Abstract
Cholangiocarcinoma (CCA) is a highly lethal adenocarcinoma of the hepatobiliary system, which can be classified as intrahepatic, perihilar and distal. Each anatomic subtype has distinct genetic aberrations, clinical presentations and therapeutic approaches. In endemic regions, liver fluke infection is associated with CCA, owing to the oncogenic effect of the associated chronic biliary tract inflammation. In other regions, CCA can be associated with chronic biliary tract inflammation owing to choledocholithiasis, cholelithiasis, or primary sclerosing cholangitis, but most CCAs have no identifiable cause. Administration of the anthelmintic drug praziquantel decreases the risk of CCA from liver flukes, but reinfection is common and future vaccination strategies may be more effective. Some patients with CCA are eligible for potentially curative surgical options, such as resection or liver transplantation. Genetic studies have provided new insights into the pathogenesis of CCA, and two aberrations that drive the pathogenesis of non-fluke-associated intrahepatic CCA, fibroblast growth factor receptor 2 fusions and isocitrate dehydrogenase gain-of-function mutations, can be therapeutically targeted. CCA is a highly desmoplastic cancer and targeting the tumour immune microenvironment might be a promising therapeutic approach. CCA remains a highly lethal disease and further scientific and clinical insights are needed to improve patient outcomes.
Highlights
Cholangiocarcinoma (CCA) is a highly lethal, epithelial cell malignancy that occurs anywhere along the biliary tree and/or within the hepatic parenchyma
These cancers are heterogeneous and are best classified according to the primary, anatomic subtype as intrahepatic CCA, perihilar CCA or distal CCA5,6 (Fig. 1). iCCA is located proximally to the second-order bile ducts within the liver parenchyma, pCCA is localized between the second-order bile ducts and the insertion of the cystic duct into the common bile duct, and dCCA is confined to the common bile duct below the cystic duct insertion
Following ingestion of infected fish flesh, gastric and intestinal juices digest the encysted metacercariae, whereupon excysted juvenile flukes migrate through the ampulla of Vater into the common bile duct and into the intrahepatic bile ducts
Summary
CCA depending on aetiology and anatomic subtype. We highlight the patient experience and future directions for control and treatment of this disease. The incidence of CCA associated with liver fluke infection, calculated as an age‐standardized rate, varies by geographical region and other risk factors but has exceeded 100 per 100,000 in men and 40 per 100,000 in women in hotspots in northeast Thailand[21]. Following ingestion of infected fish flesh, gastric and intestinal juices digest the encysted metacercariae, whereupon excysted juvenile flukes migrate through the ampulla of Vater into the common bile duct and into the intrahepatic bile ducts. The lowest ASMRs (
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