Abstract

Simple SummaryCholangiocarcinoma (CCA) is the second most common liver primary malignancy and its gold-standard treatment is surgery. Unfortunately, CCA is seldom amenable to curative resection due to late-stage diagnosis and frequent major vascular invasion. Major vascular invasion has historically been considered a contraindication to resection, but lately aggressive surgeries for CCA with vascular involvement have been shown to improve outcomes. The purpose of this review is to provide a comprehensive and up to date summary of the strategies for CCA resection, focusing on the surgical techniques and results of complex procedures with tumour vascular involvements. The current review shows that satisfactory results can be achieved in patients with CCA and tumoral vascular invasion by aggressive surgical resection and challenging vascular reconstruction, ensuring a meticulous evaluation of patients in a multidisciplinary setting by experienced hepatobiliary surgeons.Cholangiocarcinoma (CCA) is an aggressive malignancy of the biliary tract. To date, surgical treatment remains the only hope for definitive cure of CCA patients. Involvement of major vascular structures was traditionally considered a contraindication for resection. Nowadays, selected cases of CCA with vascular involvement can be successfully approached. Intrahepatic CCA often involves the major hepatic veins or the inferior vena cava and might necessitate complete vascular exclusion, in situ hypothermic perfusion, ex situ surgery and reconstruction with autologous, heterologous or synthetic grafts. Hilar CCA more frequently involves the portal vein and hepatic artery. Resection and reconstruction of the portal vein is now considered a relatively safe and beneficial technique, and it is accepted as a standard option either with direct anastomosis or jump grafts. However, hepatic artery resection remains controversial; despite accumulating positive reports, the procedure remains technically challenging with increased rates of morbidity. When arterial reconstruction is not possible, arterio-portal shunting may offer salvage, while sometimes an efficient collateral system could bypass the need for arterial reconstructions. Keys to achieve success are represented by accurate selection of patients in high-volume referral centres, adequate technical skills and eclectic knowledge of the various possibilities for vascular reconstruction.

Highlights

  • Cholangiocarcinoma (CCA) is a rare cancer, yet the second most common primary liver cancer after hepatocellular carcinoma

  • The anatomical distinction between perihilar CCA (pCCA) and distal CCA (dCCA) is represented by cystic duct insertion, whereas intrahepatic CCA (iCCA) emerges from the secondary order intrahepatic bile ducts

  • Surgical treatment is the gold standard for CCA, but the tumour is frequently diagnosed in late stages due to its asymptomatic course, resulting in unresectable disease at diagnosis

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Summary

Introduction

Cholangiocarcinoma (CCA) is a rare cancer, yet the second most common primary liver cancer after hepatocellular carcinoma. CCA arises from the biliary tree and is divided into three subgroups, based on its localisation: intrahepatic CCA (iCCA), hilar/perihilar CCA (pCCA) and distal CCA (dCCA). The anatomical distinction between pCCA and dCCA is represented by cystic duct insertion, whereas iCCA emerges from the secondary order intrahepatic bile ducts. Up to 80–90% of CCAs are extrahepatic (pCCA, dCCA), while the remaining 10–20% of lesions are iCCA [2]. Surgical treatment is the gold standard for CCA, but the tumour is frequently diagnosed in late stages due to its asymptomatic course, resulting in unresectable disease at diagnosis. CCA frequently involves major hepatic vessels, such as the inferior vena cava (IVC) and/or hepatic veins, portal vein (PV) and hepatic artery, which might limit the surgical strategies

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