Cholangiocarcinoma landscape in Europe: Diagnostic, prognostic and therapeutic insights from the ENSCCA Registry

• DOI: 10.1016/j.jhep.2021.12.010
• Publication Date: 2022-02-12T17:00:21Z
• Authors: Laura Izquierdo-S...; Angela Lamarca; Adelaida La Casta; Stefan Buettner; Kirsten Utpatel; Heinz-Josef Klümpen; Jorge Adeva; Arndt Vogel; Ana Lleo; Luca Fabris; Mariano Ponz-Sarvise; Raffaele Brustia; Vincenzo Cardinale; Chiara Braconi; Gianpaolo Vidili; Nigel B. Jamieson; Rocio IR. Macias; Jan Philipp Jonas; Marco Marzioni; Wacław Hołówko; Trine Folseraas; Juozas Kupčinskas; Zeno Sparchez; Marcin Krawczyk; Łukasz Krupa; Viorel Scripcariu; Gian Luca Grazi; Ana Landa-Magdalena; Jan NM. Ijzermans; Katja Evert; Joris I. Erdmann; Flora López-López; Anna Saborowski; Alexander Scheiter; Alvaro Santos-Laso; Guido Carpino; Jesper B. Andersen; Jose JG. Marin; Domenico Alvaro; Luis Bujanda; Alejandro Forner; Juan W. Valle; Bas Groot Koerkamp; Jesus M. Banales
• Keywords: Primary Sclerosing Cholangitis; Concomitant

Cholangiocarcinoma (CCA) is a rare and heterogeneous biliary cancer, whose incidence related mortality increasing. This study investigates the clinical course of CCA subtypes (intrahepatic [iCCA], perihilar [pCCA], distal [dCCA]) in pan-European cohort.The ENSCCA Registry multicenter observational study. Patients were included if they had histologically proven diagnosis between 2010-2019. Demographic, histomorphological, biochemical, studies performed.Overall, 2,234 patients enrolled (male/female=1.29). iCCA (n = 1,243) was associated with overweight/obesity chronic liver diseases involving cirrhosis and/or viral hepatitis; pCCA 592) primary sclerosing cholangitis; dCCA 399) choledocholithiasis. At diagnosis, 42.2% local disease, 29.4% locally advanced disease (LAD), 28.4% metastatic (MD). Serum CEA CA19-9 showed low diagnostic sensitivity, but their concomitant elevation increased risk presenting LAD (odds ratio 2.16; 95% CI 1.43-3.27) or MD 5.88; 3.69-9.25). undergoing resection (50.3%) best outcomes, particularly negative-resection margin (R0) (median overall survival [mOS] 45.1 months); however, involvement (R1) (hazard 1.92; 1.53-2.41; mOS 24.7 months) lymph node invasion 2.13; 1.55-2.94; 23.3 compromised prognosis. Among unresectable (49.6%), 10.6 months for those receiving active palliative therapies, mostly chemotherapy (26.2%), 4.0 supportive care (20.6%). iCCAs worse outcomes than p/dCCAs. ECOG performance status, independent prognostic factors.CCA frequently diagnosed at an stage, proportion fail to receive cancer-specific prognosis remains dismal. Identification preventable factors implementation surveillance high-risk populations are required decrease cancer-related mortality.This is, date, largest international (pan-European: 26 hospitals 11 countries) study, which cholangiocarcinoma has been investigated, comparing 3 based on latest International Classification Diseases 11th Edition (ICD-11) (i.e., intrahepatic [2C12], [2C18], [2C15] affected bile ducts), come into effect 2022. General tumor-type specific features factors, biomarker accuracy, as well patient management presented compared, outlining current state Europe.