2026-09-16T07:44:25Zhttps://gredos.usal.es/oai/request
oai:gredos.usal.es:10366/1552392025-04-30T19:33:38Zcom_10366_3992com_10366_3947com_10366_3946com_10366_3823col_10366_3993
Gestión del Repositorio Documental de la Universidad de Salamanca author Izquierdo-Sanchez, Laura author Lamarca, Angela author La Casta, Adelaida author Buettner, Stefan author Utpatel, Kirsten author Klümpen, Heinz-Josef author Adeva, Jorge author Vogel, Arndt author Lleo, Ana author Fabris, Luca author Ponz-Sarvise, Mariano author Brustia, Raffaele author Cardinale, Vincenzo author Braconi, Chiara author Vidili, Gianpaolo author Jamieson, Nigel B author Rodríguez Macías, Rocío Isabel author Jonas, Jan Philipp author Marzioni, Marco author Hołówko, Wacław author Folseraas, Trine author Kupčinskas, Juozas author Sparchez, Zeno author Krawczyk, Marcin author Krupa, Łukasz author Scripcariu, Viorel author Grazi, Gian Luca author Landa-Magdalena, Ana author Ijzermans, Jan Nm author Evert, Katja author Erdmann, Joris I author López-López, Flora author Saborowski, Anna author Scheiter, Alexander author Santos-Laso, Alvaro author Carpino, Guido author Andersen, Jesper B author García Marín, José Juan author Alvaro, Domenico author Bujanda, Luis author Forner, Alejandro author Valle, Juan W author Koerkamp, Bas Groot author Banales, Jesus M. 2024-02-02T17:17:54Z 2024-02-02T17:17:54Z 2022-05 Izquierdo-Sanchez, L., Lamarca, A., La Casta, A., Buettner, S., Utpatel, K., Klümpen, H. J., ... & Banales, J. M. (2022). Cholangiocarcinoma landscape in Europe: Diagnostic, prognostic and therapeutic insights from the ENSCCA Registry. Journal of hepatology, 76(5), 1109-1121. https://doi.org/10.1016/j.jhep.2021.12.010 1600-0641 http://hdl.handle.net/10366/155239 10.1016/j.jhep.2021.12.010 35167909 0168-8278 [EN]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. 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. 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 CA19-9 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. 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. 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. eng Attribution-NonCommercial-NoDerivatives 4.0 Internacional Cholangiocarcinoma Epidemiology Risk factors Treatment Cholangiocarcinoma landscape in Europe: Diagnostic, prognostic and therapeutic insights from the ENSCCA Registry info:eu-repo/semantics/article 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 URL https://gredos.usal.es/bitstream/10366/155239/1/2022-Izquierdo-JHEP.pdf File MD5 6c894bebb561718e505043cd76c35874 1246738 application/pdf 2022-Izquierdo-JHEP.pdf URL https://gredos.usal.es/bitstream/10366/155239/3/2022-Izquierdo-JHEP.pdf.txt File MD5 752ced9bba6490105334eb733a7b79e3 72651 text/plain 2022-Izquierdo-JHEP.pdf.txt