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Cholangiocarcinoma
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{{Short description|Cancer of the bile ducts}} {{Featured article}} {{Use dmy dates|date=March 2025}} {{Infobox medical condition | name = Cholangiocarcinoma | synonyms = Bile duct cancer, cancer of the bile duct<ref name=NCI2018Pro/> | image = Cholangiocarcinoma - very high mag.jpg | width = | alt = | caption = [[Micrograph]] of an intrahepatic cholangiocarcinoma (right of image) adjacent to normal [[hepatocytes|liver cells]] (left of image). [[H&E stain]]. | pronounce = {{IPAc-en|k|ə|,|l|æ|n|dʒ|i|ou|,|k|a:r|s|I|'|n|ou|m|ə|}} {{respell|kə-LAN-jee-oh-KAR-sih-NOH-mə}}<ref name=NCI2019Def/> | field = [[Oncology]] | symptoms = [[Abdominal pain]], [[jaundice|yellowish skin]], [[weight loss]], [[Pruritis|generalized itching]], fever<ref name=NCI2018Pro/> | complications = | onset = 70 years old<ref name=Lancet2014/> | duration = | types = Intrahepatic, perihilar, distal<ref name=Lancet2014/> | causes = | risks = [[Primary sclerosing cholangitis]], [[ulcerative colitis]], [[smoking]], heavy [[Alcoholic beverage|alcohol]] use, infection with certain [[liver fluke]]s, some congenital liver malformations<ref name=NCI2018Pro/> | diagnosis = Confirmed by examination of the tumor under a [[microscope]]<ref name=NCI2018Sym/> | differential = | prevention = | treatment = [[Surgical resection]], [[chemotherapy]], [[radiation therapy]], [[stenting]] procedures, [[liver transplantation]]<ref name=NCI2018Pro/> | medication = | prognosis = Generally poor<ref name=WCR2014_5.6/> | frequency = 1–2 people per 100,000 per year (Western world)<ref name=Brid2016/> | deaths = }} <!-- Definition and symptoms --> '''Cholangiocarcinoma''', also known as '''bile duct cancer''', is a type of [[cancer]] that forms in the [[bile duct]]s.<ref name=NCI2019Def>{{cite web |title=cholangiocarcinoma |url=https://www.cancer.gov/publications/dictionaries/cancer-terms/def/cholangiocarcinoma |website=National Cancer Institute |access-date=21 January 2019 |date=2 February 2011}}</ref> Symptoms of cholangiocarcinoma may include [[abdominal pain]], [[jaundice|yellowish skin]], [[weight loss]], [[Pruritis|generalized itching]], and fever.<ref name=NCI2018Pro>{{cite web |title=Bile Duct Cancer (Cholangiocarcinoma) Treatment |url=https://www.cancer.gov/types/liver/hp/bile-duct-treatment-pdq#section/all |website=National Cancer Institute |access-date=29 May 2021 |date=23 September 2020}}</ref> Light colored [[human feces|stool]] or dark [[urine]] may also occur.<ref name=NCI2018Sym/> Other [[biliary tract]] cancers include [[gallbladder cancer]] and cancer of the [[ampulla of Vater]].<ref>{{cite journal | vauthors = Benavides M, Antón A, Gallego J, Gómez MA, Jiménez-Gordo A, La Casta A, Laquente B, Macarulla T, Rodríguez-Mowbray JR, Maurel J | display-authors = 6 | title = Biliary tract cancers: SEOM clinical guidelines | journal = Clinical & Translational Oncology | volume = 17 | issue = 12 | pages = 982–7 | date = December 2015 | pmid = 26607930 | pmc = 4689747 | doi = 10.1007/s12094-015-1436-2 | doi-access = free | title-link = doi }}</ref> <!-- Cause and diagnosis --> Risk factors for cholangiocarcinoma include [[primary sclerosing cholangitis]] (an inflammatory disease of the bile ducts), [[ulcerative colitis]], [[cirrhosis]], [[hepatitis C]], [[hepatitis B]], infection with certain [[liver fluke]]s, and some congenital liver malformations.<ref name=NCI2018Pro/><ref name=Lancet2014/><ref>{{cite journal | vauthors = Steele JA, Richter CH, Echaubard P, Saenna P, Stout V, Sithithaworn P, Wilcox BA | display-authors = 6 | title = Thinking beyond Opisthorchis viverrini for risk of cholangiocarcinoma in the lower Mekong region: a systematic review and meta-analysis | journal = Infectious Diseases of Poverty | volume = 7 | issue = 1 | pages = 44 | date = May 2018 | pmid = 29769113 | pmc = 5956617 | doi = 10.1186/s40249-018-0434-3 | doi-access = free | title-link = doi }}</ref> Most people have no identifiable risk factors.<ref name=Lancet2014/> The diagnosis is suspected based on a combination of [[blood test]]s, [[medical imaging]], [[endoscopy]], and sometimes surgical exploration.<ref name=NCI2018Sym/> The disease is confirmed by examination of cells from the tumor under a [[microscope]].<ref name=NCI2018Sym>{{cite web |title=Bile Duct Cancer (Cholangiocarcinoma) |url=https://www.cancer.gov/types/liver/patient/about-bile-duct-cancer-pdq#section/all |website=National Cancer Institute |access-date=21 January 2019 |date=5 July 2018}}</ref> It is typically an [[adenocarcinoma]] (a cancer that forms [[glands]] or secretes [[mucin]]).<ref name=Lancet2014/> <!-- Treatment and prognosis --> Cholangiocarcinoma is typically incurable at diagnosis which is why early detection is ideal.<ref>{{Cite journal |last1=Zhang |first1=Tan |last2=Zhang |first2=Sina |last3=Jin |first3=Chen |last4=Lin |first4=Zixia |last5=Deng |first5=Tuo |last6=Xie |first6=Xiaozai |last7=Deng |first7=Liming |last8=Li |first8=Xueyan |last9=Ma |first9=Jun |last10=Ding |first10=Xiwei |last11=Liu |first11=Yaming |last12=Shan |first12=Yunfeng |last13=Yu |first13=Zhengping |last14=Wang |first14=Yi |last15=Chen |first15=Gang |display-authors=3 |year=2021 |title=A Predictive Model Based on the Gut Microbiota Improves the Diagnostic Effect in Patients with Cholangiocarcinoma |journal=Frontiers in Cellular and Infection Microbiology |volume=11 |page=751795 |doi=10.3389/fcimb.2021.751795 |pmc=8650695 |pmid=34888258 |doi-access=free |last16=Li |first16=Jialiang}}</ref><ref name=NCI2018Pro/> In these cases [[palliative treatment]]s may include [[surgical resection]], [[chemotherapy]], [[radiation therapy]], and [[stenting]] procedures.<ref name=NCI2018Pro/> In about a third of cases involving the [[common bile duct]] and less commonly with other locations the tumor can be completely removed by surgery offering a chance of a cure.<ref name=NCI2018Pro/> Even when surgical removal is successful chemotherapy and radiation therapy are generally recommended.<ref name=NCI2018Pro/> In certain cases surgery may include a [[liver transplantation]].<ref name=Lancet2014>{{cite journal | vauthors = Razumilava N, Gores GJ | title = Cholangiocarcinoma | journal = Lancet | volume = 383 | issue = 9935 | pages = 2168–79 | date = June 2014 | pmid = 24581682 | pmc = 4069226 | doi = 10.1016/S0140-6736(13)61903-0 | doi-access = free | title-link = doi }}</ref> Even when surgery is successful the [[5-year survival]] is typically less than 50%.<ref name=Brid2016/> <!-- Epidemiology --> Cholangiocarcinoma is rare in the [[Western world]], with estimates of it occurring in 0.5–2 people per 100,000 per year.<ref name=NCI2018Pro/><ref name=Brid2016/> Rates are higher in [[Southeast Asia]] where liver flukes are common.<ref name=WCR2014_5.6>{{cite book |last1=Bosman |first1=Frank T.|editor1-last=Stewart |editor1-first=Bernard W. |editor2-last=Wild |editor2-first=Christopher P |title=World Cancer Report |date=2014 |publisher=the International Agency for Research on Cancer, World Health Organization |isbn=978-92-832-0443-5 |pages=403–12 |url=https://publications.iarc.fr/_publications/media/download/5839/bc44643f904185d5c8eddb933480b5bc18b21dba.pdf |chapter=Chapter 5.6: Liver cancer}} {{open access}}</ref> Rates in parts of [[Thailand]] are 60 per 100,000 per year.<ref name=WCR2014_5.6/> It typically occurs in people in their 70s, and in the 40s for those with primary sclerosing cholangitis.<ref name=Lancet2014/> Rates of cholangiocarcinoma within the liver in the Western world have increased.<ref name=Brid2016>{{cite journal | vauthors = Bridgewater JA, Goodman KA, Kalyan A, Mulcahy MF | title = Biliary Tract Cancer: Epidemiology, Radiotherapy, and Molecular Profiling | journal = American Society of Clinical Oncology Educational Book. American Society of Clinical Oncology. Annual Meeting | volume = 35 | issue = 36 | pages = e194-203 | date = 2016 | pmid = 27249723 | doi = 10.1200/EDBK_160831 | doi-access = free }}</ref>
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