Background: The majority of patients with cholangiocarcinoma present with advanced disease that is often challenging to diagnose and to treat. The optimal preoperative evaluation requires a coordinated multidisciplinary approach. Surgical resection is the mainstay of therapy.
Methods: This systematic review delineates surgical treatment strategies for cholangiocarcinoma in general as well as special considerations concerning the particular tumor localization. A literature search (see keywords) was conducted using PubMed and publications between 1990 and 2016 regarding resectable and advanced cholangiocarcinoma were reviewed. Selected studies were utilized based on their significance and innovation.
Results: The type and extent of resection performed depends on the location of the cholangiocarcinoma within the liver or biliary tree and the extent of local tumor invasion. The common surgical strategy contains: (i) for intrahepatic tumors: tailored partial hepatectomy combined with extended hilar, suprapancreatic, celiac axis lymphadenectomy, (ii) for hilar tumors: complete resection of the extrahepatic biliary tree combined with extended hepatectomy inclusive of segment I, resection of portal vein bifurcation, and systematic N1/N2 lymphadenectomy, and (iii) for distal tumors: en bloc pancreatoduodenectomy combined with complete resection of the extrahepatic bile duct below the hepatic confluence and systematic N1/N2 lymphadenectomy. Pathologic confirmation is not required prior to resection. Preoperative biliary drainage and remnant liver volume augmentation are necessary in selected patients with intrahepatic or hilar cholangiocarcinoma considered for extensive liver resection.
Conclusion: Cure for cholangiocarcinoma requires complete surgical resection with histologically negative margins. R0 resection provides a satisfactory long-term outcome in patients with lymph node-negative stage. Neoadjuvant treatment followed by liver transplantation provides long-term survival in highly selected cases with localized, unresectable, lymph node-negative hilar cholangiocarcinoma.
Keywords: ALPPS; Bile duct carcinoma; Cholangiocarcinoma; Hepatectomy; Liver transplantation; Portal vein embolization; Surgical strategy.