CCA
MCID: CHL065
MIFTS: 69

Cholangiocarcinoma (CCA) malady

Liver diseases, Cancer diseases, Rare diseases categories
Download this MalaCard

Summaries for Cholangiocarcinoma

About this section
Sources:
65Wikipedia, 47OMIM, 33MalaCards
See all sources

Fully expand this MalaCard
Wikipedia:65 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 gallbladder cancer, and has symptoms including abnormal colour of the urine/cholic/dark urines, extrahepatic biliary tract/gallbladder neoplasm/tumor/carcinoma/cancer and pruritus/itching. An important gene associated with Cholangiocarcinoma is PTPN3 (protein tyrosine phosphatase, non-receptor type 3), and among its related pathways are IL-9 Signaling Pathways and O-linked glycosylation of mucins. The compounds trastuzumab and [alcl] have been mentioned in the context of this disorder. Affiliated tissues include liver, small intestine and lymph node, and related mouse phenotypes are tumorigenesis and integument.

Description from OMIM:47 615619

Aliases & Classifications for Cholangiocarcinoma

About this section
Sources:
8Disease Ontology, 10DISEASES, 45Novoseek, 49Orphanet, 47OMIM, 62UMLS, 58SNOMED-CT, 40NCIt, 35MeSH, 36MESH via Orphanet, 26ICD10 via Orphanet, 59SNOMED-CT via Orphanet, 63UMLS via Orphanet
See all sources

Classifications:

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


Characteristics (Orphanet epidemiological data):

49
cholangiocarcinoma:
Inheritance: Sporadic; Prevalence: 1-9/100000; Age of onset: Adulthood


Aliases & Descriptions:

cholangiocarcinoma 8 10 45 49 47 62
adult primary cholangiocellular carcinoma 8 62
adult primary cholangiocarcinoma 8
bile duct carcinoma 62
cholangiosarcoma 8
bile duct cancer 49
cca 49


External Ids:

Disease Ontology8 DOID:4947
NCIt40 C4436
MeSH35 D018281
MESH via Orphanet36 D018281, D001650
SNOMED-CT58 312104005, 70179006
ICD10 via Orphanet26 C22.1
SNOMED-CT via Orphanet59 312104005, 70179006
UMLS via Orphanet63 C0206698, C0740277
OMIM47 615619

Related Diseases for Cholangiocarcinoma

About this section
Sources:
17GeneCards, 18GeneDecks
See all sources

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

(show top 50)    (show all 230)
idRelated DiseaseScoreTop Affiliating Genes
1hepatocellular carcinoma31.0CDKN1B, CDX2, KRT19, KRT7, OGG1, MAGEA3
2gallbladder cancer30.8CDX2, CEACAM3, MUC2
3biliary papillomatosis30.5MUC2
4adenocarcinoma30.4CDKN1B, CDX2, KRT19, KRT20, KRT7, OGG1
5colon adenocarcinoma30.3CDKN1B, KRT20, LGALS3, MUC5AC, MUC2
6bile duct carcinoma30.2MAGEA3, CEACAM3
7primary biliary cirrhosis30.2KRT19, KRT7, MCL1
8liver cirrhosis30.1KRT19, MUC5AC, TIMP3, AFP
9adenoma30.1CDKN1B, CDX2, KRT19, KRT20, KRT7, OGG1
10colon cancer30.0CDKN1B, CDX2, KRT19, KRT20, LGALS3, CEACAM6
11bile duct adenoma30.0KRT19, KRT7
12spindle cell sarcoma30.0KRT7, MUC1
13cystadenocarcinoma29.9CEACAM3, MUC5AC, MUC1, MUC2
14cystadenoma29.9KRT7, CEACAM3, VEGFC
15neuroendocrine carcinoma29.9CDX2, KRT19, KRT20, KRT7, MUC1
16parathyroid adenoma29.9CDKN1B, LGALS3
17pancreatic cancer29.8CDX2, KRT19, KRT20, KRT7, CEACAM3, MUC5AC
18colorectal cancer29.8CDKN1B, CDX2, KRT20, KRT7, OGG1, LGALS3
19melanoma29.7CDKN1B, CDX2, MAGEA3, LGALS3, CEACAM3, MUC4
20breast cancer29.6CDKN1B, CDX2, KRT19, KRT20, KRT7, LGALS3
21intrahepatic cholangiocarcinoma11.2
22hepatitis10.8
23cholangitis10.8
24sclerosing cholangitis10.7
25primary sclerosing cholangitis10.7
26hepatitis b10.5
27opisthorchiasis10.5
28pancreatitis10.5
29congenital contractural arachnodactyly10.4
30clonorchiasis10.4
31hepatitis c10.4
32choledochal cyst10.4
33schistosomiasis10.4
34hepatitis c virus10.3
35hepatitis b virus infection10.3
36viral hepatitis10.3
37whiplash10.3
38ascariasis10.2
39hypercalcemia10.2
40liver disease10.2
41endotheliitis10.2
42urinary schistosomiasis10.2
43intestinal schistosomiasis10.2
44sarcomatous intrahepatic cholangiocarcinoma10.2
45obstructive jaundice10.2
46klatskin's tumor10.2
47mucinous adenocarcinoma10.2
48liver carcinoma in situ10.2
49lynch syndrome10.2
50ampulla of vater carcinoma10.1MUC2

Graphical network of the top 20 diseases related to Cholangiocarcinoma:



Diseases related to cholangiocarcinoma

Symptoms for Cholangiocarcinoma

About this section
Sources:
47OMIM, 49Orphanet
See all sources


Clinical features from OMIM:

615619

Symptoms:

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

Drugs & Therapeutics for Cholangiocarcinoma

About this section
Sources:
42NIH Clinical Center, 6ClinicalTrials, 62UMLS, 41NDF-RT
See all sources

Drug clinical trials:

Search ClinicalTrials for Cholangiocarcinoma

Search NIH Clinical Center for Cholangiocarcinoma

Genetic Tests for Cholangiocarcinoma

About this section

Anatomical Context for Cholangiocarcinoma

About this section
Sources:
33MalaCards
See all sources

MalaCards organs/tissues related to Cholangiocarcinoma:

33
Liver, Small intestine, Lymph node, Colon, Endothelial, Brain, T cells, Kidney, Skeletal muscle, Ovary, Thyroid, Prostate, Testis, Testes, Myeloid, Breast, Monocytes, Pancreas, Fetal liver

Animal Models for Cholangiocarcinoma or affiliated genes

About this section
Sources:
37MGI
See all sources

MGI Mouse Phenotypes related to Cholangiocarcinoma:

37
idDescriptionMGI Source AccessionScoreTop Affiliating Genes
1MP:00020069.4CDKN1B, AFP, MUC2, MUC1, OGG1, KRT19
2MP:00107719.4CDKN1B, KRT19, LGALS3, MUC1, MCL1, TIMP3
3MP:00053819.4MUC2, MUC1, MUC5AC, LGALS3, KRT19, CDX2
4MP:00053799.2AFP, TIMP3, MUC2, MUC1, KRT19, CDX2
5MP:00053879.0VEGFC, PTPN3, TIMP3, MCL1, MUC2, MUC5AC
6MP:00053768.9VEGFC, AFP, TIMP3, MUC2, MUC1, MUC5AC
7MP:00053848.9TIMP3, MCL1, MUC2, LGALS3, OGG1, KRT7
8MP:00053858.9VEGFC, TIMP3, MUC2, LGALS3, KRT19, CDX2
9MP:00107688.6VEGFC, AFP, TIMP3, MCL1, MUC2, LGALS3

Publications for Cholangiocarcinoma

About this section
Sources:
52PubMed
See all sources

Articles related to Cholangiocarcinoma:

(show top 50)    (show all 1407)
idTitleAuthorsYear
1
Hydroxytyrosol inhibits cholangiocarcinoma tumor growth: An in vivo and in vitro study. (24247752)
2014
2
Prediction of the Postoperative Prognosis of Intrahepatic Cholangiocarcinoma (ICC): Importance of Preoperatively-Determined Anatomic Invasion Level and Number of Tumors. (24122559)
2013
3
Adjuvant chemotherapy in resectable cholangiocarcinoma patients. (23829232)
2013
4
Clinical significances of cancer stem cells markers in patients with intrahepatic cholangiocarcinoma who underwent hepatectomy. (23645762)
2013
5
Chemotherapy for cholangiocarcinoma: An update. (23919111)
2013
6
Pure laparoscopic right hepatectomy by anterior approach with hanging maneuver for large intrahepatic cholangiocarcinoma. (24170066)
2013
7
Hepatobiliary resection with concomitant resection of the inferior vena cava for advanced intrahepatic cholangiocarcinoma: report of a case. (22961194)
2013
8
Clinical usefulness of (18) F-fluorodeoxyglucose positron emission tomography/computed tomography for patients with primary liver cancer with special reference to rare histological types, hepatocellular carcinoma with sarcomatous change and combined hepatocellular and cholangiocarcinoma. (23145869)
2013
9
Comparison of computed tomography- and positron emission tomography-based radiotherapy planning in cholangiocarcinoma. (24051924)
2013
10
Factors affecting survival time of cholangiocarcinoma patients: a prospective study in Northeast Thailand. (23679246)
2013
11
Intrahepatic cholangiocarcinoma: impact of genetic hemochromatosis on outcome and overall survival after surgical resection. (23183056)
2013
12
Anticancer effect of arsenic trioxide on cholangiocarcinoma: in vitro experiments and in vivo xenograft mouse model. (23467906)
2013
13
Infiltration of peritumoural but tumour-free parenchyma with IgG4-positive plasma cells in hilar cholangiocarcinoma and pancreatic adenocarcinoma. (23602806)
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
Clinicopathological significance of E-cadherin, I^-catenin and epidermal growth factor receptor expression in intrahepatic cholangiocarcinoma. (22281980)
2012
16
Treatment of recurrent intrahepatic cholangiocarcinoma. (23132419)
2012
17
Periductal infiltrating type of intrahepatic cholangiocarcinoma: a rare macroscopic type without any apparent mass. (22350300)
2012
18
Cholangiocarcinoma complicating secondary sclerosing cholangitis from cryptosporidiosis in an adult patient with CD40 ligand deficiency: case report and review of the literature. (22678466)
2012
19
Claudin-18 in biliary neoplasms. Its significance in the classification of intrahepatic cholangiocarcinoma. (21607649)
2011
20
Development of a questionnaire (EORTC module) to measure quality of life in patients with cholangiocarcinoma and gallbladder cancer, the EORTC QLQ-BIL21. (21266979)
2011
21
MicroRNA down-regulated in human cholangiocarcinoma control cell cycle through multiple targets involved in the G1/S checkpoint. (21809359)
2011
22
The value of F-18 FDG PET in patients with primary sclerosing cholangitis and cholangiocarcinoma using visual and semiquantitative analysis. (21892037)
2011
23
Periostin, a matrix protein, has potential as a novel serodiagnostic marker for cholangiocarcinoma. (21347516)
2011
24
A case of intrahepatic cholangiocarcinoma with marked mucus production. (22128317)
2011
25
Mixed hepatocellular cholangiocarcinoma and intrahepatic cholangiocarcinoma in patients undergoing transplantation for hepatocellular carcinoma. (21438129)
2011
26
Ubiquitin-specific protease 14 expression associated with intrahepatic cholangiocarcinoma cell differentiation. (21627382)
2011
27
Different roles of S100P overexpression in intrahepatic cholangiocarcinoma: carcinogenesis of perihilar type and aggressive behavior of peripheral type. (21412073)
2011
28
Radiofrequency ablation of intrahepatic cholangiocarcinoma: preliminary experience. (20411389)
2010
29
Clinical and histopathological study of "follicular cholangitis": Sclerosing cholangitis with prominent lymphocytic infiltration masquerading as hilar cholangiocarcinoma. (20880060)
2010
30
The E-cadherin repressor slug and progression of human extrahepatic hilar cholangiocarcinoma. (20594328)
2010
31
The prognosis and survival outcome of intrahepatic cholangiocarcinoma following surgical resection: association of lymph node metastasis and lymph node dissection with survival. (19626372)
2009
32
Relationship between microvessel count and postoperative survival in patients with intrahepatic cholangiocarcinoma. (19434454)
2009
33
Alterations of gene expression of RB pathway in Opisthorchis viverrini infection-induced cholangiocarcinoma. (19582476)
2009
34
Clinicopathological analysis of colorectal cancer liver metastasis and intrahepatic cholangiocarcinoma: are they just apples and oranges? (18329969)
2008
35
A novel "patient-like" model of cholangiocarcinoma progression based on bile duct inoculation of tumorigenic rat cholangiocyte cell lines. (18081149)
2008
36
Glycine N-methyltransferase is a favorable prognostic marker for human cholangiocarcinoma. (18624901)
2008
37
Serum cytokeratin 19 fragment (CYFRA21-1) as a prognostic factor in intrahepatic cholangiocarcinoma. (17955299)
2008
38
Aberrant expression of alpha-fetoprotein in intrahepatic cholangiocarcinoma: an exceptional occurrence. (18417680)
2008
39
Influence of hepatic resection margin on recurrence and survival in intrahepatic cholangiocarcinoma. (18685896)
2008
40
Indications for surgical treatment of intrahepatic cholangiocarcinoma with lymph node metastases. (18670844)
2008
41
14-3-3sigma negatively regulates the cell cycle, and its down-regulation is associated with poor outcome in intrahepatic cholangiocarcinoma. (17391729)
2007
42
Relation of cholangiocarcinomas to clonorchiasis and bile duct stones. (15185021)
2004
43
Major hepatic resection for hilar cholangiocarcinoma: analysis of 46 patients. (15136352)
2004
44
A case of combined hepatocellular-cholangiocarcinoma with sarcomatous transformation and second primary colon cancer]. (15218348)
2004
45
Comparative immunohistochemical profile of hepatocellular carcinoma, cholangiocarcinoma, and metastatic adenocarcinoma. (12514785)
2002
46
Aberrant CpG island methylation of multiple genes in intrahepatic cholangiocarcinoma. (12213730)
2002
47
Involvement of 85-kd cytosolic phospholipase A(2) and cyclooxygenase-2 in the proliferation of human cholangiocarcinoma cells. (12143044)
2002
48
Inhibition of interleukin 6-mediated mitogen-activated protein kinase activation attenuates growth of a cholangiocarcinoma cell line. (10534331)
1999
49
Biliary carcinoembryonic antigen levels are a marker for cholangiocarcinoma. (8554130)
1996
50
Chronic postcolectomy pericholangitis and cholangiocarcinoma. (2995479)
1985

Variations for Cholangiocarcinoma

About this section

Expression for genes affiliated with Cholangiocarcinoma

About this section
Sources:
2BioGPS, 15Gene Expression Omnibus DataSets
See all sources
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

About this section
Sources:
50PathCards, 54R&D Systems, 38NCBI BioSystems Database, 60Thomson Reuters, 53QIAGEN, 55Reactome, 30KEGG
See all sources

Pathways related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idSuper pathways (with members indented)ScoreTop Affiliating Genes
1
Show member pathways
IL-9 Signaling Pathway38
Development Thrombopoietin regulated cell processes60
IL-7 Signaling Pathway38
Immune response IL 9 signaling pathway60
10.0CDKN1B, MUC1, MCL1
2
Show member pathways
9.8MUC5AC, MUC4, MUC1, MUC2
3
Show member pathways
9.6MUC2, MUC1, MUC4, MUC5AC, CDX2

Compounds for genes affiliated with Cholangiocarcinoma

About this section
Sources:
45Novoseek, 51PharmGKB, 11DrugBank, 29IUPHAR, 24HMDB, 61Tocris Bioscience
See all sources

Compounds related to Cholangiocarcinoma according to GeneCards/GeneDecks:

(show top 50)    (show all 97)
idCompoundScoreTop Affiliating Genes
1trastuzumab45 51 1112.0MUC4, CDKN1B, VEGFC, MUC1
2[alcl]4510.0MUC1, CDKN1B, MCL1
3mucicarmine459.9MUC1, CEACAM3, KRT7, KRT20
4galactose459.9CEACAM3, AFP, MUC2, MUC1, LGALS3
5gemcitabine45 51 1111.9MCL1, CEACAM6, MUC1, CDKN1B
612-o-tetradecanoylphorbol 13-acetate459.9MUC2, CDKN1B, MUC1, MCL1, TIMP3
7dmso459.8MUC5AC, MUC2, AFP, OGG1, CDKN1B, MUC1
8adriamycin459.8MUC1, CEACAM3, OGG1, KRT19, AFP
9actinomycin d459.7CDKN1B, MUC1, MUC2, MCL1, AFP
10carbohydrates459.7KRT19, MUC2, MUC1, MUC5AC, LGALS3, KRT7
11polysaccharide459.6MUC1, AFP, LGALS3, KRT7, MUC5AC
12paclitaxel45 51 1111.6PTPN3, KRT19, MCL1, AFP, CDKN1B
13pd 98,059459.6TIMP3, MUC5AC, CDKN1B, VEGFC, PTPN3, MUC2
14heparin45 29 24 1112.6AFP, VEGFC, TIMP3, PTPN3, LGALS3, CDKN1B
15cisplatin45 51 61 1112.6CDKN1B, AFP, MCL1, MUC1, LGALS3, KRT19
16estradiol45 24 1111.6KRT19, MUC1, AFP, TIMP3, CDKN1B, VEGFC
17formaldehyde45 2410.6KRT20, KRT7, LGALS3, MUC1, CDKN1B, KRT19
185fluorouracil459.6MCL1, AFP, MUC2, MUC1, CDKN1B, MUC5AC
19ribonucleic acid459.5VEGFC, TIMP3, MUC1, MUC5AC, KRT20, KRT19
20glutamate459.5MCL1, AFP, PTPN3, TIMP3, LGALS3, OGG1
21b 723459.5KRT20, KRT19, AFP, MUC1, CEACAM3, KRT7
22glycogen45 2410.5LGALS3, KRT7, AFP, MCL1, KRT19, CDKN1B
23ly294002459.5MCL1, TIMP3, MUC2, VEGFC, CDKN1B
24matrigel459.5KRT7, KRT19, LGALS3, MUC4, MUC1, TIMP3
25methotrexate51 45 1111.5MUC1, MUC5AC, AFP, MUC2, CDKN1B, KRT7
26threonine459.5PTPN3, AFP, CDKN1B, LGALS3, MUC5AC, MUC2
27cysteine459.5TIMP3, MUC2, PTPN3, MUC1, MUC5AC, LGALS3
28sialic acid459.4MUC2, KRT7, LGALS3, CEACAM3, MUC5AC, MUC1
29butyrate459.4MCL1, AFP, PTPN3, MUC2, MUC1, LGALS3
30cholesterol45 29 24 1112.3MUC4, MUC5AC, CDKN1B, MUC1, MUC2, PTPN3
31lipid459.3AFP, VEGFC, TIMP3, MCL1, MUC1, PTPN3
32wortmannin459.3PTPN3, TIMP3, MCL1, LGALS3, CDKN1B
33steroid459.3TIMP3, CEACAM3, CDX2, KRT19, MUC4, AFP
34thymidine45 2410.3CDKN1B, MUC1, CEACAM3, OGG1, KRT19, CDX2
35nitric oxide45 24 1111.3CDKN1B, MUC1, VEGFC, MUC5AC, CEACAM3, LGALS3
36dexamethasone45 51 29 1112.2MUC1, VEGFC, AFP, PTPN3, MUC5AC, MUC4
37hematoxylin459.2MUC2, MUC1, MUC5AC, CEACAM3, LGALS3, KRT7
38phosphatidylinositol459.1MUC4, CDKN1B, LGALS3, CEACAM6, MUC5AC, VEGFC
39serine459.1MUC2, MCL1, MUC5AC, LGALS3, OGG1, KRT19
40testosterone45 61 24 1112.0KRT19, KRT20, CDKN1B, VEGFC, AFP, TIMP3
41oligonucleotide458.8CDX2, OGG1, LGALS3, MAGEA3, VEGFC, AFP
42glucose458.7TIMP3, PTPN3, AFP, VEGFC, KRT19, CDKN1B
43retinoic acid45 249.6MUC2, CDKN1B, KRT19, AFP, TIMP3, MCL1
44vegf458.6KRT20, CEACAM3, MUC1, AFP, VEGFC, MCL1
45oxygen45 249.6VEGFC, KRT19, OGG1, LGALS3, CEACAM3, MUC5AC
46calcium45 51 24 1111.2KRT19, MCL1, CDX2, CEACAM6, CEACAM3, MUC5AC
47progesterone45 29 61 24 1112.1MUC4, CDKN1B, CDX2, KRT19, KRT20, VEGFC
48estrogen458.0LGALS3, CEACAM3, MUC5AC, MUC4, MUC1, MUC2
49tyrosine458.0MUC4, MUC1, MCL1, PTPN3, AFP, MUC5AC
50paraffin457.6KRT7, KRT20, KRT19, CDX2, CDKN1B, VEGFC

GO Terms for genes affiliated with Cholangiocarcinoma

About this section
Sources:
16Gene Ontology
See all sources

Cellular components related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1intermediate filamentGO:0058829.7KRT19, KRT20, KRT7
2Golgi lumenGO:0057969.5MUC5AC, MUC4, MUC1, MUC2

Biological processes related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1negative regulation of membrane protein ectodomain proteolysisGO:05104510.2TIMP3, PTPN3
2positive chemotaxisGO:05091810.1LGALS3, VEGFC
3maintenance of gastrointestinal epitheliumGO:03027710.0MUC4, MUC2
4O-glycan processingGO:0162669.9MUC5AC, MUC4, MUC1, MUC2
5negative regulation of mitotic cell cycleGO:0459309.8CDKN1B, PTPN3
6post-translational protein modificationGO:0436879.8MUC2, MUC1, MUC4, MUC5AC

Molecular functions related to Cholangiocarcinoma according to GeneCards/GeneDecks:

idNameGO IDScoreTop Affiliating Genes
1chemoattractant activityGO:04205610.1LGALS3, VEGFC
2protein bindingGO:0055157.4CDKN1B, VEGFC, PTPN3, TIMP3, MCL1, MUC2

Products for genes affiliated with Cholangiocarcinoma

About this section
  • Antibodies
  • Proteins
  • Lysates
  • Antibodies

Sources for Cholangiocarcinoma

About this section
4CDC
13ExPASy
14FMA
22GTR
23HGMD
24HMDB
25ICD10
26ICD10 via Orphanet
27ICD9CM
29IUPHAR
30KEGG
35MeSH
36MESH via Orphanet
37MGI
40NCIt
41NDF-RT
44NINDS
45Novoseek
47OMIM
48OMIM via Orphanet
52PubMed
53QIAGEN
59SNOMED-CT via Orphanet
62UMLS
63UMLS via Orphanet