Abstract
Simple SummaryThe treatment of peri-hilar (h-CCA) and intrahepatic (i-CCA) cholangiocarcinoma is an evolving field in hepato-pancreato-biliary surgery. Continuous development of radiological and surgical techniques currently offers different treatment strategies, ranging from traditional hepatectomies to complex approaches involving preoperative portal vein embolization or associating liver partition and portal vein ligation for staged hepatectomy. Recent advances in perioperative chemo-radiotherapy have improved patient survival and have been incorporated into transplant protocols, yielding excellent results. We report a comprehensive review of current surgical and multimodal approaches to h-CCA and i-CCA treatment.Cholangiocarcinoma accounts for approximately 10% of all hepatobiliary tumors and represents 3% of all new-diagnosed malignancies worldwide. Intrahepatic cholangiocarcinoma (i-CCA) accounts for 10% of all cases, perihilar (h-CCA) cholangiocarcinoma represents two-thirds of the cases, while distal cholangiocarcinoma accounts for the remaining quarter. Originally described by Klatskin in 1965, h-CCA represents one of the most challenging tumors for hepatobiliary surgeons, mainly because of the anatomical vascular relationships of the biliary confluence at the hepatic hilum. Surgery is the only curative option, with the goal of a radical, margin-negative (R0) tumor resection. Continuous efforts have been made by hepatobiliary surgeons in order to achieve R0 resections, leading to the progressive development of aggressive approaches that include extended hepatectomies, associating liver partition, and portal vein ligation for staged hepatectomy, pre-operative portal vein embolization, and vascular resections. i-CCA is an aggressive biliary cancer that arises from the biliary epithelium proximal to the second-degree bile ducts. The incidence of i-CCA is dramatically increasing worldwide, and surgical resection is the only potentially curative therapy. An aggressive surgical approach, including extended liver resection and vascular reconstruction, and a greater application of systemic therapy and locoregional treatments could lead to an increase in the resection rate and the overall survival in selected i-CCA patients. Improvements achieved over the last two decades and the encouraging results recently reported have led to liver transplantation now being considered an appropriate indication for CCA patients.
Highlights
Portal vein resection was proposed as a standard procedure in the ‘no-touch’ approach described by Neuhaus et al in 1999 [95]; the oncological benefit of this surgical approach was documented in a following paper by the same group, where patients approached with a ‘no-touch’ technique experienced a better overall survival compared with patients receiving a traditional hepatectomy (5-year overall survival: 58% vs. 29%) [66]
Watanabe et al demonstrated that a surgical margin width of >1 cm in negative lymph nodes Intrahepatic cholangiocarcinoma (i-CCA) patients is associated with better overall survival [143]
Extended liver resection with or without vascular or biliary reconstructions is often required to obtain R0 margins, especially in cases of voluminous lesions or multifocal tumors, which account for 50–70% of all i-CCAs cases, despite a higher rate of R1 resection related to this surgical approach compared with minor resections [142,145]
Summary
Cholangiocarcinoma (CCA) accounts for approximately 10% of all hepatobiliary tumors and represents 3% of all new-diagnosed malignancies worldwide [1]. This rare tumor arises from the biliary epithelium and can develop at any level of the intra- and extrahepatic biliary tree; according to its location, CCA is generally classified as intra-hepatic, peri-hilar, or distal, representing three distinct entities in terms of biology, treatment options, and prognosis [2], with separate American Joint Committee on Cancer (AJCC) staging systems [3]. The surgical approach to distal CCA is usually represented by pancreatoduodenectomy [5]; as such, this subgroup of tumors is generally managed by pancreatic rather than hepatobiliary surgeons. The following paper will focus on the surgical management and treatment of h-CCA and i-CCA
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