| Title: |
Cholangiocarcinoma landscape in Europe. Diagnostic, prognostic and therapeutic insights from the ENSCCA registry |
| Authors: |
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 Ir; 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 Nm; 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 |
| Contributors: |
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 Ir; Jonas, Jan Philipp; Marzioni, Marco; Hołówko, Wacław; Folseraas, Trine; Kupčinskas, Juoza; Sparchez, Zeno; Krawczyk, Marcin; Krupa, Łukasz; Scripcariu, Viorel; Grazi, Gian Luca; Landa-Magdalena, Ana; Ijzermans, Jan Nm; 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, Lui; Forner, Alejandro; Valle, Juan W; Koerkamp, Bas Groot; Banales, Jesus M |
| Publisher Information: |
ELSEVIER; RADARWEG 29, 1043 NX AMSTERDAM, NETHERLANDS |
| Publication Year: |
2022 |
| Collection: |
Sapienza Università di Roma: CINECA IRIS |
| Subject Terms: |
International Classification of Diseases 11(th) edition (ICD-11); cholangiocarcinoma; diagnosi; prognosi; risk factor; subtype; treatment; bile ducts; intrahepatic; CA-19-9 antigen; female; human; male; registrie; bile duct neoplasm |
| Description: |
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. ... |
| Document Type: |
article in journal/newspaper |
| Language: |
English |
| Relation: |
info:eu-repo/semantics/altIdentifier/pmid/35167909; info:eu-repo/semantics/altIdentifier/wos/WOS:000823493800014; volume:76; issue:5; firstpage:1109; lastpage:1121; numberofpages:13; journal:JOURNAL OF HEPATOLOGY; https://hdl.handle.net/11573/1661165 |
| DOI: |
10.1016/j.jhep.2021.12.010 |
| Availability: |
https://hdl.handle.net/11573/1661165; https://doi.org/10.1016/j.jhep.2021.12.010 |
| Rights: |
info:eu-repo/semantics/openAccess |
| Accession Number: |
edsbas.4DB8BC6 |
| Database: |
BASE |