期刊
JOURNAL OF HEPATOLOGY
卷 76, 期 5, 页码 1109-1121出版社
ELSEVIER
DOI: 10.1016/j.jhep.2021.12.010
关键词
cholangiocarcinoma; International Classification of Diseases 11th edition (ICD-11); subtypes; diagnosis; risk factors; treatment; prognosis
资金
- European Association for the Study of the Liver (EASL)
- Incyte Pharma
- Spanish Carlos III Health Institute (ISCIII) [FIS PI18/01075, PI21/00922, CPII19/00008, PI19/00819]
- Department of Health of the Basque Country [2017111010]
- BIOEF (Basque Foundation for Innovation and Health Research: EiTB Maratoi) [BIO15/CA/016/BD]
- Fundacion Cientifica de la Asociacion Espanola Contra el Cancer (AECC Scientific Foundation)
- European Union's Horizon 2020 Research and Innovation Program [825510]
- The Christie Charity
- ISCIII [PI18/00542]
- Lord Kelvin Adam Smith Leadership fellowship at University of Glasgow
- Fondazione AIRC per la Ricerca sul Cancro (IG2019 project) [23408]
- ERDF/ESF, A way to make Europe/Investing in your future, Spain [0348_CIE_6_E]
- ERDF/ESF, Centro Internacional sobre el Envejecimiento, Spain [0348_CIE_6_E]
- Fundacio la Marato de TV3 [201916-31]
- Junta de Castilla y Leon, Spain [SA074P20]
- Cancer Research UK [A25813]
- Lord Kelvin Adam Smith Leadership fellowship at the University of Glasgow
This study investigates the clinical course of cholangiocarcinoma in a pan-European cohort and finds that the disease is frequently diagnosed at an advanced stage and a proportion of patients fail to receive cancer-specific therapies, resulting in a poor prognosis.
Background & Aims: Cholangiocarcinoma (CCA) is a rare and heterogeneous biliary cancer, whose incidence and related mortality is increasing. This study investigates the clinical course of CCA and subtypes (intrahepatic [iCCA], perihilar [pCCA], and distal [dCCA]) in a pan-European cohort. Methods: The ENSCCA Registry is a multicenter observational study. Patients were included if they had a histologically proven diagnosis of CCA between 2010-2019. Demographic, histomorphological, biochemical, and clinical studies were performed. Results: Overall, 2,234 patients were enrolled (male/female=1.29). iCCA (n = 1,243) was associated with overweight/ obesity and chronic liver diseases involving cirrhosis and/or viral hepatitis; pCCA (n = 592) with primary sclerosing cholangitis; and dCCA (n = 399) with choledocholithiasis. At diagnosis, 42.2% of patients had local disease, 29.4% locally advanced disease (LAD), and 28.4% metastatic disease (MD). Serum CEA and CA199 showed low diagnostic sensitivity, but their concomitant elevation was associated with increased risk of presenting with LAD (odds ratio 2.16; 95% CI 1.43-3.27) or MD (odds ratio 5.88; 95% CI 3.69-9.25). Patients undergoing resection (50.3%) had the best outcomes, particularly with negative-resection margin (R0) (median overall survival [mOS] = 45.1 months); however, margin involvement (R1) (hazard ratio 1.92; 95% CI 1.53-2.41; mOS = 24.7 months) and lymph node invasion (hazard ratio 2.13; 95% CI 1.55-2.94; mOS = 23.3 months) compromised prognosis. Among patients with unresectable disease (49.6%), the mOS was 10.6 months for those receiving active palliative therapies, mostly chemotherapy (26.2%), and 4.0 months for those receiving best supportive care (20.6%). iCCAs were associated with worse outcomes than p/dCCAs. ECOG performance status, MD and CA19-9 were independent prognostic factors. Conclusion: CCA is frequently diagnosed at an advanced stage, a proportion of patients fail to receive cancer-specific therapies, and prognosis remains dismal. Identification of preventable risk factors and implementation of surveillance in high-risk populations are required to decrease cancer-related mortality. Lay summary: This is, to date, the largest international (pan-European: 26 hospitals and 11 countries) observational study, in which the course of cholangiocarcinoma has been investigated, comparing the 3 subtypes based on the latest International Classification of Diseases 11th Edition (ICD-11) (i.e., intrahepatic [2C12], perihilar [2C18], or distal [2C15] affected bile ducts), which come into effect in 2022. General and tumor-type specific features at diagnosis, risk factors, biomarker accuracy, as well as patient management and outcomes, are presented and compared, outlining the current clinical state of cholangiocarcinoma in Europe. (C) 2021 The Authors. Published by Elsevier B.V. on behalf of European Association for the Study of the Liver.
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