Bile duct cancer, also known as cholangiocarcinoma (CCA), is a rare cancer often diagnosed only at an advanced stage. A comprehensive analysis of diagnostic, prognostic and therapeutic aspects of more than 2,200 patients in Europe now provides a valuable knowledge base for raising awareness and managing ACC to improve outcomes. This study is reported in the Journal of Hepatology, the official journal of the European Association for the Study of the Liver, published by Elsevier.
In 2015, the European Network for the Study of Cholangiocarcinoma (ENSCCA) was founded with the aim of establishing a pan-European interdisciplinary cooperative network to address the problem of ACC at basic, translational and clinical levels. Its first initiative was the creation of the ENSCCA clinical registry in 2016 to describe in detail the natural history of ACC in Europe, determine potential risk factors, improve the current classification system, study precise tumor biomarkers early and not invasive drugs for diagnosis and estimating prognosis, comparing the efficacy of therapies and selecting patients for clinical trials.
“CCA is a rare cancer, but its incidence is increasing worldwide, and we need to stop treating it as an orphan disease,” explained lead researcher Dr. Jesus M. Banales, Professor Ikerbasque at Biodonostia, University of Navarre and CIBERehd, Spain. “Although ACC poses 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 .”
In the most comprehensive international observational study to date, researchers assessed the natural history of histologically proven ACC in more than 2,200 patients diagnosed between 2010 and 2019 and whose details were included in the ENSCCA registry. The data was provided by 26 reference hospitals in 11 European countries. At diagnosis, 42.2% of patients had local disease, 29.4% had locally advanced disease, and 28.4% had metastatic disease. They compared general and tumor type-specific characteristics at diagnosis, risk factors, biomarker accuracy, and similarities and differences between the three CCA subtypes based on the latest classification of the World Health Organization according to their anatomical origin (intrahepatic, perihilar or distal). ), and a comparison of patient management and outcomes.
The results suggest that the location of CCA tumors along the biliary tree is associated with different risk factors and tumor characteristics. Diagnoses require invasive confirmations by biopsy or cytology, as blood tests for tumor biomarkers show low sensitivity in the early stages. Surgical resection remains the only potentially curative treatment, although cure is unlikely in cases of compromised resection margins or lymph node invasion. Investigators noted that chemotherapy increased the life expectancy of patients compared to patients receiving the best supportive care. They also noted that deterioration in the patient’s performance status (ECOG), the presence of metastases and increased levels of the tumor marker CA19-9 independently affected the outcome.
A comparative risk factor analysis of the three different locations of CCA tumors revealed some potential lifestyle risk factors such as excess alcohol, smoking, overweight/obesity, diabetes, etc. These are very common in Europe and could predispose people at risk to developing ACC.
“In conclusion, our study provides a comprehensive analysis of the diagnostic, prognostic and therapeutic aspects of the complex landscape of ACC,” commented Dr. Banales. “Results showed that CCA is mostly diagnosed at an advanced stage, a significant proportion of patients receive no cancer-specific treatment, and the prognosis is poor. Treatment options are limited. As a result, awareness campaigns and education programs aimed at preventing lifestyle Cancer risk factors and new techniques for early detection of CCA in high-risk populations are urgently needed to reduce cancer-related mortality Our results represent valuable insights for future comparisons with novel targeted therapies and the design of next-generation personalized clinical therapies in trials.”
ACC is a highly heterogeneous and aggressive malignancy responsible for approximately 2% of all cancer-related deaths worldwide each year. It is often diagnosed when it is already advanced, which makes it difficult to treat successfully. Currently, it accounts for approximately 15% of all primary liver cancers and 3% of all gastrointestinal cancers worldwide.
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