KEGG   DISEASE: CholangiocarcinomaHelp
Entry
H00046                      Disease                                

Name
Cholangiocarcinoma
Description
Cholangiocarcinoma is a highly malignant neoplasm that carries a poor prognosis and lacks effective therapy. It is the second most common primary hepatic tumor, and it is increasing in incidence and carries a high mortality. The tumor arises from the ductular epithelium of the biliary tree, either within the liver (intrahepatic cholangiocarcinoma: ICC) or more commonly from the extrahepatic bile ducts (extrahepatic cholangiocarcinoma). Several studies have demonstrated mutations resulting in overexpression of K-ras and p53 genes. These genetic alterations are associated with a more aggressive phenotype in this cancer. Many reports have implicated overexpression of the tyrosine kinase proto-oncogenes c-erbB-2 (HER-2/neu) and c-Met, as well as cyclo-oxygenase-2 (COX-2) activity in intrahepatic cholangiocarcinoma.
Category
Cancer
Brite
Human diseases [BR:br08402]
 Cancers
  Cancers of the digestive system
   H00046  Cholangiocarcinoma
Human diseases in ICD-11 classification [BR:br08403]
 02 Neoplasms
  Malignant neoplasms, except of lymphoid, haematopoietic, central nervous system or related tissues
   Malignant neoplasms, stated or presumed to be primary, of specified sites, except of lymphoid, haematopoietic, central nervous system or related tissues
    Malignant neoplasms of digestive organs
     Malignant neoplasms of intestine
      Malignant neoplasms of large intestine
       2C12  Malignant neoplasms of liver or intrahepatic bile ducts
        H00046  Cholangiocarcinoma
Tumor markers [br08442.html]
 H00046
BRITE hierarchy
Gene
K-ras (mutation) [HSA:3845] [KO:K07827]
p53 (mutation) [HSA:7157] [KO:K04451]
c-Met (overexpression) [HSA:4233] [KO:K05099]
ERBB2 (overexpression, amplification) [HSA:2064] [KO:K05083]
p16/INK4A (mutation) [HSA:1029] [KO:K06621]
COX2 (overexpression) [HSA:5743] [KO:K11987]
Carcinogen
Opisthorchis viverrini (infection with)
Radium-224 and its decay products [CPD:C16455]
*Chimney sweeping
*2,3,7,8-Tetrachlorodibenzo-para-dioxin [CPD:C07557]
*Thorium-232 and its decay products, administered intravenously as a colloidal dispersion of thorium232 dioxide
*Tobacco smoking and tobacco smoke
*: Although this is a risk factor for liver cancer in general, the strength of association with different histological tumor types is not well understood.
Pathogen
Opisthorchis viverrini [GN:ovi]
Comment
ICD-O: 8160/3, Tumor type: Intrahepatic cholangiocarcinoma
Other DBs
ICD-11: 2C12.1
ICD-10: C22
MeSH: D018281
Reference
PMID:15218306 (gene, tumor type)
  Authors
Olnes MJ, Erlich R.
  Title
A review and update on cholangiocarcinoma.
  Journal
Oncology 66:167-79 (2004)
DOI:10.1159/000077991
Reference
  Authors
Fava G, Marzioni M, Benedetti A, Glaser S, DeMorrow S, Francis H, Alpini G.
  Title
Molecular pathology of biliary tract cancers.
  Journal
Cancer Lett 250:155-67 (2007)
DOI:10.1016/j.canlet.2006.09.011
Reference
  Authors
Lazaridis KN, Gores GJ.
  Title
Cholangiocarcinoma.
  Journal
Gastroenterology 128:1655-67 (2005)
DOI:10.1053/j.gastro.2005.03.040
Reference
PMID:15928434 (carcinogen)
  Authors
Khurana S, Dubey ML, Malla N.
  Title
Association of parasitic infections and cancers.
  Journal
Indian J Med Microbiol 23:74-9 (2005)
DOI:10.4103/0255-0857.16044
Reference
PMID:15744742 (carcinogen)
  Authors
Herrera LA, Benitez-Bribiesca L, Mohar A, Ostrosky-Wegman P.
  Title
Role of infectious diseases in human carcinogenesis.
  Journal
Environ Mol Mutagen 45:284-303 (2005)
DOI:10.1002/em.20122
Reference
PMID:10564925 (carcinogene)
  Authors
Nekolla EA, Kellerer AM, Kuse-Isingschulte M, Eder E, Spiess H.
  Title
Malignancies in patients treated with high doses of radium-224.
  Journal
Radiat Res 152:S3-7 (1999)
DOI:10.2307/3580102
Reference
PMID:9498904 (carcinogen)
  Authors
Boffetta P, Jourenkova N, Gustavsson P.
  Title
Cancer risk from occupational and environmental exposure to polycyclic aromatic hydrocarbons.
  Journal
Cancer Causes Control 8:444-72 (1997)
Reference
PMID:15660110 (carcinogen)
  Authors
Luch A.
  Title
Nature and nurture - lessons from chemical carcinogenesis.
  Journal
Nat Rev Cancer 5:113-25 (2005)
DOI:10.1038/nrc1546
Reference
PMID:12556326 (carcinogen)
  Authors
Harrison JD, Muirhead CR.
  Title
Quantitative comparisons of cancer induction in humans by internally deposited radionuclides and external radiation.
  Journal
Int J Radiat Biol 79:1-13 (2003)
DOI:10.1080/0955300021000038671

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