dc.identifier.citation | Izquierdo-Sanchez, Laura Lamarca, Angela La Casta, Adelaida Buettner, Stefan Utpatel, Kirsten Klümpen, Heinz-Josef Adeva, Jorge Vogel, Arndt Lleo, Ana Fabris, Luca Ponz-Sarvise, Mariano Brustia, Raffaele Cardinale, Vincenzo Braconi, Chiara Vidili, Gianpaolo Jamieson, Nigel B. Macias, Rocio I.R. Jonas, Jan Philipp Marzioni, Marco Hołówko, Wacław Folseraas, Trine Kupčinskas, Juozas Sparchez, Zeno Krawczyk, Marcin Krupa, Łukasz Scripcariu, Viorel Grazi, Gian Luca Landa-Magdalena, Ana Ijzermans, Jan N.M. Evert, Katja Erdmann, Joris I. López-López, Flora Saborowski, Anna Scheiter, Alexander Santos-Laso, Alvaro Carpino, Guido Andersen, Jesper B. Marin, Jose JG. Alvaro, Domenico Bujanda, Luis Forner, Alejandro Valle, Juan W. Koerkamp, Bas Groot Banales, Jesus M. . Cholangiocarcinoma landscape in Europe: Diagnostic, prognostic and therapeutic insights from the ENSCCA Registry. Journal of Hepatology. 2022, 76(5), 1109-1121 | |
dc.description.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 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.
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. | |