Abstract
Cholangiocarcinoma (CCA) arises from the biliary tract epithelium and accounts for 10–15% of all hepatobiliary malignancies. Depending on anatomic location, CCA is classified as intrahepatic (iCCA), perihilar (pCCA) and distal (dCCA). The best treatment option for pCCA is liver resection and when a radical oncological surgery is obtained, 5-year survival rate are around 20–40%. In unresectable patients, following a specific protocol, liver transplantation (LT) for pCCA showed excellent long-term disease-free survival rates. Fewer data are available for iCCA in LT setting. Nevertheless, patients with very early unresectable iCCA appear to achieve excellent outcomes after LT. This review aims to evaluate existing evidence to define the current role of LT in the management of patients with CCA.
Highlights
Cholangiocarcinoma (CCA) is the second most common hepatic malignancy after hepatocellular carcinoma (HCC); its overall incidence has progressively increased worldwide over the past four decades [1,2,3]
CCAs represent an estimated 3% of all gastrointestinal system malignancies. They are classically divided into three groups, depending on the anatomic site of origin: intrahepatic CCAs, perihilar CCAs and distal CCAs [7]. iCCAs are defined as CCAs located proximally to the second-order bile ducts. iCCA can present three different patterns of growth: mass-forming (MF-iCCA), periductal infiltrating (PI-iCCA) and intraductal growing (IG-iCCA)
In the era of transplant oncology, liver transplantation (LT) represents a good option for unresectable perihilar CCAs (pCCAs)
Summary
Cholangiocarcinoma (CCA) is the second most common hepatic malignancy after hepatocellular carcinoma (HCC); its overall incidence has progressively increased worldwide over the past four decades [1,2,3]. CCA is a malignant tumor of the cholangiocytes (i.e., the epithelial cells lining the bile ducts), which can develop at any portion of the biliary tree, from the canals of Hering to the main bile duct [4,5]. Most CCAs are well, moderately or poorly differentiated adenocarcinomas; other histological subtypes are rarely encountered. Surgery is the preferred therapeutic option for all subtypes of CCA, involvement of the vascular structures and lymph nodes needs to be considered when evaluating the best approach. Refinements in CCA diagnosis and better understanding of genetic profiles may lead to an optimized surgical treatment. This review aims to analyze the recent advances in the management of CCA, focusing on surgical treatments and highlighting the role of liver transplantation in the era of transplant oncology
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