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

In 2015, ENSCCA (European Network for the Study of Cholangiocarcinoma) was founded with the aim of creating a pan-European interdisciplinary cooperative network to address the problem of cholangiocarcinoma (CCA) at the basic, translational and clinical levels.  Its first initiative was the creation of the ENSCCA Clinical Registry in 2016. 

CCA, also known as bile duct cancer, is a cancer that is frequently diagnosed  at an advanced stage.  In the most comprehensive international observational study of CCA to date, researchers evaluated the natural course of this histologically proven cancer in more than 2,200 patients who were diagnosed between 2010-2019 and whose details were included in the ENSCCA registry.

This study was published in the Journal of Hepatology, the official journal of  EASL (European Association for the Study of the Liver) on 12 February 2022, World Cholangiocarcinoma Day, and provides a valuable knowledge base to raise awareness and manage CCA in order to improve results.

The ENSCCA Registry

Lead researcher Dr. Jesús M. Banales, Ikerbasque Professor at Biodonostia, University of Navarra and CIBERehd, Spain, explains: “CCA is a rare cancer, but its incidence is increasing worldwide, and we have to stop treating it as an orphan disease.”

“Despite the fact that CCA constitutes a major challenge for clinicians, scientists, national health systems and society, there is a lack of coordinated multidisciplinary pan-European studies. We therefore used the ENSCCA registry to gather vital information.”

This 10-year effort by ENSCCA has drawn data from treatment centres in Austria, France, Germany, Italy, Lithuania, the Netherlands, Norway, Poland, Romania, Spain, and the United Kingdom.  At diagnosis, 42.2% of patients had local disease, 29.4% had locally advanced disease, and 28.4% had metastatic disease.

ENSCCA compared the general and specific characteristics of the type of tumor at the time of diagnosis, the risk factors, the precision of the biomarkers, as well as the similarities and differences between the three subtypes of CCA according to the latest classification of the WHO (World Health Organisation) based on their anatomical origin (intrahepatic, perihilar, or extrahepatic/distal), and a comparison of patient management and outcomes.

The researchers found that surgical resection remains the only potentially curative treatment, although cure is unlikely with involvement of resection margins or lymph node invasion, and that chemotherapy increased the life expectancy of patients compared to those receiving the best supportive care. They also found that deteriorating patient performance status (ECOG), the presence of metastases, and increased levels of the tumor marker CA19-9 independently affected outcome.

A comparative analysis of the risk factors of the three different locations of the CCA tumors revealed some potential risk factors related to lifestyle, such as excess alcohol, smoking, overweight/obesity, diabetes, etc. These factors are highly prevalent in Europe and could predispose individuals at risk to developing CCA.

“In conclusion, our study provides a comprehensive analysis of the diagnostic, prognostic, and therapeutic aspects of the complex landscape of CCA,” comments Dr. Banales. “The results showed that CCA is mostly diagnosed at an advanced stage, a significant proportion of patients do not receive any specific therapy for cancer and the prognosis is poor.

He continued, “Therapeutic options are limited. Consequently, awareness campaigns and education programs aimed at preventing lifestyle-related risk factors and new techniques for early detection of CCA in high-risk populations are urgently needed in order to decrease cancer-related mortality. Our findings represent valuable knowledge for future comparisons with new targeted therapies and the design of next-generation personalized clinical trials.”

To read the paper in full, click here


February 2022