Izquierdo-Sanchez, Laura and Lamarca, Angela and La Casta, Adelaida and Buettner, Stefan and Utpatel, Kirsten and Klumpen, Heinz-Josef and Adeva, Jorge and Vogel, Arndt and Lleo, Ana and Fabris, Luca and Ponz-Sarvise, Mariano and Brustia, Raffaele and Cardinale, Vincenzo and Braconi, Chiara and Vidili, Gianpaolo and Jamieson, Nigel B. and Macias, Rocio Ir and Jonas, Jan Philipp and Marzioni, Marco and Holowko, Waclaw and Folseraas, Trine and Kupcinskas, Juozas and Sparchez, Zeno and Krawczyk, Marcin and Krupa, Lukasz and Scripcariu, Viorel and Grazi, Gian Luca and Landa-Magdalena, Ana and Ijzermans, Jan Nm and Evert, Katja and Erdmann, Joris and Lopez-Lopez, Flora and Saborowski, Anna and Scheiter, Alexander and Santos-Laso, Alvaro and Carpino, Guido and Andersen, Jesper B. and Marin, Jose Jg and Alvaro, Domenico and Bujanda, Luis and Forner, Alejandro and Valle, Juan W. and Koerkamp, Bas Groot and Banales, Jesus M. (2022) Cholangiocarcinoma landscape in Europe: Diagnostic, prognostic and therapeutic insights from the ENSCCA Registry. JOURNAL OF HEPATOLOGY, 76 (5). pp. 1109-1121. ISSN 0168-8278, 1600-0641
Full text not available from this repository. (Request a copy)Abstract
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.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | ; cholangiocarcinoma; International Classification of Diseases 11th edition (ICD-11); subtypes; diagnosis; risk factors; treatment; prognosis |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Lehrstuhl für Pathologie |
| Depositing User: | Dr. Gernot Deinzer |
| Date Deposited: | 19 Sep 2023 10:24 |
| Last Modified: | 19 Sep 2023 10:24 |
| URI: | https://pred.uni-regensburg.de/id/eprint/58827 |
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