Cholangiocarcinoma

Table of Contents

1. Overview
2. Epidemiology
3. Pathophysiology
4. Etiology and Risk Factors
5. Clinical Presentation
6. Diagnosis
7. Treatment
8. Prognosis
9. Prevention and Surveillance
10. Recent Research
11. Key Research Papers
12. PubMed Topic Searches
13. Connections
14. Featured Videos

1. Overview

Cholangiocarcinoma is grouped by where it arises in the biliary tree. A 2018 review describes each of the three subtypes as having a distinct epidemiology, biology, prognosis and management (PubMed PMID: 28994423):

• Intrahepatic CCA (iCCA): arises from bile ducts inside the liver. The incidence of cholangiocarcinoma, particularly iCCA, has increased globally over the past few decades (PubMed PMID: 28994423).
• Perihilar CCA (pCCA, Klatskin tumour): arises at the hilum, where the right and left hepatic ducts join. A 2014 review describes management of the bile-duct blockage as obligatory in pCCA, and liver transplantation as a curative option for selected patients with pCCA but not with intrahepatic or distal tumours (PubMed PMID: 24581682).
• Distal CCA (dCCA): arises in the common bile duct further down, outside the liver.

In the BILCAP trial report, the authors note that only about 20% of patients with biliary tract cancer are eligible for surgery with curative intent, and that 5-year overall survival is below 10% for all patients (PubMed PMID: 30922733).

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2. Epidemiology

The liver fluke Opisthorchis viverrini is endemic in Southeast Asia, where more than 10 million people are estimated to be infected; northeast Thailand is a hotspot for transmission, and a 2017 review reports that infection remained common there despite extensive government prevention campaigns (PubMed PMID: 28754415). A 2021 review of the Greater Mekong Subregion reports more than 12 million O. viverrini infections across Thailand, Lao PDR, Cambodia and central-southern Vietnam, and an estimated 3.5 to 5 million Clonorchis sinensis infections in northern Vietnam and Guangxi; the main risk behaviour it describes is the regional culture of eating raw fish (PubMed PMID: 34509453).

Outside these regions, a 2026 international consensus statement describes a global increase in CCA incidence and mortality, linked to greater awareness, better diagnosis and the rising prevalence of some risk factors, and notes that most cases are sporadic, with no identifiable underlying condition (PubMed PMID: 41372578).

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3. Pathophysiology

CCAs are highly heterogeneous at the genomic, epigenetic and molecular levels, and the 2020 consensus statement describes this heterogeneity as a major reason the available therapies work poorly (PubMed PMID: 32606456). Whole-exome and transcriptome sequencing have mapped the genetic landscape of each subtype and identified molecular targets now tested in trials (PubMed PMID: 28994423).

• IDH1 mutations: reported in about 13% of patients with intrahepatic CCA in the ClarIDHy trial report (PubMed PMID: 32416072) and in up to about 20% in its final overall-survival report (PubMed PMID: 34554208).
• FGFR2 fusions or rearrangements: reported in 13% to 17% of intrahepatic CCAs (PubMed PMID: 29182496), or 10% to 16% in the later infigratinib report (PubMed PMID: 34358484).
• HER2 (ERBB2): overexpressed or amplified in a subset of biliary tract cancers (PubMed PMID: 37276871).

Most biliary tract cancers have an immune-suppressed microenvironment, and immune checkpoint inhibitors given alone produce low response rates (PubMed PMID: 37075781). The 2018 review describes the tumour's rich stroma as a possible additional treatment target (PubMed PMID: 28994423). In PSC, a 2020 review describes inflammation-driven carcinogenesis together with genetic and epigenetic abnormalities, and reports that most PSC-associated CCAs develop from a dominant stricture (PubMed PMID: 31463807).

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4. Etiology and Risk Factors

A 2020 meta-analysis of 25 case-control studies from 7 countries found that the known risk factors vary around the world and together account for a minority of cases (PubMed PMID: 31536748):

• Biliary cysts and stones: among the strongest risk factors for both intrahepatic and extrahepatic CCA; choledochal cysts carried the greatest risk, with pooled odds ratios of 26.71 for iCCA and 34.94 for extrahepatic CCA (PubMed PMID: 31536748).
• Cirrhosis, hepatitis B and hepatitis C: also among the strongest risk factors for both forms; the association of hepatitis B with iCCA differed between Eastern and Western studies (PubMed PMID: 31536748). A 2014 review notes that iCCA can arise in cirrhosis and that liver masses in cirrhosis need assessment for CCA (PubMed PMID: 24581682).
• Primary sclerosing cholangitis (PSC): CCA is the most common cancer in people with PSC. A 2020 review reports that 30% to 50% of PSC-associated CCAs are found within the first year after the PSC diagnosis, followed by an annual incidence of 0.5 to 1.5 per 100 people; cumulative incidence is 7% at 5 years, 8% to 11% at 10 years and 9% to 20% over a lifetime. Risk is higher in older men with large-duct PSC, possibly with ulcerative colitis, and lower in children, women, small-duct PSC and PSC with Crohn's disease (PubMed PMID: 31463807).
• Liver flukes: the International Agency for Research on Cancer classifies Opisthorchis viverrini and Clonorchis sinensis as carcinogenic to humans (Group 1); they are covered in IARC Monographs Volume 100B (2012), summarised in PubMed PMID: 19350698. The infections can cause cholangitis, periductal fibrosis, gallstones and CCA (PubMed PMID: 34509453).
• Obesity, hypertension and diabetes: the 2020 meta-analysis found no significant association between obesity or hypertension and either form of CCA; diabetes was a weaker risk factor that is becoming more common globally and may be contributing to rising rates (PubMed PMID: 31536748).

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5. Clinical Presentation

CCAs usually develop and progress silently, and most are found at a stage too late for curative surgery (PubMed PMID: 41372578).

Perihilar and Distal CCA

Tumours at or below the hilum block the flow of bile. In people with PSC, a 2020 review lists a sudden worsening of jaundice, pain, fatigue, itching (pruritus), weight loss or worsening liver blood tests as the features that raise suspicion of CCA (PubMed PMID: 31463807). A 2014 review describes management of the bile-duct blockage as obligatory in perihilar CCA (PubMed PMID: 24581682).

Intrahepatic CCA

Intrahepatic tumours grow inside the liver tissue. A 2014 review notes that iCCA can arise in a cirrhotic liver and that a liver mass in cirrhosis needs assessment for CCA (PubMed PMID: 24581682).

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6. Diagnosis

The 2020 consensus statement reports that non-invasive diagnosis of CCA is not accurate enough and that histological (tissue) confirmation is necessary (PubMed PMID: 32606456). A 2024 review of PSC surveillance describes the available tests as highly specific but of low sensitivity (PubMed PMID: 37945159).

Laboratory Studies

• CA19-9: the serum tumour marker measured in PSC surveillance (PubMed PMID: 37945159). About 10% of patients cannot make CA19-9 because they are Lewis-antigen negative; in one study, 94.7% of Lewis-negative patients had undetectable CA19-9, and the authors conclude that CA19-9 is not useful for diagnosis or follow-up in these patients (PubMed PMID: 30275214).
• Liver blood tests: worsening liver biochemistry is one of the features that raises suspicion of CCA in PSC (PubMed PMID: 31463807).

Imaging

• MRI with MRCP (magnetic resonance cholangiopancreatography): the imaging test most societies include in PSC surveillance, together with CA19-9 (PubMed PMID: 37945159).

Biliary Endoscopy

• ERCP with brush cytology and FISH: a 2014 review describes advanced cytological tests such as fluorescence in situ hybridisation (FISH) for aneusomy as helpful in diagnosing perihilar CCA (PubMed PMID: 24581682).
• Cholangioscopy with targeted biopsies: in a 2016 meta-analysis of 21 studies in PSC, single-operator cholangioscopy with targeted biopsies was the most accurate ERCP-based method, with a pooled sensitivity of 65% and specificity of 97% (PubMed PMID: 27696456).

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7. Treatment

Surgical Resection

Surgical resection remains the mainstay of potentially curative treatment for all three subtypes (PubMed PMID: 28994423). Only about 20% of patients with biliary tract cancer are eligible for curative-intent surgery (PubMed PMID: 30922733); in the BILCAP trial, resection included liver resection, pancreatic resection or, less commonly, both (PubMed PMID: 30922733).

Liver Transplantation

Liver transplantation after neoadjuvant chemoradiation is restricted to a subset of patients with early-stage perihilar CCA (PubMed PMID: 28994423). The Mayo Clinic protocol, first reported in 2000, combined external-beam radiation with fluorouracil, then brachytherapy, then an exploratory operation to exclude spread before transplantation (PubMed PMID: 10827231). In the centre's 1993–2004 series, which enrolled patients with unresectable CCA or CCA arising in PSC, 5-year survival was 82% after transplantation versus 21% after resection, with fewer recurrences (13% versus 27%) (PubMed PMID: 16135931). Across 12 US centres (287 patients, 1993–2010), intention-to-treat survival was 68% at 2 years and 53% at 5 years, and recurrence-free survival after transplantation was 65% at 5 years; patients outside the United Network for Organ Sharing criteria (tumour mass over 3 cm, a biopsy through the abdominal wall, or metastatic disease) or with a prior cancer had significantly shorter survival (PubMed PMID: 22504095).

Systemic Therapy — First-Line

• Gemcitabine + Cisplatin (ABC-02): in 410 patients with advanced biliary tract cancer, median overall survival was 11.7 months with cisplatin plus gemcitabine versus 8.1 months with gemcitabine alone (hazard ratio 0.64), and median progression-free survival was 8.0 versus 5.0 months (PubMed PMID: 20375404).
• Gemcitabine + Cisplatin + Durvalumab (TOPAZ-1): in 685 patients, adding the PD-L1 inhibitor durvalumab to gemcitabine–cisplatin gave a hazard ratio for overall survival of 0.80, an estimated 24-month survival of 24.9% versus 10.4% with placebo, and objective response rates of 26.7% versus 18.7% (PubMed PMID: 38319896). The FDA label lists durvalumab with gemcitabine and cisplatin for locally advanced or metastatic biliary tract cancer.
• Gemcitabine + Cisplatin + Pembrolizumab (KEYNOTE-966): in 1,069 patients, median overall survival was 12.7 months with pembrolizumab versus 10.9 months with placebo (hazard ratio 0.83) (PubMed PMID: 37075781). The FDA approved this combination on October 31, 2023 (FDA notice).

Molecularly Targeted Therapy

• FGFR2 fusions or rearrangements:
  • Pemigatinib (FIGHT-202): an FGFR1/2/3 inhibitor; 38 of 107 patients with FGFR2 fusions or rearrangements (35.5%) had an objective response (PubMed PMID: 32203698). Its FDA label covers previously treated, unresectable locally advanced or metastatic cholangiocarcinoma with an FGFR2 fusion or other rearrangement, under accelerated approval based on response rate and duration of response; Drugs@FDA records the original approval on April 17, 2020.
  • Futibatinib (FOENIX-CCA2): a covalently binding FGFR1–4 inhibitor; 43 of 103 patients (42%) responded, with a median response duration of 9.7 months, median progression-free survival of 9.0 months and overall survival of 21.7 months (PubMed PMID: 36652354). Its FDA label covers previously treated intrahepatic CCA with FGFR2 fusions or rearrangements, under accelerated approval.
  • Infigratinib: in the first phase II report (61 patients with FGFR alterations), the response rate was 14.8% (18.8% in FGFR2 fusions only) and median progression-free survival 5.8 months (PubMed PMID: 29182496); in the mature results for 108 patients with FGFR2 fusions or rearrangements, the centrally assessed response rate was 23.1% (PubMed PMID: 34358484).
  • Lirafugratinib: approved by the FDA on September 23, 2026 for previously treated cholangiocarcinoma with an FGFR2 fusion or rearrangement, based on the single-arm REFOCUS trial in 116 patients (FDA notice).
• IDH1 mutations:
  • Ivosidenib (ClarIDHy): in 185 patients, median progression-free survival was 2.7 months versus 1.4 months with placebo (hazard ratio 0.37) (PubMed PMID: 32416072). In the final analysis, median overall survival was 10.3 versus 7.5 months (hazard ratio 0.79; not statistically significant, one-sided P = .09); 43 placebo patients crossed over to ivosidenib, and after adjustment for crossover the placebo median was 5.1 months (hazard ratio 0.49) (PubMed PMID: 34554208). Its FDA label covers previously treated, locally advanced or metastatic cholangiocarcinoma with an IDH1 mutation.
• HER2:
  • Zanidatamab (HERIZON-BTC-01): a bispecific antibody that binds two sites on HER2; 33 of 80 patients with HER2-positive (IHC 2+ or 3+) biliary tract cancer (41.3%) had a confirmed response (PubMed PMID: 37276871). The FDA granted accelerated approval on November 20, 2024 for previously treated HER2-positive (IHC 3+) biliary tract cancer (FDA notice).

Second-Line Chemotherapy

FOLFOX (folinic acid, fluorouracil, oxaliplatin): in the ABC-06 trial, 162 patients whose biliary tract cancer had progressed on cisplatin and gemcitabine received active symptom control with or without FOLFOX. Median overall survival was 6.2 versus 5.3 months (adjusted hazard ratio 0.69), and 12-month survival 25.9% versus 11.4%; grade 3–5 adverse events occurred in 69% versus 52% (PubMed PMID: 33798493). The authors describe it as the first prospective randomised study to give reliable, high-quality evidence on second-line FOLFOX, and propose FOLFOX as the standard second-line regimen and reference for later trials (PubMed PMID: 33798493).

Adjuvant Therapy

In the BILCAP trial, 447 patients after curative-intent resection received oral capecitabine for eight 21-day cycles or observation. The trial missed its primary endpoint: in the intention-to-treat analysis, median overall survival was 51.1 versus 36.4 months (hazard ratio 0.81, p = 0.097). The prespecified sensitivity analysis (hazard ratio 0.71) and per-protocol analysis (53 versus 36 months, hazard ratio 0.75, p = 0.028) favoured capecitabine (PubMed PMID: 30922733). At a median follow-up of 106 months the intention-to-treat figures were 49.6 versus 36.1 months (hazard ratio 0.84), and the authors report that capecitabine has become the standard of care in this setting (PubMed PMID: 35316080).

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8. Prognosis

Prognosis depends heavily on the stage at diagnosis and whether surgery is possible. Figures reported in the sources on this page:

• All patients with biliary tract cancer: 5-year overall survival below 10% (PubMed PMID: 30922733).
• After curative-intent resection (BILCAP): median overall survival 36.4 months with observation and 51.1 months with capecitabine (PubMed PMID: 30922733); the long-term analysis describes the prognostic impact of resection margin status, tumour grade, lymph-node status and sex (PubMed PMID: 35316080).
• Transplantation for perihilar CCA (12 US centres): intention-to-treat survival 53% at 5 years; recurrence-free survival after transplantation 65% at 5 years (PubMed PMID: 22504095).
• Advanced disease, gemcitabine–cisplatin: median overall survival 11.7 months in ABC-02 (PubMed PMID: 20375404).
• Advanced disease, chemotherapy plus immunotherapy: estimated 24-month survival 24.9% with durvalumab in TOPAZ-1 (PubMed PMID: 38319896); median overall survival 12.7 months with pembrolizumab in KEYNOTE-966 (PubMed PMID: 37075781).
• Targeted therapy after earlier treatment: median progression-free survival 9.0 months with futibatinib in FGFR2-rearranged iCCA (PubMed PMID: 36652354) and 2.7 months with ivosidenib in IDH1-mutant CCA (PubMed PMID: 32416072).
• PSC-associated CCA diagnosed after symptoms appear: median survival under 12 months (PubMed PMID: 37945159).

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9. Prevention and Surveillance

Primary Prevention

• Liver fluke control: after several rounds of national campaigns using an integrated control approach over three decades, the prevalence of O. viverrini infection in Thailand fell from over 15% in 1996 to 2.2% in 2019. High prevalence continues in Lao PDR and central Vietnam, and the 2021 review describes a shift of high-risk areas toward Lao PDR, Cambodia, Vietnam, Myanmar and Guangxi (PubMed PMID: 34509453). A 2017 review attributes persistent transmission to wet-rice farming habitats, a centuries-old raw-food culture and the parasite's complex life cycle (PubMed PMID: 28754415).

Surveillance in High-Risk Populations

• PSC: a 2024 review reports that no surveillance strategy has been widely agreed upon, but that most societies recommend serum CA19-9 and MRCP every 6 to 12 months in people with PSC, and that early identification of CCA leads to better outcomes (PubMed PMID: 37945159). A 2020 review highlights the first year after a PSC diagnosis, older men with large-duct PSC, possibly with ulcerative colitis, as the groups at highest risk (PubMed PMID: 31463807).

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10. Recent Research

The TOPAZ-1 report notes that the first-line standard of care, gemcitabine plus cisplatin, had remained unchanged for more than 10 years (PubMed PMID: 38319896).

• Immunotherapy: pembrolizumab given alone after standard treatment produced responses in 5.8% of 104 patients in KEYNOTE-158 and 13.0% of 23 patients in KEYNOTE-028; the authors describe durable activity in 6% to 13% regardless of PD-L1 expression (PubMed PMID: 32359091). Added to gemcitabine–cisplatin, both durvalumab (TOPAZ-1) and pembrolizumab (KEYNOTE-966) improved overall survival (PubMed PMID: 38319896; PubMed PMID: 37075781).
• FGFR inhibitors and resistance: pemigatinib, futibatinib and lirafugratinib carry FDA approvals for FGFR2-altered cholangiocarcinoma. In a 2025 analysis of 226 patients treated with futibatinib, acquired resistance was often polyclonal and driven by secondary FGFR2 kinase-domain mutations (mainly V565L, V565F and N550K), seen in most patients who had benefited; progression-free survival was shorter with CDKN2B alterations (4.8 versus 11.0 months) (PubMed PMID: 39672383).
• HER2-directed therapy: zanidatamab produced a 41.3% confirmed response rate in HER2-positive biliary tract cancer in HERIZON-BTC-01 (PubMed PMID: 37276871), leading to FDA accelerated approval in 2024 (FDA notice).
• Liquid biopsy: in the same 2025 futibatinib analysis, circulating tumour DNA detected 84% of the FGFR2 fusions or rearrangements found in tissue, and the authors describe ctDNA as a potential non-invasive way to profile tumours and track resistance (PubMed PMID: 39672383).
• Tumour stroma: a 2018 review describes targeting the rich tumour stroma together with targeted therapies as a possible strategy (PubMed PMID: 28994423).
• Tumour-agnostic approvals: the FDA labels of larotrectinib and entrectinib cover solid tumours with an NTRK gene fusion, and the label of dabrafenib with trametinib covers unresectable or metastatic solid tumours with a BRAF V600E mutation that progressed after prior treatment; these indications are not limited to one cancer type.
• Research priorities: a 2026 international consensus statement, endorsed by the European Network for the Study of Cholangiocarcinoma, sets out consensus recommendations and research priorities from a Delphi survey of experts (PubMed PMID: 41372578).

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Key Research Papers

1. Valle JW, Wasan H, Palmer DH, et al. Cisplatin plus gemcitabine versus gemcitabine for biliary tract cancer (ABC-02). N Engl J Med. 2010;362(14):1273–1281. — PubMed PMID: 20375404 · doi:10.1056/NEJMoa0908721
2. Oh DY, He AR, Qin S, et al. Durvalumab plus gemcitabine and cisplatin in advanced biliary tract cancer (TOPAZ-1). NEJM Evid. 2022;1(8):EVIDoa2200015. — PubMed PMID: 38319896 · doi:10.1056/EVIDoa2200015
3. Abou-Alfa GK, Macarulla T, Javle MM, et al. Ivosidenib in IDH1-mutant, chemotherapy-refractory cholangiocarcinoma (ClarIDHy). Lancet Oncol. 2020;21(6):796–807. — PubMed PMID: 32416072 · doi:10.1016/S1470-2045(20)30157-1
4. Abou-Alfa GK, Sahai V, Hollebecque A, et al. Pemigatinib for previously treated, locally advanced or metastatic cholangiocarcinoma with FGFR2 fusions or rearrangements (FIGHT-202). Lancet Oncol. 2020;21(5):671–684. — PubMed PMID: 32203698 · doi:10.1016/S1470-2045(20)30109-1
5. Lamarca A, Palmer DH, Wasan HS, et al. Second-line FOLFOX chemotherapy versus active symptom control for advanced biliary tract cancer (ABC-06). Lancet Oncol. 2021;22(5):690–701. — PubMed PMID: 33798493 · doi:10.1016/S1470-2045(21)00027-9
6. Ilyas SI, Khan SA, Hallemeier CL, Kelley RK, Gores GJ. Cholangiocarcinoma — evolving concepts and therapeutic strategies. Nat Rev Clin Oncol. 2018;15(2):95–111. — PubMed PMID: 28994423 · doi:10.1038/nrclinonc.2017.157
7. Banales JM, Marin JJG, Lamarca A, et al. Cholangiocarcinoma 2020: the next horizon in mechanisms and management. Nat Rev Gastroenterol Hepatol. 2020;17(9):557–588. — PubMed PMID: 32606456 · doi:10.1038/s41575-020-0310-z
8. Razumilava N, Gores GJ. Cholangiocarcinoma. Lancet. 2014;383(9935):2168–2179. — PubMed PMID: 24581682 · doi:10.1016/S0140-6736(13)61903-0
9. De Vreede I, Steers JL, Burch PA, et al. Prolonged disease-free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma. Liver Transpl. 2000;6(3):309–316. — PubMed PMID: 10827231 · doi:10.1053/lv.2000.6143
10. Javle M, Lowery M, Shroff RT, et al. Phase II study of BGJ398 in patients with FGFR-altered advanced cholangiocarcinoma. J Clin Oncol. 2018;36(3):276–282. — PubMed PMID: 29182496 · doi:10.1200/JCO.2017.75.5009
11. Nakanuma Y, Curado MP, Franceschi S, et al. Intrahepatic cholangiocarcinoma. In: Bosman FT, ed. WHO Classification of Tumours of the Digestive System. Lyon: IARC; 2010. — Search PubMed
12. Piha-Paul SA, Oh DY, Ueno M, et al. Efficacy and safety of pembrolizumab for the treatment of advanced biliary cancer: results from the KEYNOTE-158 and KEYNOTE-028 studies. Int J Cancer. 2020;147(8):2190–2198. — PubMed PMID: 32359091 · doi:10.1002/ijc.33013
13. Kelley RK, Ueno M, Yoo C, et al. Pembrolizumab in combination with gemcitabine and cisplatin compared with gemcitabine and cisplatin alone for patients with advanced biliary tract cancer (KEYNOTE-966): a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet. 2023;401(10391):1853-1865. — PubMed PMID: 37075781 · doi:10.1016/S0140-6736(23)00727-4
14. Zhu AX, Macarulla T, Javle MM, et al. Final Overall Survival Efficacy Results of Ivosidenib for Patients With Advanced Cholangiocarcinoma With IDH1 Mutation: The Phase 3 Randomized Clinical ClarIDHy Trial. JAMA Oncol. 2021;7(11):1669-1677. — PubMed PMID: 34554208 · doi:10.1001/jamaoncol.2021.3836
15. Primrose JN, Fox RP, Palmer DH, et al. Capecitabine compared with observation in resected biliary tract cancer (BILCAP): a randomised, controlled, multicentre, phase 3 study. Lancet Oncol. 2019;20(5):663-673. — PubMed PMID: 30922733 · doi:10.1016/S1470-2045(18)30915-X
16. Bridgewater J, Fletcher P, Palmer DH, et al. Long-Term Outcomes and Exploratory Analyses of the Randomized Phase III BILCAP Study. J Clin Oncol. 2022;40(18):2048-2057. — PubMed PMID: 35316080 · doi:10.1200/JCO.21.02568
17. Javle M, Roychowdhury S, Kelley RK, et al. Infigratinib (BGJ398) in previously treated patients with advanced or metastatic cholangiocarcinoma with FGFR2 fusions or rearrangements: mature results from a multicentre, open-label, single-arm, phase 2 study. Lancet Gastroenterol Hepatol. 2021;6(10):803-815. — PubMed PMID: 34358484 · doi:10.1016/S2468-1253(21)00196-5
18. Goyal L, Meric-Bernstam F, Hollebecque A, et al. Futibatinib for FGFR2-Rearranged Intrahepatic Cholangiocarcinoma. N Engl J Med. 2023;388(3):228-239. — PubMed PMID: 36652354 · doi:10.1056/NEJMoa2206834
19. Harding JJ, Fan J, Oh DY, et al. Zanidatamab for HER2-amplified, unresectable, locally advanced or metastatic biliary tract cancer (HERIZON-BTC-01): a multicentre, single-arm, phase 2b study. Lancet Oncol. 2023;24(7):772-782. — PubMed PMID: 37276871 · doi:10.1016/S1470-2045(23)00242-5
20. Rea DJ, Heimbach JK, Rosen CB, et al. Liver transplantation with neoadjuvant chemoradiation is more effective than resection for hilar cholangiocarcinoma. Ann Surg. 2005;242(3):451-8. — PubMed PMID: 16135931 · doi:10.1097/01.sla.0000179678.13285.fa
21. Darwish Murad S, Kim WR, Harnois DM, et al. Efficacy of neoadjuvant chemoradiation, followed by liver transplantation, for perihilar cholangiocarcinoma at 12 US centers. Gastroenterology. 2012;143(1):88-98.e3. — PubMed PMID: 22504095 · doi:10.1053/j.gastro.2012.04.008
22. Song J, Li Y, Bowlus CL, et al. Cholangiocarcinoma in Patients with Primary Sclerosing Cholangitis (PSC): a Comprehensive Review. Clin Rev Allergy Immunol. 2020;58(1):134-149. — PubMed PMID: 31463807 · doi:10.1007/s12016-019-08764-7
23. Saca D, Flamm SL. Cholangiocarcinoma Surveillance Recommendations in Patients with Primary Sclerosing Cholangitis. Clin Liver Dis. 2024;28(1):183-192. — PubMed PMID: 37945159 · doi:10.1016/j.cld.2023.07.010
24. Njei B, McCarty TR, Varadarajulu S, et al. Systematic review with meta-analysis: endoscopic retrograde cholangiopancreatography-based modalities for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. Aliment Pharmacol Ther. 2016;44(11-12):1139-1151. — PubMed PMID: 27696456 · doi:10.1111/apt.13817
25. Clements O, Eliahoo J, Kim JU, et al. Risk factors for intrahepatic and extrahepatic cholangiocarcinoma: A systematic review and meta-analysis. J Hepatol. 2020;72(1):95-103. — PubMed PMID: 31536748 · doi:10.1016/j.jhep.2019.09.007
26. Parra-Robert M, Santos VM, Canis SM, et al. Relationship Between CA 19.9 and the Lewis Phenotype: Options to Improve Diagnostic Efficiency. Anticancer Res. 2018;38(10):5883-5888. — PubMed PMID: 30275214 · doi:10.21873/anticanres.12931
27. Sripa B, Suwannatrai AT, Sayasone S, et al. Current status of human liver fluke infections in the Greater Mekong Subregion. Acta Trop. 2021;224:106133. — PubMed PMID: 34509453 · doi:10.1016/j.actatropica.2021.106133
28. Sripa B, Echaubard P. Prospects and Challenges towards Sustainable Liver Fluke Control. Trends Parasitol. 2017;33(10):799-812. — PubMed PMID: 28754415 · doi:10.1016/j.pt.2017.06.002
29. Bouvard V, Baan R, Straif K, et al. A review of human carcinogens--Part B: biological agents. Lancet Oncol. 2009;10(4):321-2. — PubMed PMID: 19350698 · doi:10.1016/s1470-2045(09)70096-8
30. Goyal L, DiToro D, Hollebecque A, et al. Genomic correlates of response and resistance to the irreversible FGFR1-4 inhibitor futibatinib based on biopsy and circulating tumor DNA profiling. Ann Oncol. 2025;36(4):414-425. — PubMed PMID: 39672383 · doi:10.1016/j.annonc.2024.11.017
31. Banales JM, Cardinale V, Carpino G, et al. Expert consensus document: Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA). Nat Rev Gastroenterol Hepatol. 2016;13(5):261-80. — PubMed PMID: 27095655 · doi:10.1038/nrgastro.2016.51
32. Banales JM, Rodrigues PM, Affò S, et al. Cholangiocarcinoma 2026: status quo, unmet needs and priorities. Nat Rev Gastroenterol Hepatol. 2026;23(1):65-96. — PubMed PMID: 41372578 · doi:10.1038/s41575-025-01153-w

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PubMed Topic Searches

1. Cholangiocarcinoma treatment
2. Intrahepatic CCA FGFR2 fusions
3. CCA IDH1 ivosidenib
4. PSC and cholangiocarcinoma risk
5. Perihilar CCA surgical resection
6. Cholangiocarcinoma liver transplantation
7. Biliary tract cancer chemotherapy
8. CCA immunotherapy TOPAZ-1
9. Liver fluke cholangiocarcinoma
10. CCA prognosis and survival
11. CA19-9 cholangiocarcinoma diagnosis
12. CCA molecular targeted therapy

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Connections

• Oncology
• Cancer Overview
• Hepatocellular Carcinoma
• Pancreatic Cancer
• Primary Sclerosing Cholangitis
• Cirrhosis
• Hepatitis B
• Hepatitis C
• Metastatic Cancers
• NAFLD
• Gallbladder Cancer
• Jaundice
• Liver Function Tests

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