Editing Cholangiocarcinoma (section)

===Treatment of advanced disease===
The majority of cases of cholangiocarcinoma present as inoperable (unresectable) disease<ref name="ReferenceA">{{cite journal | vauthors = Vauthey JN, Blumgart LH | title = Recent advances in the management of cholangiocarcinomas | journal = Seminars in Liver Disease | volume = 14 | issue = 2 | pages = 109–14 | date = May 1994 | pmid = 8047893 | doi = 10.1055/s-2007-1007302 | s2cid = 37111064 }}</ref> in which case people are generally treated with [[palliation|palliative]] [[chemotherapy]], with or without [[radiotherapy]]. Chemotherapy has been shown in a [[randomized controlled trial]] to improve [[quality of life]] and extend survival in people with inoperable cholangiocarcinoma.<ref>{{cite journal | vauthors = Glimelius B, Hoffman K, Sjödén PO, Jacobsson G, Sellström H, Enander LK, Linné T, Svensson C | display-authors = 6 | title = Chemotherapy improves survival and quality of life in advanced pancreatic and biliary cancer | journal = Annals of Oncology | volume = 7 | issue = 6 | pages = 593–600 | date = August 1996 | pmid = 8879373 | doi = 10.1093/oxfordjournals.annonc.a010676 | doi-access = free }}</ref> There is no single chemotherapy regimen which is universally used, and enrollment in [[clinical trial]]s is often recommended when possible.<ref name="nccn2021"/> Chemotherapy agents used to treat cholangiocarcinoma include [[5-fluorouracil]] with [[leucovorin]],<ref>{{cite journal | vauthors = Choi CW, Choi IK, Seo JH, Kim BS, Kim JS, Kim CD, Um SH, Kim JS, Kim YH | display-authors = 6 | title = Effects of 5-fluorouracil and leucovorin in the treatment of pancreatic-biliary tract adenocarcinomas | journal = American Journal of Clinical Oncology | volume = 23 | issue = 4 | pages = 425–8 | date = August 2000 | pmid = 10955877 | doi = 10.1097/00000421-200008000-00023 }}</ref> [[gemcitabine]] as a single agent,<ref>{{cite journal | vauthors = Park JS, Oh SY, Kim SH, Kwon HC, Kim JS, Jin-Kim H, Kim YH | display-authors = 6 | title = Single-agent gemcitabine in the treatment of advanced biliary tract cancers: a phase II study | journal = Japanese Journal of Clinical Oncology | volume = 35 | issue = 2 | pages = 68–73 | date = February 2005 | pmid = 15709089 | doi = 10.1093/jjco/hyi021 | doi-access = free }}</ref> or gemcitabine plus [[cisplatin]],<ref>{{cite journal | vauthors = Giuliani F, Gebbia V, Maiello E, Borsellino N, Bajardi E, Colucci G | title = Gemcitabine and cisplatin for inoperable and/or metastatic biliary tree carcinomas: a multicenter phase II study of the Gruppo Oncologico dell'Italia Meridionale (GOIM) | journal = Annals of Oncology | volume = 17 | issue = Suppl 7 | pages = vii73–7 | date = June 2006 | pmid = 16760299 | doi = 10.1093/annonc/mdl956 | doi-access = free }}</ref> [[irinotecan]],<ref>{{cite journal | vauthors = Bhargava P, Jani CR, Savarese DM, O'Donnell JL, Stuart KE, Rocha Lima CM | title = Gemcitabine and irinotecan in locally advanced or metastatic biliary cancer: preliminary report | journal = Oncology | volume = 17 | issue = 9 Suppl 8 | pages = 23–6 | date = September 2003 | pmid = 14569844 }}</ref> or [[capecitabine]].<ref>{{cite journal | vauthors = Knox JJ, Hedley D, Oza A, Feld R, Siu LL, Chen E, Nematollahi M, Pond GR, Zhang J, Moore MJ | display-authors = 6 | title = Combining gemcitabine and capecitabine in patients with advanced biliary cancer: a phase II trial | journal = Journal of Clinical Oncology | volume = 23 | issue = 10 | pages = 2332–8 | date = April 2005 | pmid = 15800324 | doi = 10.1200/JCO.2005.51.008 }}</ref> A small pilot study suggested possible benefit from the [[tyrosine kinase]] inhibitor [[erlotinib]] in people with advanced cholangiocarcinoma.<ref>{{cite journal | vauthors = Philip PA, Mahoney MR, Allmer C, Thomas J, Pitot HC, Kim G, Donehower RC, Fitch T, Picus J, Erlichman C | display-authors = 6 | title = Phase II study of erlotinib in patients with advanced biliary cancer | journal = Journal of Clinical Oncology | volume = 24 | issue = 19 | pages = 3069–74 | date = July 2006 | pmid = 16809731 | doi = 10.1200/JCO.2005.05.3579 }}</ref>
Radiation therapy appears to prolong survival in people with resected extrahepatic cholangiocarcinoma,<ref>{{cite journal|vauthors=Bonet Beltrán M, Allal AS, Gich I, et al. |title=Is adjuvant radiotherapy needed after curative resection of extrahepatic biliary tract cancers? A systematic review with a meta-analysis of observational studies |journal=Cancer Treat Rev.|year=2012|volume=38|issue=2 |pages=111–119|doi= 10.1016/j.ctrv.2011.05.003|pmid=21652148}}</ref> and the few reports of its use in unresectable cholangiocarcinoma appear to show improved survival, but numbers are small.<ref name=Brid2016 />

[[Infigratinib]] (Truseltiq) is a [[tyrosine kinase inhibitor]] of [[fibroblast growth factor receptor]] (FGFR) that was approved for medical use in the United States in May 2021.<ref name="BridgeBio Pharma PR">{{cite press release | title=BridgeBio Pharma's Affiliate QED Therapeutics and Partner Helsinn Group Announce FDA Approval of Truseltiq (infigratinib) for Patients with Cholangiocarcinoma | publisher=BridgeBio Pharma | via=GlobeNewswire | date=28 May 2021 | url=https://www.globenewswire.com/news-release/2021/05/28/2238443/0/en/BridgeBio-Pharma-s-Affiliate-QED-Therapeutics-and-Partner-Helsinn-Group-Announce-FDA-Approval-of-TRUSELTIQ-infigratinib-for-Patients-with-Cholangiocarcinoma.html | access-date=28 May 2021}}</ref> It is [[Indication (medicine)|indicated]] for the treatment of people with previously treated locally advanced or metastatic cholangiocarcinoma harboring an FGFR2 fusion or rearrangement.<ref name="BridgeBio Pharma PR" />

[[Pemigatinib]] (Pemazyre) is a kinase inhibitor of [[fibroblast growth factor receptor 2]] (FGFR2) that was approved for medical use in the United States in April 2020.<ref>{{Cite web |date=2020 |title=Pemazyre Prescribing Information |url=https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213736s000lbl.pdf |url-status=dead |access-date=5 March 2022 |website=Food and Drug Administration|archive-url=https://web.archive.org/web/20200720125745/https://www.accessdata.fda.gov/drugsatfda_docs/label/2020/213736s000lbl.pdf |archive-date=20 July 2020 }}</ref> It is indicated for the treatment of adults with previously treated, unresectable locally advanced or metastatic cholangiocarcinoma with a fibroblast growth factor receptor 2 (FGFR2) fusion or other rearrangement as detected by an FDA-approved test.

[[Ivosidenib|Ivodesinib]] (Tibsovo) is a small molecule inhibitor of [[IDH1|isocitrate dehydrogenase 1]]. The FDA approved ivosidenib in August 2021 for adults with previously treated, locally advanced or metastatic cholangiocarcinoma with an isocitrate dehydrogenase-1 (IDH1) mutation as detected by an FDA-approved test.<ref>{{Cite journal |last=Research |first=Center for Drug Evaluation and |date=2022-02-01 |title=FDA approves ivosidenib for advanced or metastatic cholangiocarcinoma |url=https://www.fda.gov/drugs/resources-information-approved-drugs/fda-approves-ivosidenib-advanced-or-metastatic-cholangiocarcinoma |journal=FDA |language=en}}</ref>

[[Durvalumab]], (Imfinzi) is an immune checkpoint inhibitor that blocks the PD-L1 protein on the surface of immune cells, thereby allowing the immune system to recognize and attack tumor cells. In Phase III clinical trials, durvalumab, in combination with standard-of-care chemotherapy, demonstrated a statistically significant and clinically meaningful improvement in [[Survival rate|overall survival]] and [[progression-free survival]] versus chemotherapy alone as a 1st-line treatment for patients with advanced biliary tract cancer.<ref>{{Cite web |title=Imfinzi plus chemotherapy reduced risk of death by 20% in 1st-line advanced biliary tract cancer |url=https://www.astrazeneca.com/media-centre/press-releases/2022/imfinzi-plus-chemotherapy-reduced-risk-of-death-by-20-in-1st-line-advanced-biliary-tract-cancer.html |access-date=2022-03-05 |website=www.astrazeneca.com |date=18 January 2022 |language=en}}</ref>

[[Futibatinib]] (Lytgobi) was approved for medical use in the United States in September 2022.<ref>{{cite press release | title=FDA Approves Taiho's Lytgobi (futibatinib) Tablets for Previously Treated, Unresectable, Locally Advanced or Metastatic Intrahepatic Cholangiocarcinoma | publisher=Taiho Oncology | via=PR Newswire | date=30 September 2022 | url=https://www.prnewswire.com/news-releases/fda-approves-taihos-lytgobi-futibatinib-tablets-for-previously-treated-unresectable-locally-advanced-or-metastatic-intrahepatic-cholangiocarcinoma-301638254.html | access-date=4 October 2022}}</ref>