis, and has symptoms including fever/chilling, hepatitis/icterus/cholestasis and pruritus/itching. An important gene associated with Cholangiocarcinoma is MUC2 (mucin 2, oligomeric mucus/gel-forming), and among its related pathways are Rho Family GTPases and PI3K-Akt signaling pathway. The compounds paraffin and tyrosine have been mentioned in the context of this disorder. Affiliated tissues include liver, small intestine and lymph node, and related mouse phenotypes are mortality/aging and cellular.

Description from OMIM:46 615619

Aliases & Classifications for Cholangiocarcinoma

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Sources:
60UMLS, 8Disease Ontology, 10DISEASES, 44Novoseek, 48Orphanet, 46OMIM, 56SNOMED-CT, 34MeSH, 39NCIt, 35MESH via Orphanet, 26ICD10 via Orphanet, 57SNOMED-CT via Orphanet, 61UMLS via Orphanet
See all sources

Classifications:

Malacards categories (disease lists): (See all malacards categories)
Global: Cancer diseases
Anatomical: Liver diseases


Characteristics (Orphanet epidemiological data):

48
cholangiocarcinoma:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

cholangiocarcinoma 8 10 44 48 46 60
adult primary cholangiocellular carcinoma 8 60
adult primary cholangiocarcinoma 8
bile duct carcinoma 60
cholangiosarcoma 8
bile duct cancer 48
cca 48


External Ids:

Disease Ontology8 DOID:4947
MeSH34 D018281
NCIt39 C4436
MESH via Orphanet35 D018281, D001650
SNOMED-CT56 70179006, 312104005
ICD10 via Orphanet26 C22.1
SNOMED-CT via Orphanet57 312104005, 70179006
UMLS via Orphanet61 C0206698, C0740277
OMIM46 615619

Related Diseases for Cholangiocarcinoma

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Sources:
17GeneCards, 18GeneDecks
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Diseases related to Cholangiocarcinoma via text searches within MalaCards or GeneCards/GeneDecks gene sharing:

(show top 50)    (show all 283)
idRelated DiseaseScoreTop Affiliating Genes
1hepatocellular carcinoma31.2CEACAM3, KRT7, KRT19, CFLAR, MCL1, CDKN1B
2hepatitis31.2AFP, HGF
3hepatitis b30.7AFP
4adenocarcinoma30.7CEACAM3, LGALS3, KRT20, KRT7, KRT19, CFLAR
5hepatitis c30.5AFP, TIMP3
6gallbladder cancer30.5CEACAM3, KRT7, CDKN1B, CDX2, MUC5AC, MUC2
7bladder carcinoma30.4KRT20, KRT19, MUC2, MAGEA3
8viral hepatitis30.4AFP
9adenoma30.3CEACAM3, LGALS3, KRT20, KRT7, KRT19, MCL1
10liver cirrhosis30.2AFP, HGF
11gastric adenocarcinoma30.2KRT20, KRT7, CFLAR, MCL1, CDX2, MUC4
12biliary papillomatosis30.2MUC2
13colon adenocarcinoma30.1LGALS3, KRT20, CFLAR, CDKN1B, MUC5AC, MUC2
14squamous cell carcinoma30.0CEACAM3, LGALS3, KRT20, KRT7, KRT19, CFLAR
15colorectal cancer30.0CEACAM3, LGALS3, KRT20, KRT7, CFLAR, CDKN1B
16colon cancer30.0CEACAM3, LGALS3, KRT20, KRT19, CFLAR, CDKN1B
17primary biliary cirrhosis29.9KRT7, KRT19, MCL1
18bile duct carcinoma29.9CEACAM3, MAGEA3
19breast cancer29.7CEACAM3, LGALS3, KRT20, KRT7, KRT19, MCL1
20lung cancer29.7CEACAM3, KRT20, KRT7, KRT19, CDX2, MUC4
21melanoma29.7CEACAM3, LGALS3, CFLAR, MCL1, CDKN1B, CDX2
22papillary carcinoma29.7CEACAM3, LGALS3, KRT20, KRT7, KRT19, CDKN1B
23pancreatic cancer29.7CEACAM3, LGALS3, KRT19, CDX2, MUC4, MUC1
24neuroendocrine carcinoma29.7KRT20, KRT7, KRT19, CDX2, MUC1
25sarcoma29.7CFLAR, MUC1, MAGEA3, MET
26cystadenocarcinoma29.7CEACAM3, MUC1, MUC5AC, MUC2
27cystadenoma29.7CEACAM3, KRT7, VEGFC
28non-hodgkin lymphoma29.7CFLAR, MCL1, CDKN1B, OGG1
29bile duct adenoma29.7KRT7, KRT19
30inflammatory bowel disease29.7MUC2
31spindle cell sarcoma29.7KRT7, MUC1
32lung squamous cell carcinoma29.7KRT19, TIMP3
33intrahepatic cholangiocarcinoma11.2
34cholangitis10.7
35sclerosing cholangitis10.7
36hepatitis a10.5
37bile duct carcinoma in situ10.5
38opisthorchiasis10.4
39pancreatitis10.4
40congenital contractural arachnodactyly10.4
41adenosquamous bile duct carcinoma10.4
42squamous cell bile duct carcinoma10.4
43mucinous intrahepatic cholangiocarcinoma10.4
44clonorchiasis10.4
45choledochal cyst10.3
46schistosomiasis10.3
47extrahepatic bile duct small cell adenocarcinoma10.3
48extrahepatic bile duct adenocarcinoma10.3
49bile duct adenocarcinoma10.3
50sarcomatous intrahepatic cholangiocarcinoma10.2

Graphical network of the top 20 diseases related to Cholangiocarcinoma:



Diseases related to cholangiocarcinoma

Clinical Features for Cholangiocarcinoma

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Sources:
46OMIM, 48Orphanet
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Clinical features from OMIM:

615619

Symptoms:

48 (show all 9)
  • fever/chilling
  • hepatitis/icterus/cholestasis
  • pruritus/itching
  • weight loss/loss of appetite/break in weight curve/general health alteration
  • acute abdominal pain/colic
  • anorexia
  • abnormal colour of the urine/cholic/dark urines
  • extrahepatic biliary tract/gallbladder neoplasm/tumor/carcinoma/cancer
  • asthenia/fatigue/weakness

Drugs & Therapeutics for Cholangiocarcinoma

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Sources:
5CenterWatch, 41NIH Clinical Center, 6ClinicalTrials, 60UMLS, 40NDF-RT
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Approved drugs:

Search CenterWatch for Cholangiocarcinoma

Drug clinical trials:

Search ClinicalTrials for Cholangiocarcinoma

Search NIH Clinical Center for Cholangiocarcinoma

Search CenterWatch for Cholangiocarcinoma

Genetic Tests for Cholangiocarcinoma

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Anatomical Context for Cholangiocarcinoma

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32MalaCards
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MalaCards organs/tissues related to Cholangiocarcinoma:

32
Liver, Small intestine, Lymph node, Colon, Endothelial, Brain, T cells, Kidney, Thyroid, Ovary, Skeletal muscle, Testis, Monocytes, Pancreas, Breast, Prostate, Myeloid, Lung, Fetal liver, Testes

Animal Models for Cholangiocarcinoma or affiliated genes

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36MGI
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MGI Mouse Phenotypes related to Cholangiocarcinoma:

36
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:001076812.7AFP, MET, OGG1, HGF, TIMP3, VEGFC
2MP:000538412.6MUC2, MET, OGG1, HGF, TIMP3, CDX2
3MP:000538512.2TIMP3, HGF, MET, VEGFC, MUC2, CDX2
4MP:000538712.0TIMP3, OGG1, MET, VEGFC, MUC2, MUC5AC
5MP:000538111.8MET, MUC2, MUC5AC, MUC1, CDX2, CDKN1B
6MP:000539711.8HGF, OGG1, MUC2, CDKN1B, MCL1, CFLAR
7MP:000200611.5KRT19, CDKN1B, MUC2, AFP, MET
8MP:000537611.4TIMP3, AFP, MUC2, MUC5AC, CDX2
9MP:000537911.3AFP, MUC2, CDX2, CDKN1B, KRT19
10MP:000537011.1TIMP3, HGF, OGG1, MET, MUC1, CDKN1B

Publications for Cholangiocarcinoma

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Sources:
50PubMed
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Articles related to Cholangiocarcinoma:

(show top 50)    (show all 1419)
idTitleAuthorsYear
1
A review of the clinical diagnosis and therapy of cholangiocarcinoma. (24366497)
2014
2
Hepatocellular carcinoma and cholangiocarcinoma: an update. (24245910)
2014
3
Anticancer effect of arsenic trioxide on cholangiocarcinoma: in vitro experiments and in vivo xenograft mouse model. (23467906)
2013
4
A comparison of trends in mortality from primary liver cancer and intrahepatic cholangiocarcinoma in Europe. (23378539)
2013
5
Activation of mammalian target of rapamycin complex 1 (mTORC1) and Raf/Pyk2 by growth factor-mediated Eph receptor 2 (EphA2) is required for cholangiocarcinoma growth and metastasis. (23315987)
2013
6
Cholangiocarcinoma among workers in the printing industry. (24142971)
2013
7
Yttrium-90 radioembolization for intrahepatic cholangiocarcinoma: safety, response, and survival analysis. (23602420)
2013
8
Global alterations of DNA methylation in cholangiocarcinoma target the wnt signaling pathway. (24002901)
2013
9
ERCC1 Can Be a Prognostic Factor in Hilar Cholangiocarcinoma and Extrahepatic Bile Duct Cancer, But Not in Intrahepatic Cholangiocarcinoma. (23613672)
2013
10
Identification of prognostic factors and the impact of palliative resection on survival of patients with stage IV hepatolithiasis-associated intrahepatic cholangiocarcinoma. (24310342)
2013
11
Concomitant dysregulation of microRNAs miR-151-3p and miR-126 correlates with improved survival in resected cholangiocarcinoma. (23458262)
2013
12
Molecular profiling of cholangiocarcinoma shows potential for targeted therapy treatment decisions. (23391413)
2013
13
Retrospective analysis of survival benefits of chemotherapy for metastatic or non-resectable intrahepatic cholangiocarcinoma. (23711827)
2013
14
A zebrafish model of intrahepatic cholangiocarcinoma by dual expression of hepatitis B virus X and hepatitis C virus core protein in liver. (22729936)
2012
15
Isocitrate dehydrogenase 1 and 2 mutations in cholangiocarcinoma. (22503487)
2012
16
Immunization with aspartate-I^-hydroxylase-loaded dendritic cells produces antitumor effects in a rat model of intrahepatic cholangiocarcinoma. (21898484)
2012
17
Duodenoportal fistula resulting from peptic ulcer after extended right hepatectomy for cholangiocarcinoma. (22524745)
2012
18
Differentiating mass-forming intrahepatic cholangiocarcinoma from atypical hepatocellular carcinoma using gadoxetic acid-enhanced MRI. (22425613)
2012
19
Angiotensin II enhances epithelial-to-mesenchymal transition through the interaction between activated hepatic stellate cells and the stromal cell-derived factor-1/CXCR4 axis in intrahepatic cholangiocarcinoma. (22664794)
2012
20
A phase II trial of gemcitabine and capecitabine in patients with unresectable or metastatic gallbladder cancer or cholangiocarcinoma: Southwest Oncology Group study S0202. (21556747)
2011
21
Slug inhibition upregulates radiation-induced PUMA activity leading to apoptosis in cholangiocarcinomas. (21120639)
2011
22
Long-term survival after resection of mass-forming type intrahepatic cholangiocarcinoma directly infiltrating the transverse colon and sequential brain metastasis: Report of a case. (21922366)
2011
23
A case of clonorchiasis with focal intrahepatic duct dilatation mimicking an intrahepatic cholangiocarcinoma. (22741114)
2011
24
Methyl-CpG binding protein MBD2 is implicated in methylation-mediated suppression of miR-373 in hilar cholangiocarcinoma. (21165562)
2011
25
Role of dihydropyrimidine dehydrogenase and thymidylate synthase expression in immunohistochemistry of intrahepatic cholangiocarcinoma. (20880064)
2011
26
Opisthorchiasis and Opisthorchis-associated cholangiocarcinoma in Thailand and Laos. (20655862)
2011
27
Common bile duct carcinoid mimicking the clinical, EUS, and ERCP findings of cholangiocarcinoma: a rare but potentially curable cause of obstructive jaundice. (21723230)
2011
28
p62+ Hyaline inclusions in intrahepatic cholangiocarcinoma associated with viral hepatitis or alcoholic liver disease. (20716803)
2010
29
Detection of distant metastasis to skeletal muscle by 18F-FDG-PET in a case of intrahepatic cholangiocarcinoma. (20924217)
2010
30
Immunohistochemically detected expression of p27(Kip1) and Skp2 predicts survival in patients with intrahepatic cholangiocarcinomas. (19034576)
2009
31
Intrahepatic cholangiocarcinoma progression: prognostic factors and basic mechanisms. (19896103)
2009
32
Serum cytokeratin 19 fragment (CYFRA21-1) as a prognostic factor in intrahepatic cholangiocarcinoma. (17955299)
2008
33
Intrahepatic cholangiocarcinoma arising in chronic viral hepatitis-associated cirrhosis: two transplant cases. (19100500)
2008
34
Clinicopathological and prognostic significance of EGFR, VEGF, and HER2 expression in cholangiocarcinoma. (18087285)
2008
35
Transcatheter arterial chemoembolization or chemoinfusion for unresectable intrahepatic cholangiocarcinoma: clinical efficacy and factors influencing outcomes. (18704990)
2008
36
Serotonin metabolism is dysregulated in cholangiocarcinoma, which has implications for tumor growth. (19010890)
2008
37
Identification of osteopontin as the most consistently over-expressed gene in intrahepatic cholangiocarcinoma: detection by oligonucleotide microarray and real-time PCR analysis. (18442196)
2008
38
GNAS1 T393C polymorphism is associated with clinical course in patients with intrahepatic cholangiocarcinoma. (17356712)
2007
39
Dual blockade of the Hedgehog and ERK1/2 pathways coordinately decreases proliferation and survival of cholangiocarcinoma cells. (17294242)
2007
40
Sodium iodide symporter is expressed at the preneoplastic stages of liver carcinogenesis and in human cholangiocarcinoma. (17408651)
2007
41
Elevated expression of cyclooxygenase-2 is a negative prognostic factor for overall survival in intrahepatic cholangiocarcinoma. (17165088)
2007
42
Analysis of p53 mutations for a mutational signature in human intrahepatic cholangiocarcinoma. (16596244)
2006
43
Expression of phosphorylated ERK1/2 and homeodomain protein CDX2 in cholangiocarcinoma. (16794828)
2006
44
Altered expression of thioredoxin reductase-1 in dysplastic bile ducts and cholangiocarcinoma in a hamster model. (16871013)
2006
45
Drug sensitivity and drug resistance profiles of human intrahepatic cholangiocarcinoma cell lines. (15884115)
2005
46
Metastatic colon cancer from intrahepatic cholangiocarcinoma. (16258812)
2005
47
Cyclooxygenase-2 and prostaglandin signaling in cholangiocarcinoma. (15921858)
2005
48
An intraductal papillary component is associated with prolonged survival after hepatic resection for intrahepatic cholangiocarcinoma. (14716802)
2004
49
Aberrant expression of trefoil factor family 1 in biliary epithelium in hepatolithiasis and cholangiocarcinoma. (14563942)
2003
50
Expression of Kit and platelet-derived growth factor receptors alpha and beta in cholangiocarcinoma, and case report of therapy with imatinib mesylate (STI571). (14501388)
2003

Genetic Variations for Cholangiocarcinoma

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Expression for genes affiliated with Cholangiocarcinoma

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1BioGPS, 15Gene Expression Omnibus DataSets
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Expression patterns in normal tissues for genes affiliated with Cholangiocarcinoma

Search GEO for disease gene expression data for Cholangiocarcinoma.

Pathways for genes affiliated with Cholangiocarcinoma

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Sources:
51QIAGEN, 29KEGG, 37NCBI BioSystems Database, 53Reactome
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Compounds for genes affiliated with Cholangiocarcinoma

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Sources:
44Novoseek, 59Tocris Bioscience, 28IUPHAR, 11DrugBank, 24HMDB, 49PharmGKB
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Compounds related to Cholangiocarcinoma according to GeneCards/GeneDecks:

(show top 50)    (show all 106)
idCompoundScoreTop Affiliating Genes
1paraffin4413.6TIMP3, CEACAM3, HGF, MET, VEGFC, MAGEA3
2tyrosine4413.6KRT20, KRT7, KRT19, CFLAR, MCL1, CDKN1B
3progesterone44 59 28 11 2417.3TIMP3, MET, AFP, VEGFC, CEACAM3, LGALS3
4retinoic acid44 2414.3TIMP3, HGF, MET, AFP, VEGFC, MUC2
5calcium44 49 11 2416.3MUC2, MUC5AC, MUC1, MUC4, CDX2, CFLAR
6oligonucleotide4413.2AFP, MET, OGG1, TIMP3, MCL1, CDKN1B
7oxygen44 2414.2TIMP3, HGF, OGG1, MET, AFP, VEGFC
8vegf4413.2KRT20, KRT7, LGALS3, CEACAM3, KRT19, CFLAR
9testosterone44 59 11 2415.8KRT7, AFP, CFLAR, MUC1, HGF, VEGFC
10thymidine44 2413.7CEACAM3, VEGFC, AFP, MET, HGF, OGG1
11glucose4412.7KRT19, MET, TIMP3, CEACAM3, KRT7, MUC2
12phosphatidylinositol4412.5CFLAR, MCL1, CDKN1B, MUC4, MUC5AC, VEGFC
135fluorouracil4412.3MUC5AC, CFLAR, MCL1, CDKN1B, MUC1, CEACAM3
14matrigel4412.3KRT7, KRT19, MUC4, MUC1, VEGFC, MET
15lipid4412.3MCL1, MUC1, VEGFC, CFLAR, AFP, KRT7
16pd 98,0594412.3MCL1, CDKN1B, MUC5AC, MUC2, VEGFC, MET
17glycogen44 2413.3MET, AFP, MUC1, HGF, MCL1, KRT19
18cisplatin44 49 59 1115.3MCL1, CDKN1B, LGALS3, KRT19, CFLAR, MUC1
19dexamethasone44 49 28 1115.3MCL1, CDKN1B, MUC4, MUC5AC, MUC2, AFP
20nitric oxide44 11 2414.2LGALS3, CEACAM3, CFLAR, MUC1, MUC5AC, MET
21threonine4412.2LGALS3, CFLAR, CDKN1B, MUC1, MUC5AC, MCL1
22hematoxylin4412.2MUC5AC, MUC2, KRT20, LGALS3, CEACAM3, MUC1
23methotrexate44 49 1114.2KRT7, CDKN1B, MUC1, MUC5AC, AFP, MUC2
24ly2940024412.1MUC2, CFLAR, VEGFC, MET, HGF, CDKN1B
25actinomycin d4412.1HGF, CFLAR, MCL1, CDKN1B, MET, AFP
26cysteine4412.1LGALS3, MET, MUC2, MUC5AC, CFLAR, MUC1
27cholesterol44 28 11 2415.0AFP, MET, MUC2, MUC5AC, MUC4, CDKN1B
28dmso4412.0HGF, MUC1, CDKN1B, MUC5AC, MUC2, AFP
29steroid4412.0CEACAM3, TIMP3, MET, MUC4, CDX2, KRT19
30ribonucleic acid4411.9KRT19, VEGFC, MET, MUC5AC, TIMP3, KRT20
3112-o-tetradecanoylphorbol 13-acetate4411.9HGF, TIMP3, MCL1, CDKN1B, MUC1, MUC2
32wortmannin4411.9TIMP3, LGALS3, CFLAR, CDKN1B, MET, MCL1
33doxorubicin44 49 1113.9CDKN1B, AFP, CFLAR, KRT20, CEACAM3, HGF
34adriamycin4411.9CEACAM3, CFLAR, AFP, MUC1, HGF, OGG1
35b 7234411.9KRT20, KRT19, AFP, CEACAM3, KRT7, MUC1
36serine4411.8MUC5AC, MUC2, AFP, CDKN1B, CFLAR, KRT19
37formaldehyde44 2412.7KRT19, CDKN1B, MUC1, KRT7, KRT20, LGALS3
38agar4411.7LGALS3, CDKN1B, MET, HGF, AFP, MUC1
39etoposide44 49 59 1114.6CDKN1B, MCL1, LGALS3, HGF, AFP, CFLAR
40estradiol44 11 2413.5CFLAR, VEGFC, AFP, TIMP3
41polysaccharide4411.4AFP, LGALS3, KRT7, MUC1, MUC5AC, HGF
42mucicarmine4411.4MUC1, KRT7, KRT20, CEACAM3
43paclitaxel44 49 1113.3HGF, CDKN1B, MCL1, CFLAR, KRT19, AFP
44carbohydrates4411.3KRT19, MUC5AC, MUC2
45sialic acid4411.2CEACAM3, KRT7
46butyrate4411.2MCL1, CDX2
47trastuzumab44 49 1113.1CDKN1B, MUC4, MUC1, VEGFC
48galactose4411.1CEACAM3, LGALS3, MUC2
49heparin44 28 11 2414.0CDKN1B, AFP, TIMP3
505-aza-2deoxycytidine4411.0CDX2, KRT7, CFLAR, MUC2, TIMP3

GO Terms for genes affiliated with Cholangiocarcinoma

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16Gene Ontology
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Cellular components related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1intermediate filamentGO:00588211.0KRT20, KRT7, KRT19
2Golgi lumenGO:00579610.8MUC4, MUC1, MUC5AC, MUC2

Biological processes related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1O-glycan processingGO:01626611.2MUC4, MUC1, MUC5AC, MUC2
2post-translational protein modificationGO:04368711.1MUC4, MUC1, MUC5AC, MUC2
3negative regulation of hydrogen peroxide-mediated programmed cell deathGO:190129910.8MET, HGF
4myoblast proliferationGO:05145010.7HGF, MET
5regulation of branching involved in salivary gland morphogenesis by mesenchymal-epithelial signalingGO:06066510.5MET, HGF

Molecular functions related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1protein bindingGO:00551512.9LGALS3, HGF, OGG1, MET, VEGFC, MAGEA3

Products for genes affiliated with Cholangiocarcinoma

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  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Cholangiocarcinoma

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3CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
28IUPHAR
29KEGG
34MeSH
35MESH via Orphanet
36MGI
39NCIt
40NDF-RT
43NINDS
44Novoseek
46OMIM
47OMIM via Orphanet
50PubMed
51QIAGEN
57SNOMED-CT via Orphanet
60UMLS
61UMLS via Orphanet