Kolangiokarsinoma: Perbedaan antara revisi

| caption = Gambar mikroskopokmikroskopik pada kolangiokarsinoma intrahepatik (gambar sebelah kanan) yang berdekatan dengan sel hati normal (gambar sebelah kiri). Noda H&E.

Faktor risiko untuk kolangiokarsinoma antara lain adalah ''primary sclerosing cholangitis'' (penyakit [[radang]] saluran empedu), [[kolitis ulserativaulseratif]], sirosis, [[hepatitis C]], [[hepatitis B]], infeksi cacing hati tertentu, dan beberapa malformasi hati kongenital.<ref name=":0" /><ref name=":2">{{Cite journal|last=Razumilava|first=Nataliya|last2=Gores|first2=Gregory J.|date=2014-06-21|title=Cholangiocarcinoma|url=https://www.thelancet.com/journals/lancet/article/PIIS0140-6736(13)61903-0/abstract|journal=The Lancet|language=English|volume=383|issue=9935|pages=2168–2179|doi=10.1016/S0140-6736(13)61903-0|issn=0140-6736|pmc=PMC4069226|pmid=24581682}}</ref><ref>{{Cite journal|last=Steele|first=Jennifer A.|last2=Richter|first2=Carsten H.|last3=Echaubard|first3=Pierre|last4=Saenna|first4=Parichat|last5=Stout|first5=Virginia|last6=Sithithaworn|first6=Paiboon|last7=Wilcox|first7=Bruce A.|date=2018-05-17|title=Thinking beyond Opisthorchis viverrini for risk of cholangiocarcinoma in the lower Mekong region: a systematic review and meta-analysis|url=https://doi.org/10.1186/s40249-018-0434-3|journal=Infectious Diseases of Poverty|volume=7|issue=1|pages=44|doi=10.1186/s40249-018-0434-3|issn=2049-9957|pmc=PMC5956617|pmid=29769113}}</ref> Namun, kebanyakan orang tidak memiliki faktor risiko yang dapat diidentifikasi. Diagnosis dicurigai berdasarkan kombinasi tes darah, [[pencitraan medis]], [[endoskopi]], dan terkadang melalui tindakan bedah. Untuk mengonfirmasi penyakit ini dilakukan pemeriksaan sel tumor menggunakan mikroskop.<ref name=":1" /> Kolangiokarsinoma biasanya merupakan adenokarsinoma (kanker yang membentuk kelenjar atau mengeluarkan musin).<ref name=":2" />

Setelah didiagnosis, kolangiokarsinoma biasanya tidak dapat disembuhkan. Oleh karena itu, deteksi dini sangat diperlukan.<ref name=":0" /><ref>{{Cite journal|last=Zhang|first=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|date=2021-11-23|title=A Predictive Model Based on the Gut Microbiota Improves the Diagnostic Effect in Patients With Cholangiocarcinoma|url=https://www.frontiersin.org/articles/10.3389/fcimb.2021.751795/full|journal=Frontiers in Cellular and Infection Microbiology|volume=11|pages=751795|doi=10.3389/fcimb.2021.751795|issn=2235-2988|pmc=PMC8650695|pmid=34888258}}</ref>Dalam kasus ini, [[perawatan paliatif]] yang dapat dilakukan antara lain adalah tindakan bedah, [[kemoterapi]], [[terapi radiasi]], dan prosedur pemasangan [[stent]].<ref name=":0" /> Pada sekitar sepertiga kasus yang melibatkan saluran empedu dan lebih jarang dengan lokasi lain, [[tumor]] dapat diangkat sepenuhnya melalui tindakan pembedahan yang memberikan kesempatan untuk sembuh.<ref name=":0" /> Bahkan ketika operasi pengangkatan berhasil, kemoterapi dan terapi radiasi umumnya akan tetap  direkomendasikan sebagai terapi tambahan.<ref name=":0" />Dalam kasus tertentu, tindakan operasi termasuk transplantasi [[hati]] dapat dilakukan.<ref name=":2" /> Namun, ketika operasi berhasil, kelangsungan hidup 5 tahun biasanya kurang dari 50%.<ref name=":3">{{Cite journal|last=Bridgewater|first=John A.|last2=Goodman|first2=Karyn A.|last3=Kalyan|first3=Aparna|last4=Mulcahy|first4=Mary F.|date=2016-05-01|title=Biliary Tract Cancer: Epidemiology, Radiotherapy, and Molecular Profiling|url=https://ascopubs.org/doi/10.1200/EDBK_160831|journal=American Society of Clinical Oncology Educational Book|issue=36|pages=e194–e203|doi=10.1200/EDBK_160831|issn=1548-8748}}</ref>

Kolangiokarsinoma jarang terjadi di negara Barat, dengan perkiraan kejadian 0,5-2 dari 100.000 orang per tahun.<ref name=":0" /><ref name=":3" />Kolangiokarsinoma lebih banyak diidap oleh masyarakat di [[Asia Tenggara]] di mana banyak terjadi kasus infeksi oleh [[cacing hati]].<ref name=":4">{{Cite book|last=Bosman|first=Frank T.|date=2014|url=https://publications.iarc.fr/_publications/media/download/5839/bc44643f904185d5c8eddb933480b5bc18b21dba.pdf|title=World Cancer Report 2014|location=Lyon, France|publisher=International Agency for Research on Cancer WHO|isbn=978-92-832-0429-9|pages=403-12|others=Bernard W. Stewart, Christopher P. Wild|oclc=903962851|url-status=live}}</ref> Di [[Thailand]], tingkat kejadian kolangiokarsinoma  adalah 60 dari 100.000 orang per tahun.<ref name=":4" />Kanker ini biasanya terjadi pada orang berusia 70-an. Namun, pada orang yang mengidap penyakit radang saluran empedu (''primary sclerosing cholangitis''), peluang kemungkinan munculnya kanker ini terjadi lebih awal yaitu pada usia 40-an. Tingkat kejadian kolangiokarsinoma di negara Barat telah mengalami peningkatan.

Indikasi paling umum yang ditemukan pada penderita kolangiokarsinoma adalah abnormalitas pada hasil tes fungsi hati, [[penyakit kuning]] (mata dan kulit menguning terjadi ketika [[saluran empedu]] tersumbat oleh [[tumor]]), sakit perut (30-50%), gatal-gatal (66%), penurunan berat badan ( 30–50%), [[demam]] (hingga 20%), dan perubahan warna [[tinja]] atau [[Urine|urin]].<ref>{{Cite journal|last=NagorneyLlano|first=DavidRodrigo M.|last2=Donohue|first2=John H.|last3=Farnell|first3=Michael B.|last4=Schleck|first4=Cathy D.|last5=Ilstrup|first5=Duane M.Castaño|date=1993-08-012011|title=OutcomesHilar AfterCholangiocarcinoma Curative(Klatskin Resections of Cholangiocarcinomatumor)|url=httpshttp://doiwww.scielo.org/10.1001co/pdf/archsurg.1993rcg/v26n2/en_v26n2a08.01420200045008pdf|journal=ArchivesCol of SurgeryGastroenterol|volume=12826|issue=8|pages=871–878|doi=10.1001/archsurg.1993.01420200045008|issn=0004-00102}}</ref> Gejala yang timbul bergantung pada lokasi tumor. Penderita kolangiokarsinoma di saluran empedu ekstrahepatik (di luar hati) lebih mungkin mengalami penyakit kuning, sedangkan penderita kolangiokarsinoma yang tumornya tumbuh pada saluran empedu di dalam hati lebih sering mengalami nyeri tanpa adanya penyakit kuning.<ref name=":7">{{Cite journal|last=Nakeeb|first=Attila|last2=Pitt|first2=Henry A.|last3=Sohn|first3=Taylor A.|last4=Coleman|first4=JoAnn|last5=Abrams|first5=Ross A.|last6=Piantadosi|first6=Steven|last7=Hruban|first7=Ralph H.|last8=Lillemoe|first8=Keith D.|last9=Yeo|first9=Charles J.|date=1996-10|title=Cholangiocarcinoma: A Spectrum of Intrahepatic, Perihilar, and Distal Tumors|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1235406/pdf/annsurg00032-0063.pdf|journal=Annals of Surgery|language=en-US|volume=224|issue=4|pages=463–475|doi=10.1097/00000658-199610000-00005|issn=0003-4932|pmc=PMC1235406|pmid=8857851}}</ref>

Tes fungsi hati pada orang dengan kolangiokarsinoma sering menunjukkan apa yang disebut "gambaran obstruktif", dengan peningkatan kadar [[bilirubin]], alkalin fosfatase, dan gama glutamil transferase, dan kadar transaminase yang relatif normal. Temuan laboratorium tersebut menunjukkan terjadinya obstruksi saluran empedu sebagai penyebab utama penyakit kuning bukan peradangan atau infeksi parenkim hati.<ref name=":6">{{Cite bookjournal|last=FeldmanRoche|first=MarkSean P.|last2=FriedmanKobos|first2=Lawrence S.Rebecca|last3date=Brandt2004|first3title=LawrenceJaundice Jin the Adult Patient|url=https://www.aafp.org/afp/2004/0115/afp20040115p299.pdf|journal=American Family Physician|volume=69|issue=2|access-date=20062022-02-20|archive-date=2022-03-03|archive-url=https://web.archive.org/detailsweb/sleisengerfordtr0000unse_j1o520220303084806/pagehttps:/1493/www.aafp.org/afp/2004/0115/afp20040115p299.pdf|titledead-url=Sleisengeryes}}</ref><ref>{{Cite andjournal|last=Fargo|first=Matthew Fordtran'sV.|last2=Grogan|first2=Scott GastrointestinalP.|last3=Saguil|first3=Aaron|date=2017|title=Evaluation andof LiverJaundice Diseasein (8thAdults|url=https://www.aafp.org/afp/2017/0201/afp20170201p164.pdf|journal=American ed)Family Physician|isbnvolume=97895|issue=3|access-1date=2022-416002-0245-120|pagesarchive-date=14932022-602-20|archive-url=https://web.archive.org/web/20220220113833/https://www.aafp.org/afp/2017/0201/afp20170201p164.pdf|dead-statusurl=liveyes}}</ref>

Meskipun kebanyakan pengidap tidak memiliki faktor risiko, sejumlah faktor risiko untuk perkembangan kolangiokarsinoma dapat dideskripsikan. Di negara Barat, faktor risiko yang paling umum adalah ''[[:en:Primary_sclerosing_cholangitis|primary sclerosing cholangitis]]'' (PSC), penyakit radang [[saluran empedu]] yang terkait erat dengan [[:en:Ulcerative_colitis|kolitis ulserativaulseratif]] (UC).<ref>{{Cite journal|last=Chapman|first=R.W.|date=1999|title=Risk factors for biliary tract carcinogenesis|url=https://www.researchgateannalsofoncology.netorg/publicationarticle/S0923-7534(20)31169-8/12862628_Risk_factors_for_biliary_tract_carcinogenesispdf|journal=Annals of Oncology|volume=10|issue=2|doi=DOI: 10.1093/annonc/10.suppl_4.S308}}</ref> Studi [[Epidemiologi|epidemiologis]] menyebutkan bahwa seseorang yang mengidap ''primary sclerosing cholangitis'' (PSC) akan lebih berisiko sebesar 10-15% untuk terkena kolangiokarsinoma.<ref>{{Cite journal|last=Bergquist|first=Annika|last2=Ekbom|first2=Anders|last3=Olsson|first3=Rolf|last4=Kornfeldt|first4=Dan|last5=Lööf|first5=Lars|last6=Danielsson|first6=Åke|last7=Hultcrantz|first7=Rolf|last8=Lindgren|first8=Stefan|last9=Prytz|first9=Hanne|date=2002-03-01|title=Hepatic and extrahepatic malignancies in primary sclerosing cholangitis|url=https://www.journal-of-hepatology.eu/article/S0168-8278(01)00288-4/abstract|journal=Journal of Hepatology|language=English|volume=36|issue=3|pages=321–327|doi=10.1016/S0168-8278(01)00288-4|issn=0168-8278}}</ref><ref>{{Cite journal|last=Bergquist|first=Annika|last2=Glaumann|first2=Hans|last3=Persson|first3=Bo|last4=Broomé|first4=Ulrika|date=1998|title=Risk factors and clinical presentation of hepatobiliary carcinoma in patients with primary sclerosing cholangitis: A case-control study|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/hep.510270201|journal=Hepatology|language=en|volume=27|issue=2|pages=311–316|doi=10.1002/hep.510270201|issn=1527-3350}}</ref><ref>{{Cite journal|last=Burak|first=Kelly|last2=Angulo|first2=Paul|last3=Pasha|first3=Tousif M|last4=Egan|first4=Kathleen|last5=Petz|first5=Jan|last6=Lindor|first6=Keith D|date=2004-03|title=Incidence and Risk Factors for Cholangiocarcinoma in Primary Sclerosing Cholangitis|url=https://sites.ualberta.ca/~loewen/Medicine/GIM%20Residents%20Core%20Reading/CIRRHOSIS,%20ALCOHOL,%20PBC,%20PSC,%20AUTOIMMUNE%20HEP/PSC%20incidence,%20risks.pdf|journal=American Journal of Gastroenterology|volume=99|issue=3|pages=523–526|doi=10.1111/j.1572-0241.2004.04067.x|issn=0002-9270}}</ref> Meskipun, dari suatu seri [[otopsi]] telah menemukan risiko yang lebih tinggi yaitu sebesar 30% pada penderita ''primary sclerosing cholangitis'' (PSC) untuk terkena kolangiokarsinoma.<ref name=":8">{{Cite journal|last=Rosen|first=Charles B.|last2=Nagorney|first2=David M.|last3=Wiesner|first3=Russell H.|last4=Coffey|first4=ROBERT J. Jr|last5=Larusso|first5=Nicholas F.|date=1991-01|title=Cholangiocarcinoma Complicating Primary Sclerosing Cholangitis|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1358305/|journal=Annals of Surgery|language=en-US|volume=213|issue=1|pages=21–25|doi=10.1097/00000658-199101000-00004|issn=0003-4932|pmc=PMC1358305|pmid=1845927}}</ref>

Penyakit [[hati]] yang disebabkan oleh [[parasit]] tertentu dapat juga menjadi faktor risiko dari kolangiokarsinoma. Kolonisasi dengan [[cacing hati]] ''[[Opisthorchis viverrini|Opisthorchis viverrine]]'' (ditemukan di [[Thailand]], [[Laos]], dan [[Vietnam]])<ref>{{Cite journal|last=Watanapa|first=P|last2=Watanapa|first2=W B|date=2002-08-01|title=Liver fluke-associated cholangiocarcinoma|url=https://doi.org/10.1046/j.1365-2168.2002.02143.x|journal=British Journal of Surgery|volume=89|issue=8|pages=962–970|doi=10.1046/j.1365-2168.2002.02143.x|issn=0007-1323}}</ref><ref>{{Cite journal|last=Sripa|first=Banchob|last2=Kaewkes|first2=Sasithorn|last3=Sithithaworn|first3=Paiboon|last4=Mairiang|first4=Eimorn|last5=Laha|first5=Thewarach|last6=Smout|first6=Michael|last7=Pairojkul|first7=Chawalit|last8=Bhudhisawasdi|first8=Vajaraphongsa|last9=Tesana|first9=Smarn|date=2007-07-10|title=Liver Fluke Induces Cholangiocarcinoma|url=https://journals.plos.org/plosmedicine/article/file?id=10.1371/journal.pmed.0040201&type=printable|journal=PLOS Medicine|language=en|volume=4|issue=7|pages=e201|doi=10.1371/journal.pmed.0040201|issn=1549-1676|pmc=PMC1913093|pmid=17622191}}</ref> atau ''[[:en:Clonorchis_sinensis|Clonorchis sinensis]]'' (ditemukan di [[Tiongkok|Cina]], [[Republik Tiongkok|Taiwan]], [[Rusia Timur Jauh|Rusia Timur]], [[Korea]], dan [[Vietnam]])<ref>{{Cite journalbook|last=RustagiLocke|first=TarunVictoria|last2=DasanuKusnik|first2=ConstantinAlexander|last3=Richardson|first3=Melissa AS.|date=2012-062022-01|title=Risk Factors for Gallbladder Cancer and Cholangiocarcinoma: Similarities, Differences and Updates-09|url=https://doiwww.org/10ncbi.nlm.1007nih.gov/books/NBK532892/s12029-011-9284-y|journaltitle=JournalClonorchis of GastrointestinalSinensis|publisher=StatPearls CancerPublishing|language=en|volumepmid=43|issue=2|pages=137–147|doi=10.1007/s12029-011-9284-y|issn=1941-663630422487}}</ref><ref>{{Cite journal|last=HongKim|first=SungTong-TaeSoo|last2=FangPak|first2=YueyiJhang Ho|last3=Kim|first3=Jong-Bo|last4=Bahk|first4=Young Yil|date=20122016-0311-0130|title=Clonorchis sinensis, an oriental andliver clonorchiasisfluke, anas updatea human biological agent of cholangiocarcinoma: a brief review|url=https://www.sciencedirectncbi.comnlm.nih.gov/sciencepmc/articlearticles/piiPMC5346318/S1383576911000766pdf/bmb-49-590.pdf|journal=ParasitologyBMB International|series=Opisthorchiasis and clonorchiasis: Major neglected tropical diseases in EurasiaReports|language=en|volume=6149|issue=111|pages=17–24590–597|doi=10.10165483/jBMBRep.parint2016.201149.0611.007109|issn=13831976-57696696|pmc=PMC5346318|pmid=27418285}}</ref> telah dikaitkan dengan perkembangan kolangiokarsinoma. Program pengendalian (''Integrated Opisthorchiasis Control Program'') yang bertujuan untuk mengurangi konsumsi makanan mentah dan setengah matang telah berhasil menurunkan angka kejadian kolangiokarsinoma di beberapa negara.<ref>{{Cite journal|last=Sripa|first=Banchob|last2=Tangkawattana|first2=Sirikachorn|last3=Sangnikul|first3=Thinnakorn|date=2017-08-01|title=The Lawa model: A sustainable, integrated opisthorchiasis control program using the EcoHealth approach in the Lawa Lake region of Thailand|url=https://europepmc.org/backend/ptpmcrender.fcgi?accid=PMC5443708&blobtype=pdf|journal=Parasitology International|series=Special Issue: Opisthorchiasis: 100 Years of Discovery|language=en|volume=66|issue=4|pages=346–354|doi=10.1016/j.parint.2016.11.013|issn=1383-5769|pmc=PMC5443708|pmid=27890720}}</ref> Orang dengan penyakit hati kronis, baik berupa [[hepatitis]] akibat [[virus]] (misalnya [[hepatitis B]] atau [[hepatitis C]]),<ref>{{Cite journal|last=Kobayashi|first=Masahiro|last2=Ikeda|first2=Kenji|last3=Saitoh|first3=Satoshi|last4=Suzuki|first4=Fumitaka|last5=Tsubota|first5=Akihito|last6=Suzuki|first6=Yoshiyuki|last7=Arase|first7=Yasuji|last8=Murashima|first8=Naoya|last9=Chayama|first9=Kazuaki|date=2000|title=Incidence of primary cholangiocellular carcinoma of the liver in Japanese patients with hepatitis C virus–related cirrhosis|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/1097-0142%2820000601%2988%3A11%3C2471%3A%3AAID-CNCR7%3E3.0.CO%3B2-T|journal=Cancer|language=en|volume=88|issue=11|pages=2471–2477|doi=10.1002/1097-0142(20000601)88:11<2471::AID-CNCR7>3.0.CO;2-T|issn=1097-0142}}</ref><ref>{{Cite journal|last=Yamamoto|first=Satoshi|last2=Kubo|first2=Shoji|last3=Hai|first3=Seikan|last4=Uenishi|first4=Takahiro|last5=Yamamoto|first5=Takatsugu|last6=Shuto|first6=Taichi|last7=Takemura|first7=Shigekazu|last8=Tanaka|first8=Hiromu|last9=Yamazaki|first9=Osamu|date=2004|title=Hepatitis C virus infection as a likely etiology of intrahepatic cholangiocarcinoma|url=https://onlinelibrary.wiley.com/doi/abs/10.1111/j.1349-7006.2004.tb02492.x|journal=Cancer Science|language=en|volume=95|issue=7|pages=592–595|doi=10.1111/j.1349-7006.2004.tb02492.x|issn=1349-7006}}</ref><ref>{{Cite journal|last=Huiying|first=Lu|last2=Ye|first2=Michele Q.|last3=Thung|first3=Swan N.|last4=Dash|first4=Srikanta|last5=Gerber|first5=Michael A.|date=2000|title=Detection of hepatitis C virus RNA sequences in cholangiocarcinomas in Chinese and American patients|url=https://medcentral.net/doi/pdf/10.3760/cma.j.issn.0366-6999.2000.12.116|journal=Chinese Medical Journal|volume=113|issue=12|pages=1138-1141|doi=https://doi.org/10.3760/cma.j.issn.0366-6999.2000.12.116}}</ref> penyakit hati pada [[alkoholik]], atau [[sirosis hati]], secara signifikan meningkatkan risiko kolangiokarsinoma.<ref name=":9">{{Cite journal|last=Shaib|first=Yasser H.|last2=El-Serag|first2=Hashem B.|last3=Davila|first3=Jessica A.|last4=Morgan|first4=Robert|last5=McGlynn|first5=Katherine A.|date=2005-03-01|title=Risk factors of intrahepatic cholangiocarcinoma in the United States: A case-control study|url=https://www.gastrojournal.org/article/S0016-5085(04)02351-0/abstract|journal=Gastroenterology|language=English|volume=128|issue=3|pages=620–626|doi=10.1053/j.gastro.2004.12.048|issn=0016-5085|pmid=15765398}}</ref><ref>{{Cite journal|last=Sørensen|first=Henrik Toft|last2=Friis|first2=Søren|last3=Olsen|first3=Jørgen H.|last4=Thulstrup|first4=Ane Marie|last5=Mellemkjær|first5=Lene|last6=Linet|first6=Martha|last7=Trichopoulos|first7=Dimitrios|last8=Vilstrup|first8=Hendrik|last9=Olsen|first9=Jørn|date=1998|title=Risk of liver and other types of cancer in patients with cirrhosis: A nationwide cohort study in Denmark|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/hep.510280404|journal=Hepatology|language=en|volume=28|issue=4|pages=921–925|doi=10.1002/hep.510280404|issn=1527-3350}}</ref> Infeksi [[HIV]] juga teridentifikasi dalam satu penelitian sebagai faktor risiko potensial untuk kolangiokarsinoma, meskipun tidak jelas apakah penyebab utamanya adalah HIV itu sendiri atau terdapat faktor lain yang berkorelasi (misalnya infeksi [[hepatitis C]]).

[[Berkas:Digestive system showing bile duct.svg|jmpl|Diagram [[sistem pencernaan]] yang menunjukkan lokasi [[saluran empedu]]]]Kolangiokarsinoma dapat mempengaruhi area di [[saluran empedu]], baik di dalam maupun di luar [[hati]]. [[Tumor]] yang terjadi di saluran empedu di dalam hati disebut sebagai intrahepatik, sementara yang terjadi di saluran di luar hati adalah ekstrahepatik, dan tumor yang terjadi di tempat keluarnya saluran empedu dari hati dapat disebut sebagai perihilar. Kolangiokarsinoma yang terjadi pada pertemuan di mana duktus hepatik bagian kiri dan kanan bertemu untuk membentuk [[:en:Common_hepatic_duct|duktus hepatikus]] dapat disebut sebagai [[:en:Klatskin_tumor|tumor Klatskin]].<ref>{{Cite journal|last=KlatskinPatel|first=GeraldTushar|date=19652011-02-0104|title=Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis: An unusual tumor with distinctive clinicalCholangiocarcinoma—controversies and pathological featureschallenges|url=https://www.amjmedncbi.comnlm.nih.gov/articlepmc/0002-9343(65)90178-6articles/PMC3888819/pdf/abstractnihms533803.pdf|journal=TheNature AmericanReviews JournalGastroenterology of& MedicineHepatology|language=Englishen|volume=388|issue=24|pages=241–256189–200|doi=10.10161038/0002-9343(65)90178-6nrgastro.2011.20|issn=00021759-93435045|pmc=PMC3888819|pmid=21460876}}</ref>

Meskipun kolangiokarsinoma diketahui memiliki gambaran [[Histologi|histologis]] dan molekuler dari [[:en:Adenocarcinoma|adenokarsinoma]] [[sel epitel]] yang melapisi saluran empedu, asal [[Sel (biologi)|sel]] sebenarnya tidak diketahui. Bukti terbaru menunjukkan bahwa sel transformasi awal yang menghasilkan [[tumor]] primer mungkin muncul dari [[sel punca]] pluripotentpluripoten hepatik.<ref>{{Cite journal|last=Roskams|first=T.|date=2006-06|title=Liver stem cells and their implication in hepatocellular and cholangiocarcinoma|url=https://www.nature.com/articles/1209558|journal=Oncogene|language=en|volume=25|issue=27|pages=3818–3822|doi=10.1038/sj.onc.1209558|issn=1476-5594}}</ref><ref>{{Cite journal|last=Liu|first=Chao|date=2004|title=Possible stem cell origin of human cholangiocarcinoma|url=http://www.wjgnet.com/1007-9327/full/v10/i22/3374.htm|journal=World Journal of Gastroenterology|language=en|volume=10|issue=22|pages=3374|doi=10.3748/wjg.v10.i22.3374|issn=1007-9327|pmc=PMC4572317|pmid=15484322}}</ref><ref>{{Cite journal|last=Sell|first=S.|last2=Dunsford|first2=H. A.|date=1989|title=Evidence for the stem cell origin of hepatocellular carcinoma and cholangiocarcinoma.|url=https://www.ncbi.nlm.nih.gov/pmc/articles/PMC1879951/|journal=The American Journal of Pathology|volume=134|issue=6|pages=1347–1363}}</ref> Kolangiokarsinoma diperkirakan berkembang melalui serangkaian tahap dari [[Hiperplasi|hiperplasia]] dan [[metaplasia]] dini, melalui [[displasia]], hingga perkembangan karsinoma yang nyata   dalam proses yang serupa dengan yang terlihat pada perkembangan [[kanker usus besar]]. <ref name=":5">{{Cite journal|last=Sirica|first=Alphonse E.|date=2005|title=Cholangiocarcinoma: Molecular targeting strategies for chemoprevention and therapy|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/hep.20537|journal=Hepatology|language=en|volume=41|issue=1|pages=5–15|doi=10.1002/hep.20537|issn=1527-3350}}</ref> Peradangan kronis dan obstruksi saluran empedu, dan aliran empedu yang terganggu, dianggap berperan dalam perkembangan kolangiokarsinoma.<ref name=":5" /><ref>{{Cite journal|last=Holzinger|first=F.|last2=Z'graggen|first2=K.|last3=Buchler|first3=M.W.|date=1999|title=Mechanisms of biliary carcinogenesis: A pathogenetic multi-stage cascade towards cholangiocarcinoma|url=https://www.annalsofoncology.org/article/S0923-7534(20)31124-8/pdf|journal=Annals of Oncology|volume=10|issue=4}}</ref><ref>{{Cite journal|last=Gores|first=G|date=2003-05|title=Cholangiocarcinoma: Current concepts and insights|url=https://doi.org/10.1053/jhep.2003.50200|journal=Hepatology|volume=37|issue=5|pages=961–969|doi=10.1053/jhep.2003.50200|issn=0270-9139}}</ref>

Secara histologis, kolangiokarsinoma dapat bervariasi mulai dari tidak berdiferensiasi hingga berdiferensiasi dengan baik. Sel-sel kanker tersebut sering dikelilingi oleh responrespons jaringan fibrotik atau desmoplastik yang cepat dengan adanya [[fibrosis]] yang luas sehingga akan sulit untuk membedakan kolangiokarsinoma yang berdiferensiasi dengan baik dari epitel normal. Tidak ada pewarnaan imunohistokimia yang sepenuhnya spesifik yang dapat membedakan jaringan ganas pada kantungkantong empedu dari jaringan jinak pada kantungkantong empedu. Namun, pewarnaan untuk [[:en:Cytokeratin|sitokeratin]], [[antigen karsinoembrionik]], dan [[:en:Mucin|musin]] dapat membantu dalam diagnosis.<ref>{{Cite journal|last=de GroenWard|first=PietStephen C.|last2=GoresHuang|first2=Gregory J.Jiaoti|last3=LaRussoTickoo|first3=NicholasSatish FK.|last4=GundersonThung|first4=LeonardSwan LN.|last5=NagorneyLadanyi|first5=Marc|last6=Klimstra|first6=David MS.|date=1999-102010-2809|title=BiliaryFibrolamellar Tractcarcinoma Cancersof the liver exhibits immunohistochemical evidence of both hepatocyte and bile duct differentiation|url=https://doiwww.org/10nature.1056com/NEJM199910283411807articles/modpathol2010105|journal=New England JournalModern of MedicinePathology|language=en|volume=34123|issue=189|pages=1368–13781180–1190|doi=10.10561038/NEJM199910283411807modpathol.2010.105|issn=00281530-4793|pmid=105361300285}}</ref> Sebagian besar [[tumor]] yang terbentuk (>90%) adalah [[:en:Adenocarcinoma|adenokarsinoma]].<ref name=":10">{{Cite journal|last=Henson|first=Donald Earl|last2=Albores-Saavedra|first2=Jorge|last3=Corle|first3=Donald|date=1992|title=Carcinoma of the extrahepatic bile ducts. Histologic types, stage of disease, grade, and survival rates|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/1097-0142%2819920915%2970%3A6%3C1498%3A%3AAID-CNCR2820700609%3E3.0.CO%3B2-C|journal=Cancer|language=en|volume=70|issue=6|pages=1498–1501|doi=10.1002/1097-0142(19920915)70:6<1498::AID-CNCR2820700609>3.0.CO;2-C|issn=1097-0142}}</ref>

[[Berkas:Cholangiocarcinoma - high mag.jpg|jmpl|Mikrograf intrahepatik, yaitu di dalam [[hati]], kolangiokarsinoma (gambar sebelah kanan dari gambar); [[hepatosit]] jinak terlihat (gambar sebelah kiri). Secara histologis, gambar ini menunjukkan kolangiokarsinoma sebagai (1) sel [[saluran empedu]] yang tidak normal (gambar sebelah kiri) memanjang dari [[tumor]] dalam septum interlobular (lokasi anatomi normal saluran empedu) dan (2) tumor yang memiliki stroma desmoplastik berlimpah yang sering terlihat pada kolangiokarsinoma. [[:en:Portal_triad|Portal triad]] (gambar sebelah kiri atas) memiliki saluran empedu yang normal secara histologis. |195x195px]]Tidak ada [[:en:Blood_test|tes darah]] khusus yang dapat dilakukan mendiagnosis kolangiokarsinoma. Pada kondisi kolangiokarsinoma, kadar [[:en:Carcinoembryonic_antigen|serum antigen karsinoembrionik]] (CEA) dan [[:en:CA19-9|CA19-9]] sering kali mengalami peningkatan, tetapi hal tersebut tidak cukup sensitif atau spesifik untuk digunakan sebagai alat skrining umum. Namun, penentuan kadar serum antigen karsinoembrionik (CEA) dan CA19-9   dapat berguna   pada metode pencitraan dalam mendukung diagnosis kolangiokarsinoma.<ref>{{Cite journal|last=Siqueira|first=Eduardo|last2=Schoen|first2=Robert E.|last3=Silverman|first3=William|last4=Martini|first4=John|last5=Rabinovitz|first5=Mordechai|last6=Weissfeld|first6=Joel L.|last7=Elmaagd|first7=Kareem Abu|last8=Madariaga|first8=Juan R.|last9=Slivka|first9=Adam|date=2002-07-01|title=Detecting cholangiocarcinoma in patients with primary sclerosing cholangitis|url=https://www.giejournal.org/article/S0016-5107(02)00005-6/abstract|journal=Gastrointestinal Endoscopy|language=English|volume=56|issue=1|pages=40–47|doi=10.1067/mge.2002.125105|issn=0016-5107}}</ref><ref>{{Cite journal|last=Coelho|first=Rosa|last2=Silva|first2=Marco|last3=Rodrigues-Pinto|first3=Eduardo|last4=Cardoso|first4=Hélder|last5=Lopes|first5=Susana|last6=Pereira|first6=Pedro|last7=Vilas-Boas|first7=Filipe|last8=Santos-Antunes|first8=João|last9=Costa-Maia|first9=José|date=2017|title=CA 19-9 as a Marker of Survival and a Predictor of Metastization in Cholangiocarcinoma|url=https://www.karger.com/Article/FullText/452691|journal=GE - Portuguese Journal of Gastroenterology|language=en|volume=24|issue=3|pages=114–121|doi=10.1159/000452691|issn=2341-4545|pmc=PMC5553366|pmid=28848795}}</ref><ref>{{Cite journal|last=Patel|first=Anand H.|last2=Harnois|first2=Denise M.|date=2000|title=The Utility of CA 19-9 in the

[[Ultrasonografi medis|Ultrasonografi]] pada [[hati]] dan [[saluran empedu]] sering digunakan sebagai pencitraan awal pada orang dengan dugaan obstruktif penyakit kuning ([[jaundis]]).<ref>{{Cite journal|last=SainiFadahunsi|first=SanjayOlufunke O.|last2=Ibitoye|first2=Bolanle O.|last3=Adisa|first3=Adewale O.|last4=Alatise|first4=Olusegun I.|last5=Adetiloye|first5=Victor A.|last6=Idowu|first6=Bukunmi Michael|date=19972020-06-2615|title=ImagingDiagnostic accuracy of theultrasonography in adults with Hepatobiliaryobstructive Tractjaundice|url=https://doiwww.orgncbi.nlm.nih.gov/10.1056pmc/NEJM199706263362607articles/PMC7409548/pdf/jou-20-81-e100.pdf|journal=New England Journal of MedicineUltrasonography|volume=33620|issue=2681|pages=1889–1894e100–e105|doi=10.105615557/NEJM199706263362607JoU.2020.0016|issnpmc=0028-4793PMC7409548|pmid=919721832609962}}</ref> Ultrasonografi dapat mengidentifikasi obstruksi dan dilatasi saluran dan dalam beberapa kasus, cukup untuk mendiagnosis kolangiokarsinoma.<ref>{{Cite journal|last=Bloom|first=Carl M.|last2=Langer|first2=Bernard|last3=Wilson|first3=Stephanie R.|date=1999-09-01|title=Role of US in the Detection, Characterization, and Staging of Cholangiocarcinoma|url=https://pubs.rsna.org/doi/10.1148/radiographics.19.5.g99se081199|journal=RadioGraphics|volume=19|issue=5|pages=1199–1218|doi=10.1148/radiographics.19.5.g99se081199|issn=0271-5333}}</ref> [[Tomografi terkomputasi|Pemindaian tomografi terkomputasi]] (''CT Scan'') juga memainkan peran penting dalam proses diagnosis kolangiokarsinoma.<ref>{{Cite journal|last=Valls|first=C.|last2=Gumà|first2=A.|last3=Puig|first3=I.|last4=Sanchez|first4=A.|last5=Andía|first5=E.|last6=Serrano|first6=T.|last7=Figueras|first7=J.|date=2000-09-01|title=Intrahepatic peripheral cholangiocarcinoma: CT evaluation|url=https://doi.org/10.1007/s002610000079|journal=Abdominal Imaging|language=en|volume=25|issue=5|pages=490–496|doi=10.1007/s002610000079|issn=1432-0509}}</ref><ref>{{Cite journal|last=Tillich|first=M|last2=Mischinger|first2=H J|last3=Preisegger|first3=K H|last4=Rabl|first4=H|last5=Szolar|first5=D H|date=1998-09-01|title=Multiphasic helical CT in diagnosis and staging of hilar cholangiocarcinoma.|url=https://www.ajronline.org/doi/10.2214/ajr.171.3.9725291|journal=American Journal of Roentgenology|volume=171|issue=3|pages=651–658|doi=10.2214/ajr.171.3.9725291|issn=0361-803X}}</ref><ref>{{Cite journal|last=ZhangOlthof|first=YanSusann-Cathrin|last2=UchidaOthman|first2=MasafumiAhmed|last3=AbeClasen|first3=ToshiStephan|last4=NishimuraSchraml|first4=HiroshiChristina|last5=HayabuchiNikolaou|first5=NaofumiKonstantin|last6=NakashimaBongers|first6=YutakaMalte|date=1999-092016|title=IntrahepaticImaging Peripheralof Cholangiocarcinoma: Comparison of Dynamic CT and Dynamic MRI|url=https://journalswww.lwwncbi.comnlm.nih.gov/jcatpmc/Abstractarticles/1999PMC5290452/09000pdf/Intrahepatic_Peripheral_Cholangiocarcinoma_.4vis-0032-0402.aspxpdf|journal=JournalVisceral of Computer Assisted TomographyMedicine|language=en-US|volume=2332|issue=56|pages=670–677402–410|doi=10.10971159/00004728-199909000-00004000453009|issn=03632297-87154725|pmc=PMC5290452|pmid=28229074}}</ref>

Meskipun pencitraan abdominal dapat berguna dalam diagnosis kolangiokarsinoma, pencitraan langsung dari [[saluran empedu]] sering kali tetap diperlukan. E''ndoscopic retrograde cholangiopancreatography'' (ERCP), prosedur [[endoskopi]] yang dilakukan oleh gastroenterolog atau dokter bedah yang terlatih khusus, telah banyak dilakukan. Meskipun ERCP adalah prosedur invasif dengan risiko yang menyertainya, keuntungannya adalah mencakup kemampuan untuk mendapatkan [[biopsi]] dan memasang [[:en:Stent|stent]] atau melakukan intervensi lain untuk meredakan obstruksi saluran empedu.<ref name=":6">{{Cite book|last=Feldman|first=Mark|last2=Friedman|first2=Lawrence S.|last3=Brandt|first3=Lawrence J.|date=2006|url=https://archive.org/details/sleisengerfordtr0000unse_j1o5/page/1493|title=Sleisenger and Fordtran's Gastrointestinal and Liver Disease (8th ed)|isbn=978-1-4160-0245-1|pages=1493-6|url-status=live}}</ref> [[Ultrasonografi medis|Ultrasonografi]] endoskopi juga dapat dilakukan pada saat ERCP dan dapat meningkatkan keakuratan biopsi dan menghasilkan informasi tentang invasi dan kelayakan [[kelenjar getah bening]] untuk dapat dibedah atau tidak.<ref>{{Cite journal|last=SugiyamaTamada|first=M.Kiichi|last2=HagiUshio|first2=H.Jun|last3=AtomiSugano|first3=Y.|last4=Saito|first4=M.Kentaro|date=1997-07-012011|title=DiagnosisEndoscopic diagnosis of portalextrahepatic venousbile invasion by pancreatobiliaryduct carcinoma: valueAdvances ofand endoscopiccurrent ultrasonographylimitations|url=https://doiwww.ncbi.org/10nlm.1007nih.gov/s002619900227pmc/articles/PMC3100496/pdf/WJCO-2-203.pdf|journal=AbdominalWorld ImagingJournal of Clinical Oncology|language=en|volume=222|issue=45|pages=434–438203|doi=10.10075306/s002619900227wjco.v2.i5.203|issn=14322218-05094333|pmc=PMC3100496|pmid=21611097}}</ref> Sebagai alternatif untuk ERCP, ''percutaneous transhepatic cholangiography'' (PTC) dapat dilakukan. ''Magnetic resonance cholangiopancreatography'' (MRCP) adalah alternatif noninvasif untuk ERCP.<ref>{{Cite journal|last=Schwartz|first=L H|last2=Coakley|first2=F V|last3=Sun|first3=Y|last4=Blumgart|first4=L H|last5=Fong|first5=Y|last6=Panicek|first6=D M|date=1998-06-01|title=Neoplastic pancreaticobiliary duct obstruction: evaluation with breath-hold MR cholangiopancreatography.|url=https://www.ajronline.org/doi/10.2214/ajr.170.6.9609160|journal=American Journal of Roentgenology|volume=170|issue=6|pages=1491–1495|doi=10.2214/ajr.170.6.9609160|issn=0361-803X}}</ref><ref>{{Cite journal|last=Zidi|first=S. H.|last2=Prat|first2=F.|last3=Guen|first3=O. Le|last4=Rondeau|first4=Y.|last5=Pelletier|first5=G.|date=2000-01-01|title=Performance characteristics of magnetic resonance cholangiography in the staging of malignant hilar strictures|url=https://gut.bmj.com/content/46/1/103|journal=Gut|language=en|volume=46|issue=1|pages=103–106|doi=10.1136/gut.46.1.103|issn=0017-5749|pmc=PMC1727781|pmid=10601064}}</ref><ref>{{Cite journal|last=Lee|first=Moon-Gyu|last2=Park|first2=Kwang Bo|last3=Shin|first3=Yong Moon|last4=Yoon|first4=Hyun Ki|last5=Sung|first5=Kyu Bo|last6=Kim|first6=Myung Hwan|last7=Lee|first7=Sung Gyu|last8=Kang|first8=Eun Mee|date=2003-03-01|title=Preoperative Evaluation of Hilar Cholangiocarcinoma with Contrast-enhanced Three-dimensional Fast Imaging with Steady-state Precession Magnetic Resonance Angiography: Comparison with Intraarterial Digital Subtraction Angiography|url=https://doi.org/10.1007/s00268-002-6701-1|journal=World Journal of Surgery|language=en|volume=27|issue=3|pages=278–283|doi=10.1007/s00268-002-6701-1|issn=1432-2323}}</ref> Terdapat penulis yang menyarankan bahwa MRCP harus menggantikan ERCP dalam diagnosis [[kanker]] [[saluran empedu]] karena dapat lebih akurat menentukan [[tumor]] dan menghindari risiko daridaripada ERCP.<ref>{{Cite journal|last=SzklarukHalefoglu|first=Janio|last2=Tamm|first2=Eric|last3=Charnsangavej|first3=ChusilpAhmet Mesrur|date=2002-102007|title=PreoperativeMagnetic imagingresonance cholangiopancreatography: A useful tool in the evaluation of biliarypancreatic tractand biliary cancersdisorders|url=https://doiwww.org/10ncbi.1016nlm.nih.gov/S1055pmc/articles/PMC4146812/pdf/WJG-3207(02)0003213-72529.pdf|journal=SurgicalWorld Oncology ClinicsJournal of North AmericaGastroenterology|language=en|volume=1113|issue=417|pages=865–8762529|doi=10.10163748/s1055-3207(02)00032-7wjg.v13.i18.2529|issn=10551007-32079327|pmc=PMC4146812|pmid=17551999}}</ref>[[Berkas:CT cholangioca-mark.jpg|jmpl|''CT scan'' yang menunjukkan kolangiokarsinoma]]

[[Bedah|Eksplorasi bedah]] mungkin diperlukan untuk mendapatkan [[biopsi]] yang sesuai dan untuk menentukan [[:en:Cancer_staging|stadium]] seseorang dengan kolangiokarsinoma secara akurat. [[Laparoskopi]] dapat digunakan untuk tujuan penentuan stadium dan dapat menghindari kebutuhan akan prosedur bedah yang lebih invasif, seperti [[laparotomi]], pada beberapa orang.<ref>{{Cite journal|last=Weber|first=Sharon M.|last2=DeMatteo|first2=Ronald P.|last3=Fong|first3=Yuman|last4=Blumgart|first4=Leslie H.|last5=Jarnagin|first5=William R.|date=2002-03|title=Staging Laparoscopy in Patients With Extrahepatic Biliary Carcinoma: Analysis of 100 Patients|url=https://journalswww.lwwncbi.comnlm.nih.gov/annalsofsurgerypmc/Abstractarticles/2002PMC1422445/03000pdf/Staging_Laparoscopy_in_Patients_With_Extrahepatic.1120020300s00011p392.aspxpdf|journal=Annals of Surgery|language=en-US|volume=235|issue=3|pages=392–399|doi=10.1097/00000658-200203000-00011|issn=0003-4932|pmc=PMC1422445|pmid=11882761}}</ref>

Secara [[Histologi|histologis]], kolangiokarsinoma secara klasik adalah adenokarsinoma yang berdiferensiasi baik hingga sedang. Imunohistokimia berguna dalam diagnosis dan dapat digunakan untuk membantu membedakan kolangiokarsinoma dari [[karsinoma]] hepatoseluler dan [[metastasis]] [[tumor]] gastrointestinal lainnya.<ref>{{Cite journal|last=LängerTakahashi|first=F.Yoshihisa|last2=v. WasielewskiDungubat|first2=R.Erdenetsogt|last3=KreipeKusano|first3=H. H.Hiroyuki|last4=Ganbat|first4=Dariimaa|last5=Tomita|first5=Yasuhiko|last6=Odgerel|first6=Sarandelger|last7=Fukusato|first7=Toshio|date=2006-072021-01|title=BedeutungApplication derof ImmunhistochemieImmunohistochemistry fürin diethe DiagnosePathological desDiagnosis Cholangiokarzinomsof Liver Tumors|url=https://doiwww.org/10mdpi.1007com/s00292-0061422-0836-z0067/22/11/5780|journal=DerInternational Journal of Molecular PathologeSciences|language=deen|volume=2722|issue=411|pages=244–2505780|doi=10.10073390/s00292-006-0836-zijms22115780|issn=14321422-19630067|pmc=PMC8198626|pmid=34071338}}</ref> Kuretase sitologi yang sering kali dilakukan terkadang tidak dapat mendiagnosis<ref>{{Cite web|last=Darwin|first=Peter E.|date=2022-01-12|title=Cholangiocarcinoma|url=https://emedicine.medscape.com/article/277393-overview|website=medscape|access-date=22-02-10}}</ref> karena tumor ini biasanya memiliki stroma desmoplastik yang tidak melepaskan sel tumor diagnostik melalui proses pengkuretan.

Meskipun setidaknya ada tiga sistem penentuan stadium untuk kolangiokarsinoma (misalnya Bismuth, Blumgart, dan [[:en:American_Joint_Committee_on_Cancer|American Joint Committee on Cancer]]), tidak ada yang terbukti berguna dalam memprediksi kelangsungan hidup.<ref>{{Cite journal|last=ZervosJarnagin|first=EmmanuelWilliam ER.|last2=OsborneFong|first2=DanaYuman|last3=GoldinDeMatteo|first3=StevenRonald BP.|last4=VilladolidGonen|first4=Desiree V.Mithat|last5=ThometzBurke|first5=DonaldEdmund PC.|last6=DurkinBodniewicz|first6=AlanJessica|last7=CareyYoussef|first7=Larry C.Miranda|last8=RosemurgyKlimstra|first8=AlexanderDavid|last9=Blumgart|first9=Leslie SH.|date=20052001-11-0110|title=StageStaging, doesResectability, notand predictOutcome survivalin after225 resectionPatients ofWith hilarHilar cholangiocarcinomas promoting an aggressive operative approachCholangiocarcinoma:|url=https://www.americanjournalofsurgeryncbi.comnlm.nih.gov/articlepmc/S0002-9610(05)00653-7articles/abstractPMC1422074/pdf/20011000s00010p507.pdf|journal=The American JournalAnnals of Surgery|language=Englishen|volume=190234|issue=54|pages=810–815507–519|doi=10.10161097/j.amjsurg.2005.07.02500000658-200110000-00010|issn=00020003-96104932|pmc=PMC1422074|pmid=1622696311573044}}</ref> Masalah penentuan stadium yang paling penting adalah apakah tumor dapat diangkat melalui pembedahan atau apakah terlalu dini untuk melakukan pembedahan pada [[tumor]]. Seringkali, penentuan ini hanya dapat dilakukan pada saat pembedahan berlangsung.<ref name=":6" />

Pedoman umum untuk pembedahan meliputi:<ref>{{Cite journal|last=Tsao|first=Jane I.|last2=Nimura|first2=Yuji|last3=Kamiya|first3=Junichi|last4=Hayakawa|first4=Naokazu|last5=Kondo|first5=Satoshi|last6=Nagino|first6=Masato|last7=Miyachi|first7=Masahiko|last8=Kanai|first8=Michio|last9=Uesaka|first9=Katsuhiko|date=2000-08|title=Management of Hilar Cholangiocarcinoma: Comparison of an American and a Japanese Experience|url=https://journalswww.lwwncbi.comnlm.nih.gov/annalsofsurgerypmc/Abstractarticles/2000PMC1421125/08000pdf/Management_of_Hilar_Cholangiocarcinoma__Comparison.320000800s00003p166.aspxpdf|journal=Annals of Surgery|language=en-US|volume=232|issue=2|pages=166–174|doi=10.1097/00000658-200008000-00003|issn=0003-4932|pmc=PMC1421125|pmid=10903592}}</ref><ref>{{Cite journal|last=Rajagopalan|first=Vandana|last2=Grossbard|first2=Michael L.|last3=Kozuch|first3=Peter|date=2004|title=Gallbladder and Biliary Tract Carcinoma: A Comprehensive Update, Part 1|url=https://www.cancernetwork.com/view/journalgallbladder-lookingand-aheadbiliary-whattract-scarcinoma-nextcomprehensive-forupdate-hematology-oncology-in-2022part-1|journal=Oncology|language=en|volume=18|issue=7}}</ref>

Kolangiokarsinoma dianggap sebagai penyakit yang tidak dapat disembuhkan dan bersifat letal kecuali semua tumor dapat sepenuhnya direseksi (diangkat melalui pembedahan). Dalam beberapa kasus, kemampuan tumor untuk dapat dibedah hanya dapat diketahui selama pembedahan berlangsung,<ref>{{Cite journal|last=Su|first=Cheng-Hsi|last2=Tsay|first2=Shyh-Haw|last3=Wu|first3=Cheng-Chung|last4=Shyr|first4=Yi-Ming|last5=King|first5=Kuang-Liang|last6=Lee|first6=Chen-Hsen|last7=Lui|first7=Wing-Yiu|last8=Liu|first8=Tse-Jia|last9=P'eng|first9=Fang-Ku|date=1996-04|title=Factors Influencing Postoperative Morbidity, Mortality, and Survival After Resection for Hilar Cholangiocarcinoma|url=https://journalswww.lwwncbi.comnlm.nih.gov/annalsofsurgerypmc/Abstractarticles/1996PMC1235134/04000pdf/Factors_Influencing_Postoperative_Morbidity,.7annsurg00038-0048.aspxpdf|journal=Annals of Surgery|language=en-US|volume=223|issue=4|pages=384–394|doi=10.1097/00000658-199604000-00007|issn=0003-4932|pmc=PMC1235134|pmid=8633917}}</ref> mayoritas penderita akan menjalani eksplorasi pembedahan kecuali terdapat indikasi yang jelas bahwa terhadap [[tumor]] tidak dapat dilakukan pembedahan.<ref name=":6" /> Namun, [[Mayo Clinic]] telah melaporkan keberhasilan yang signifikan dalam mengobati [[kanker]] [[saluran empedu]] dini dengan [[transplantasi]] [[hati]] menggunakan pendekatan protokol dan kriteria seleksi yang ketat.<ref>{{Cite journal|last=Rosen|first=C. B.|last2=Heimbach|first2=J. K.|last3=Gores|first3=G. J.|date=2008-06-01|title=Surgery for cholangiocarcinoma: the role of liver transplantation|url=https://www.hpbonline.org/article/S1365-182X(15)30051-4/abstract|journal=HPB|language=English|volume=10|issue=3|pages=186–189|doi=10.1080/13651820801992542|issn=1365-182X|pmc=PMC2504373|pmid=18773052}}</ref> [[:en:Adjuvant_therapy|Terapi adjuvan]] diikuti dengan transplantasi hati dapat memiliki peran tertentu dalam pengobatan terhadap tumor yang tidak dapat dibedah pada beberapa kasus tertentu.<ref>{{Cite journal|last=Heimbach|first=Julie K.|last2=Gores|first2=Gregory J.|last3=Haddock|first3=Michael G.|last4=Alberts|first4=Steven R.|last5=Pedersen|first5=Rachel|last6=Kremers|first6=Walter|last7=Nyberg|first7=Scott L.|last8=Ishitani|first8=Michael B.|last9=Rosen|first9=Charles B.|date=2006-12-27|title=Predictors of Disease Recurrence Following Neoadjuvant Chemoradiotherapy and Liver Transplantation for Unresectable Perihilar Cholangiocarcinoma|url=https://journals.lww.com/transplantjournal/Fulltext/2006/12270/Predictors_of_Disease_Recurrence_Following.34.aspx|journal=Transplantation|language=en-US|volume=82|issue=12|pages=1703–1707|doi=10.1097/01.tp.0000253551.43583.d1|issn=0041-1337}}</ref> Terapi lokoregional termasuk kemoembolisasi transarterial (TACE), radioembolisasi transarterial (TARE), dan terapi ablasi memiliki peran dalam varian intrahepatik kolangiokarsinoma untuk memberikan [[perawatan paliatif]] atau penyembuhan potensial pada penderita kolangiokarsinoma yang bukan kandidat layak untuk dibedah.<ref>{{Cite journal|last=Kuhlmann|first=Jan B.|last2=Blum|first2=Hubert E.|date=2013-05|title=Locoregional therapy for cholangiocarcinoma|url=https://journalswww.lwwncbi.nlm.comnih.gov/co-gastroenterologypmc/Abstractarticles/2013PMC5777616/05000pdf/Locoregional_therapy_for_cholangiocarcinomahep-04-99.15.aspxpdf|journal=Current Opinion in Gastroenterology|language=en-US|volume=29|issue=3|pages=324–328|doi=10.1097/MOG.0b013e32835d9dea|issn=0267-1379}}</ref>

Jika [[tumor]] dapat diangkat melalui [[pembedahan]], penderita akan menerima [[kemoterapi]] [[:en:Adjuvant_therapy|adjuvan]] atau [[terapi radiasi]] setelah operasi untuk meningkatkan kemungkinan penyembuhan. Jika margin jaringan negatif (yaitu tumor telah dieksisi total), kemoterapi adjuvan tidak dapat dipasti manfaatnya. Hasil positif<ref>{{Cite journal|last=TodorokiDoherty|first=TakeshiMark K.|last2=OharaKnox|first2=Kiyoshi|last3=Kawamoto|first3=Toru|last4=Koike|first4=Naoto|last5=Yoshida|first5=Sadao|last6=Kashiwagi|first6=Hironobu|last7=Otsuka|first7=Masaaki|last8=Fukao|first8=KatashiJennifer J.|date=20002016-02-0110|title=BenefitsAdjuvant oftherapy adjuvantfor radiotherapyresected afterbiliary radicaltract resection of locally advanced main hepaticcancer: ducta carcinomareview|url=https://wwwcco.redjournalamegroups.orgcom/article/S0360-3016(99)00472-1view/11561/abstract12687|journal=InternationalChinese Journal of RadiationClinical Oncology, Biology, Physics|language=English|volume=465|issue=35|pages=581–58764–64|doi=10.101621037/S0360-3016(99)00472-1|issn=0360-3016|pmid=10701737}}</ref><ref>{{Cite journal|last=Alden|first=Mark Ecco.|last2=Mohiuddin|first2=Mohammed|date=1994-03-01|title=The impact of radiation dose in combined external beam and intraluminal IR-192 brachytherapy for bile duct cancer|url=https://www2016.redjournal08.org/article/0360-3016(94)90115-5/abstract|journal=International Journal of Radiation Oncology, Biology, Physics|language=English|volume=28|issue=4|pages=945–951|doi=10.1016/0360-3016(94)90115-5|issn=0360-3016|pmid=813844805}}</ref> dan negatif<ref name=":7" /><ref>{{Cite journal|last=Gonzalez|first=D.Gonziilez|last2=Gouma|first2=D.J.|last3=Rauws|first3=E.A.J.|last4=Gulik|first4=T.M. van|last5=Bosma|first5=A.|last6=Koedooder|first6=C.|date=1999|title=Role of radiotherapy, in particular intraluminal brachytherapy, in the treatment

of proximal bile duct carcinoma|url=https://www.annalsofoncology.org/article/S0923-7534(20)31148-0/pdf|journal=Annals of Oncology|volume=10|issue=4}}</ref><ref>{{Cite journal|last=Pitt|first=Henry A.|last2=Nakeeb|first2=Attila|last3=Abrams|first3=Ross A.|last4=Coleman|first4=JoAnn|last5=Piantadosi|first5=Steven|last6=Yeo|first6=Charles J.|last7=Lillemoe|first7=Keith D.|last8=Cameron|first8=John L.|date=1995-06|title=Perihilar Cholangiocarcinoma Postoperative Radiotherapy Does Not Improve Survival|url=https://journalswww.lwwncbi.comnlm.nih.gov/annalsofsurgerypmc/Abstractarticles/1995PMC1234714/06000pdf/Perihilar_Cholangiocarcinoma_Postoperative.17annsurg00052-0188.aspxpdf|journal=Annals of Surgery|language=en-US|volume=221|issue=6|pages=788–802|doi=10.1097/00000658-199506000-00017|issn=0003-4932|pmc=PMC1234714|pmid=7794082}}</ref> dari terapi radiasi adjuvan telah dilaporkan dan tidak ada uji coba terkontrol acak prospektif yang telah dilakukan pada Maret 2007. Kemoterapi adjuvan tampaknya tidak efektif pada orang dengan tumor yang direseksi total. <ref>{{Cite journal|last=Takada|first=Tadahiro|last2=Amano|first2=Hodaka|last3=Yasuda|first3=Hideki|last4=Nimura|first4=Yuji|last5=Matsushiro|first5=Takashi|last6=Kato|first6=Hiroyuki|last7=Nagakawa|first7=Takukazu|last8=Nakayama|first8=Toshimichi|date=2002|title=Is postoperative adjuvant chemotherapy useful for gallbladder carcinoma?|url=https://onlinelibrary.wiley.com/doi/abs/10.1002/cncr.10831|journal=Cancer|language=en|volume=95|issue=8|pages=1685–1695|doi=10.1002/cncr.10831|issn=1097-0142}}</ref> Peran kemoradioterapi kombinasi dalam pengobatan terhadap kolangiokarsinoma masih belum jelas. Namun, jika margin jaringan tumor positif, menunjukkan bahwa tumor tidak sepenuhnya diangkat melalui pembedahan, maka terapi adjuvan dengan radiasi dan kemungkinan kemoterapi umumnya direkomendasikan berdasarkan data yang tersedia.

Mayoritas kasus pada kolangikarsinoma bersifat tidak dapat dilakukan pembedahan (tidak dapat dioperasi).<ref name=":11">{{Cite journal|last=VautheyBlechacz|first=Jean-NicolasBoris|last2=BlumgartGores|first2=LeslieGregory HJ.|date=19942008-0507|title=RecentCholangiocarcinoma: Advances in thepathogenesis, Managementdiagnosis, ofand Cholangiocarcinomastreatment|url=httphttps://www.thieme-connectncbi.denlm.nih.gov/DOIpmc/DOI?10.1055articles/s-2007-1007302PMC2547491/pdf/nihms60349.pdf|journal=Seminars in Liver DiseaseHepatology|language=en|volume=1448|issue=021|pages=109–114308–321|doi=10.10551002/s-2007-1007302|issn=0272-8087hep.22310}}</ref> Dalam hal ini, penderita umumnya diobati dengan [[kemoterapi]] [[Perawatan paliatif|paliatif]], dengan atau tanpa [[radioterapi]]. Kemoterapi telah ditunjukkan dalam uji coba terkontrol secara acak untuk meningkatkan kualitas hidup dan memperpanjang kelangsungan hidup pada orang dengan kolangiokarsinoma yang tidak dapat dibedah.<ref>{{Cite journal|last=Glimelius|first=B.|last2=Hoffman|first2=K.|last3=Sjödén|first3=P.-O.|last4=Jacobsson|first4=G.|last5=Sellström|first5=H.|last6=Enander|first6=L.-K.|last7=Linné|first7=T.|last8=Svensson|first8=C.|date=1996-08|title=Chemotherapy improves survival and quality of life in advanced pancreatic and biliary cancer|url=https://doi.org/10.1093/oxfordjournals.annonc.a010676|journal=Annals of Oncology|volume=7|issue=6|pages=593–600|doi=10.1093/oxfordjournals.annonc.a010676|issn=0923-7534}}</ref> Tidak ada panduan kemoterapi tunggal yang digunakan secara universal, dan pendaftaran dalam uji klinis sering direkomendasikan bila memungkinkan. Agen kemoterapi yang digunakan untuk mengobati kolangiokarsinoma termasuk [[:en:5-fluorouracilFluorourasil|5-fluorouracilfluorourasil]] dengan [[:en:Leucovorin|leucovorin]],<ref>{{Cite journal|last=ChoiPerkhofer|first=Chul WonL.|last2=ChoiBerger|first2=KeunA. W.|last3=SeoBeutel|first3=JaeA. HongK.|last4=KimGallmeier|first4=Byung SooE.|last5=KimAngermeier|first5=Jae SeonS.|last6=KimFischer von Weikersthal|first6=Chang DukL.|last7=UmGoetze|first7=SoonT. 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Rocha|date=20032013-12|title=GemcitabineA andphase IrinotecanII intrial Locallyof Advancedgemcitabine, oririnotecan Metastaticand Biliarypanitumumab Cancer:in Preliminaryadvanced Reportcholangiocarcinoma|url=https://www.cancernetworkannalsofoncology.comorg/viewaction/gemcitabineshowPdf?pii=S0923-and-irinotecan-locally-advanced7534%2819%2936750-or-metastatic-biliary-cancer-preliminary-reportX|journal=Annals of Oncology|language=en|volume=1724|issue=912|pages=3061–3065|doi=10.1093/annonc/mdt416}}</ref> atau [[capecitabine]].<ref>{{Cite journal|last=Knox|first=Jennifer J.|last2=Hedley|first2=David|last3=Oza|first3=Amit|last4=Feld|first4=Ron|last5=Siu|first5=Lillian L.|last6=Chen|first6=Eric|last7=Nematollahi|first7=Mahsan|last8=Pond|first8=Gregory R.|last9=Zhang|first9=Jessica|date=2005-04-01|title=Combining Gemcitabine and Capecitabine in Patients With Advanced Biliary Cancer: A Phase II Trial|url=https://ascopubs.org/doi/10.1200/JCO.2005.51.008|journal=Journal of Clinical Oncology|volume=23|issue=10|pages=2332–2338|doi=10.1200/JCO.2005.51.008|issn=0732-183X}}</ref> Dalam sebuah studi, dinyatakan adanya manfaat dari [[:en:Erlotinib|erlotinib]] inhibitor tirosin kinase pada penderita kolangiokarsinoma tingkat lanjut.<ref>{{Cite journal|last=Philip|first=Philip A.|last2=Mahoney|first2=Michelle R.|last3=Allmer|first3=Cristine|last4=Thomas|first4=James|last5=Pitot|first5=Henry C.|last6=Kim|first6=George|last7=Donehower|first7=Ross C.|last8=Fitch|first8=Tom|last9=Picus|first9=Joel|date=2006-07-01|title=Phase II Study of Erlotinib in Patients With Advanced Biliary Cancer|url=https://ascopubs.org/doi/10.1200/JCO.2005.05.3579|journal=Journal of Clinical Oncology|volume=24|issue=19|pages=3069–3074|doi=10.1200/JCO.2005.05.3579|issn=0732-183X}}</ref> Terapi radiasi diamati mampu memperpanjang kelangsungan hidup pada orang dengan kolangiokarsinoma ekstrahepatik yang direseksi,<ref>{{Cite journal|last=Beltrán|first=Marta Bonet|last2=Allal|first2=Abdelkarim S.|last3=Gich|first3=Ignasi|last4=Solé|first4=Josep M.|last5=Carrió|first5=Ignasi|date=2012-04-01|title=Is adjuvant radiotherapy needed after curative resection of extrahepatic biliary tract cancers? A systematic review with a meta-analysis of observational studies|url=https://www.cancertreatmentreviewsncbi.comnlm.nih.gov/articlebooks/S0305-7372(11)00080-6NBK91637/abstract|journal=Cancer Treatment Reviews|language=English|volume=38|issue=2|pages=111–119|doi=10.1016/j.ctrv.2011.05.003|issn=0305-7372|pmid=21652148}}</ref> dan beberapa laporan tentang penggunaannya pada kolangiokarsinoma yang tidak dapat direseksi menunjukkan peningkatan kelangsungan hidup, tetapi jumlahnya kecil.<ref name=":3" />

Reseksi bedah merupakan satu-satunya kemungkinan penyembuhan yang potensial pada kolangiokarsinoma. Untuk kasus kolangiokarsinoma yang tidak dapat dibedah, tingkat kelangsungan hidup dalam lima tahun ke depan adalah 0% di mana penyakit ini tidak dapat dioperasi karena [[kelenjar getah bening]] distal menunjukkan [[metastasis]],<ref>{{Cite journal|last=Yamamoto|first=Masakazu|last2=Takasaki|first2=Ken|last3=Yoshikawa|first3=Tatsuya|date=1999-03-01|title=Lymph Node Metastasis in Intrahepatic Cholangiocarcinoma|url=https://doi.org/10.1093/jjco/29.3.147|journal=Japanese Journal of Clinical Oncology|volume=29|issue=3|pages=147–150|doi=10.1093/jjco/29.3.147|issn=0368-2811}}</ref> dan kurang dari 5% pada umumnya.<ref>{{Cite journal|last=FarleyLauterio|first=David R.Andrea|last2=WeaverDe Carlis|first2=Amy L.Riccardo|last3=NagorneyCentonze|first3=DavidLeonardo|last4=Buscemi|first4=Vincenzo|last5=Incarbone|first5=Niccolò|last6=Vella|first6=Ivan|last7=De M.Carlis|first7=Luciano|date=1995-052021-01|title=“NaturalCurrent History”Surgical Management of UnresectedPeri-Hilar Cholangiocarcinoma:and PatientIntra-Hepatic Outcome After Noncurative InterventionCholangiocarcinoma|url=https://www.mayoclinicproceedingsmdpi.orgcom/article2072-6694/S0025-6196(11)63877-913/15/3657/abstractpdf|journal=Mayo Clinic ProceedingsCancers|language=Englishen|volume=7013|issue=515|pages=425–4293657|doi=10.40653390/70.5.425cancers13153657|issn=00252072-61966694|pmc=PMC8345178|pmid=34359560}}</ref> Durasi rata-rata kelangsungan hidup keseluruhan kurang dari 6 bulan pada penderita yang tumornya sudah bermetastasis.<ref>{{Cite journal|last=GroveZhu|first=MarkAndrew KX.|last2=HermannMacarulla|first2=Robert E.Teresa|last3=VogtJavle|first3=DavidMilind PM.|last4=BroughanKelley|first4=ThomasR. AKate|last5=Lubner|first5=Sam J.|last6=Adeva|first6=Jorge|last7=Cleary|first7=James M.|last8=Catenacci|first8=Daniel V. T.|last9=Borad|first9=Mitesh J.|date=19912021-0411-01|title=RoleFinal Overall Survival Efficacy Results of radiationIvosidenib afterfor operativePatients palliationWith inAdvanced cancerCholangiocarcinoma ofWith theIDH1 proximalMutation: The Phase 3 Randomized Clinical bileClarIDHy ductsTrial|url=https://wwwdoi.americanjournalofsurgeryorg/10.com1001/articlejamaoncol.2021.3836|journal=JAMA Oncology|volume=7|issue=11|pages=1669–1677|doi=10.1001/0002-9610(91)91111jamaoncol.2021.3836|issn=2374-U/abstract2437|pmc=PMC8461552|pmid=34554208}}</ref><ref>{{Cite journal|last=TheTreeprasertsuk|first=Sombat|last2=Poovorawan|first2=Kittiyod|last3=Soonthornworasiri|first3=Ngamphol|last4=Chaiteerakij|first4=Roongruedee|last5=Thanapirom|first5=Kessarin|last6=Mairiang|first6=Pisaln|last7=Sawadpanich|first7=Kookwan|last8=Sonsiri|first8=Kanokwan|last9=Mahachai|first9=Varocha|date=2017-12|title=A Americansignificant Journalcancer burden and high mortality of Surgeryintrahepatic cholangiocarcinoma in Thailand: a nationwide database study|url=https://bmcgastroenterol.biomedcentral.com/track/pdf/10.1186/s12876-016-0565-6.pdf|journal=BMC Gastroenterology|language=Englishen|volume=16117|issue=41|pages=454–4583|doi=10.10161186/0002s12876-9610(91)91111016-U0565-6|issn=00021471-9610230X|pmc=PMC5216607|pmid=170979528056836}}</ref>

Untuk kasus kolangiokarsinoma yang dapat bedah, kemungkinan penyembuhan bervariasi tergantung pada lokasi [[tumor]] dan apakah tumor dapat diangkat seluruhnya, atau hanya sebagian. 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Berbagai seri penelitian telah melaporkan perkiraan kelangsungan hidup setelah operasi mulai dari 22 hingga 66%. Hasil tersebut dapat bergantung pada keterlibatan kelenjar getah bening dan kelengkapan operasi.<ref>{{Cite journal|last=Nakeeb|first=Attila|last2=Pitt|first2=Henry A.|last3=Sohn|first3=Taylor A.|last4=Coleman|first4=JoAnn|last5=Abrams|first5=Ross A.|last6=Piantadosi|first6=Steven|last7=Hruban|first7=Ralph H.|last8=Lillemoe|first8=Keith D.|last9=Yeo|first9=Charles J.|date=1996-10|title=Cholangiocarcinoma: A Spectrum of Intrahepatic, Perihilar, and Distal Tumors|url=https://journals.lww.com/annalsofsurgery/Abstract/1996/10000/Cholangiocarcinoma__A_Spectrum_of_Intrahepatic,.5.aspx|journal=Annals of Surgery|language=en-US|volume=224|issue=4|pages=463–475|doi=10.1097/00000658-199610000-00005|issn=0003-4932|pmc=PMC1235406|pmid=8857851}}</ref><ref>{{Cite journal|last=LieserMare|first=MarkGiulio J.Di|last2=BarryMarrelli|first2=M. 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[[Prognosis]] mungkin lebih buruk bagi pengidap ''[[:en:Primary_sclerosing_cholangitis|primary sclerosing cholangitis]]'' yang mengembangkan kolangiokarsinoma, kemungkinan karena kanker tidak terdeteksi sampai stadium lanjut.<ref name=":8" /> Beberapa bukti menunjukkan bahwa hasil mungkin membaik dengan pendekatan bedah yang lebih intensif dan [[:en:Adjuvant_therapy|terapi adjuvan]].<ref>{{Cite journal|last=NakeebChen|first=AttilaXiuqiong|last2=TranMeng|first2=Khoi Q.Fanqiao|last3=BlackXiong|first3=Michael J.Hua|last4=EricksonZou|first4=Beth A.Yanmei|last5date=Ritch2021|first5title=PaulAdjuvant S.|last6=Quebbeman|first6=EdwardTherapy J.|last7=Wilson|first7=Stuartfor D.|last8=Demeure|first8=MichaelResectable J.|last9=Rilling|first9=WilliamBiliary S.|date=2002-10-01|title=ImprovedTract survivalCancer: inA resectedBayesian biliaryNetwork malignanciesAnalysis|url=https://www.surgjournalfrontiersin.comorg/article/S0039-6060(02)00133-210.3389/abstractfonc.2021.600027|journal=Surgery|language=EnglishFrontiers in Oncology|volume=132|issue=4|pages=555–56411|doi=10.10673389/msyfonc.20022021.127555600027|issn=00392234-6060943X|pmc=PMC7991284|pmid=1240733833777744}}</ref>

[[Berkas:Liver tumor types in adults by relative incidence.png|jmpl|Jenis tumor pada hati yang banyak terjadi pada orang dewasa di Amerika Serikat, dengan kolangiokarsinoma ada pada peringkat atas<ref>{{Cite web|last=Themes|first=U. F. O.|date=2016-12-27|title=Masses of the Liver|url=https://basicmedicalkey.com/masses-of-the-liver-2/|website=Basicmedical Key|language=en-US|access-date=2022-02-10}}</ref>]]Kolangiokarsinoma adalah bentuk [[kanker]] yang relatif jarang terjadi. Setiap tahun, sekitar 2.000 hingga 3.000 kasus baru didiagnosis di [[Amerika Serikat]], yang berarti 1-2 kasus per 100.000 orang dilaporkan per tahun.<ref>{{Cite journal|last=Landis|first=S. H.|last2=Murray|first2=T.|last3=Bolden|first3=S.|last4=Wingo|first4=P. A.|date=1998-01-01|title=Cancer statistics, 1998|url=https://doi.org/10.3322/canjclin.48.1.6|journal=CA: A Cancer Journal for Clinicians|volume=48|issue=1|pages=6–29|doi=10.3322/canjclin.48.1.6|issn=0007-9235}}</ref> Dalam suatu seri [[otopsi]] telah melaporkan prevalensi 0,01% hingga 0,46%.<ref name=":11">{{Cite journal|last=Vauthey|first=Jean-Nicolas|last2=Blumgart|first2=Leslie H.|date=1994-05|title=Recent Advances in the Management of Cholangiocarcinomas|url=http://www.thieme-connect.de/DOI/DOI?10.1055/s-2007-1007302|journal=Seminars in Liver Disease|language=en|volume=14|issue=02|pages=109–114|doi=10.1055/s-2007-1007302|issn=0272-8087|access-date=2022-04-05|archive-date=2018-06-07|archive-url=https://web.archive.org/web/20180607095149/https://www.thieme-connect.de/DOI/DOI?10.1055%2Fs-2007-1007302|dead-url=yes}}</ref><ref>{{Cite web|date=2015-04-02|title=Cancer Statistics - National Cancer Institute|url=https://www.cancer.gov/about-cancer/understanding/statistics|website=www.cancer.gov|language=en|access-date=2022-02-11}}</ref> Terdapat [[prevalensi]] yang lebih tinggi dari kolangiokarsinoma di [[Asia]], yang telah dikaitkan dengan infestasi [[parasit]] kronis endemik. Insiden kolangiokarsinoma meningkat seiring bertambahnya usia dan penyakit ini sedikit lebih sering terjadi pada pria daripada wanita (dimungkinkan karena tingkat ''[[:en:Primary_sclerosing_cholangitis|primary sclerosing cholangitis]]'' sebagai faktor risiko utama tingkat kejadianya lebih tinggi pada pria).<ref name=":10" /> Prevalensi dari kolangiokarsinoma pada orang dengan ''[[:en:Primary_sclerosing_cholangitis|primary sclerosing cholangitis]]'' adalah sebesar 30%, berdasarkan studi otopsi.<ref name=":8" />

Berbagai penelitian telah mendokumentasikan peningkatan yang stabil dalam insiden kolangiokarsinoma intrahepatik. Peningkatan tersebut telah terlihat di [[Amerika Utara]], [[Eropa]], [[Asia]], dan [[Australia]].<ref>{{Cite journal|last=Patel|first=Tushar|date=2002-05-03|title=Worldwide trends in mortality from biliary tract malignancies|url=https://doi.org/10.1186/1471-2407-2-10|journal=BMC Cancer|volume=2|issue=1|pages=10|doi=10.1186/1471-2407-2-10|issn=1471-2407|pmc=PMC113759|pmid=11991810}}</ref><ref>{{Cite journal|last=Patel|first=T|date=2001-06|title=Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States|url=https://doi.org/10.1053/jhep.2001.25087|journal=Hepatology|volume=33|issue=6|pages=1353–1357|doi=10.1053/jhep.2001.25087|issn=0270-9139}}</ref><ref>{{Cite journal|last=Shaib|first=Yasser H.|last2=Davila|first2=Jessica A.|last3=McGlynn|first3=Kathryn|last4=El-Serag|first4=Hashem B.|date=2004-03-01|title=Rising incidence of intrahepatic cholangiocarcinoma in the United States: a true increase?|url=https://www.journal-of-hepatology.eu/article/S0168-8278(03)00627-5/abstract|journal=Journal of Hepatology|language=English|volume=40|issue=3|pages=472–477|doi=10.1016/j.jhep.2003.11.030|issn=0168-8278}}</ref><ref>{{Cite journal|last=West|first=J.|last2=Wood|first2=H.|last3=Logan|first3=R. F. A.|last4=Quinn|first4=M.|last5=Aithal|first5=G. P.|date=2006-06|title=Trends in the incidence of primary liver and biliary tract cancers in England and Wales 1971–2001|url=https://www.nature.com/articles/6603127|journal=British Journal of Cancer|language=en|volume=94|issue=11|pages=1751–1758|doi=10.1038/sj.bjc.6603127|issn=1532-1827|pmc=PMC2361300|pmid=16736026}}</ref><ref name=":13">{{Cite journal|last=Khan|first=Shahid A.|last2=Taylor-Robinson|first2=Simon D.|last3=Toledano|first3=Mireille B.|last4=Beck|first4=Angus|last5=Elliott|first5=Paul|last6=Thomas|first6=Howard C.|date=2002-12-01|title=Changing international trends in mortality rates for liver, biliary and pancreatic tumours|url=https://www.journal-of-hepatology.eu/article/S0168-8278(02)00297-0/abstract|journal=Journal of Hepatology|language=English|volume=37|issue=6|pages=806–813|doi=10.1016/S0168-8278(02)00297-0|issn=0168-8278}}</ref><ref>{{Cite journal|last=Welzel|first=Tania M.|last2=McGlynn|first2=Katherine A.|last3=Hsing|first3=Ann W.|last4=O'Brien|first4=Thomas R.|last5=Pfeiffer|first5=Ruth M.|date=2006-06-21|title=Impact of Classification of Hilar Cholangiocarcinomas (Klatskin Tumors) on the Incidence of Intra- and Extrahepatic Cholangiocarcinoma in the United States|url=https://doi.org/10.1093/jnci/djj234|journal=JNCI: Journal of the National Cancer Institute|volume=98|issue=12|pages=873–875|doi=10.1093/jnci/djj234|issn=0027-8874}}</ref> Alasan peningkatan terjadinya kolangiokarsinoma belum bisa dijelaskan. Metode diagnostik yang lebih baik mungkin dapat membantu, tetapi prevalensi faktor risiko potensial untuk kolangiokarsinoma, seperti infeksi [[HIV]] juga telah mengalami peningkatan.<ref name=":9" />

|+Usia kematian standar dari kolangiokarsinoma intrahepatik (IC) dan ekstrahepatik (EC) untuk pria dan wanita berdasarkan negara<ref name=":13">{{Cite journal|last=Khan|first=Shahid A.|last2=Taylor-Robinson|first2=Simon D.|last3=Toledano|first3=Mireille B.|last4=Beck|first4=Angus|last5=Elliott|first5=Paul|last6=Thomas|first6=Howard C.|date=2002-12-01|title=Changing international trends in mortality rates for liver, biliary and pancreatic tumours|url=https://www.journal-of-hepatology.eu/article/S0168-8278(02)00297-0/abstract|journal=Journal of Hepatology|language=English|volume=37|issue=6|pages=806–813|doi=10.1016/S0168-8278(02)00297-0|issn=0168-8278}}</ref>