Skip to main content Accessibility help
  • Print publication year: 2009
  • Online publication date: August 2010

11.1 - Surgical treatment: resection and transplantation

from 11 - Treatment of cholangiocarcinoma


Cholangiocarcinoma can present anywhere along the biliary tree. Clinical presentation, preoperative evaluation, and treatment are dependent on the anatomic location of the tumor. Cholangiocarcinomas are divided into those that arise in the extrahepatic and those that arise in the intrahepatic bile ducts. Cholangiocarcinomas originating in the extrahepatic bile ducts are categorized as distal, mid, and proximal (hilar). Distal tumors are those lying in the retroduodenal and intrapancreatic bile duct. Mid bile duct tumors lie below the right and left bile-duct confluence but above the retroduodenal common bile duct. Proximal bile-duct tumors lie at or above the right–left bile-duct confluence.

Preoperative assessment

Extrahepatic cholangiocarcinoma

Patients with extrahepatic cholangiocarcinoma most frequently present with jaundice but have often had more non-specific accompanying symptoms for a few weeks to months prior to the onset of jaundice. Those additional symptoms include pruritus, mild abdominal pain, anorexia, and weight loss. Infrequently, asymptomatic patients with extrahepatic cholangiocarcinoma will be diagnosed because of an unexplained rise in serum alkaline phosphatase or bilirubin or because an imaging study done to evaluate for another condition demonstrates dilated bile ducts. The age at presentation is most frequently 60 years or greater, but younger patients, especially those with ulcerative colitis, primary sclerosing cholangitis, or choledochal cyst, may present with cholangiocarcinoma.

Initial laboratory assessment of patients with jaundice or with dilated bile ducts should include liver functions: total bilirubin, fractionated bilirubin, alkaline phosphatase, aspartate transaminase (AST), alanine transaminase (ALT), serum albumin; complete blood count, including platelet count; prothrombin time and partial thromboplastin time; tumor markers, including carcinoembryonic antigen (CEA), cancer antigen (CA)19–9, and alpha-fetoprotein (AFP); blood urea nitrogen (BUN) and creatinine; and hepatitis serologies, including hepatitis A antibody, hepatitis B surface antigen and antibody, hepatitis B core antibodies, and hepatitis C antibody.

Limongelli, P, Pai, M, Bansi, D, et al. Correlation between preoperative biliary drainage, bile duct contamination and postoperative outcomes for pancreatic surgery. Surgery 2007; 142: 313–18.
Povoski, SP, Karpeh, MS, Conlon, KC, Blumgart, LH, and Brennan, MF. Association of preoperative biliary drainage with postoperative outcome following pancreaticoduodenectomy. Ann Surg 1999; 230: 143–4.
Ito, F, Agni, R, Rettammel, MA, et al. Resection of hilar cholangiocarcinoma: Concomitant liver resection decreases hepatic recurrence. Ann Surg 2008; 248: 273–9.
Jarnigan, WR, Fong, Y, DeMatteo, RP, et al. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma. Ann Surg 2001; 234: 507–19.
Tsao, JI, Nimura, Y, Karniya, J, et al. Management of hilar cholangiocarcinoma: Comparison of an American and a Japanese experience. Ann Surg 2000; 232: 166–74.
Kondo, S, Takada, T, Mayazaki, M, et al. Guidelines for the management of biliary tract and ampullary carcinomas: Surgical treatment. J Hepatobiliary Pancreat Surg 2008; 15: 41–54.
Kim, JY, Kim, MH, Lee, TY, et al. Clinical role of 18F-FDG PET-CT in suspected and potentially operable cholangiocarcinoma: A prospective study compared with conventional imaging. Am J Gastroenterol 2008; 103: 1145–51.
Corvera, CU, Blumgart, LH, Akhurst, T, et al. 18F-fluorodeoxyglucose positron emission tomography influences management decisions in patients with biliary cancer. J Am Coll Surg 2008; 206: 47–65.
Pavey, DA and Gress, FG. The role of EUS-guided FNA for the evaluation of biliary strictures. Gastrointest Endosc 2006; 64: 334–7.
Corvera, CU, Blumgart, LH, Darvishian, F, et al. Clinical and pathologic features of proximal biliary strictures masquerading as hilar cholangiocarcinoma. J Am Coll Surg 2005; 201: 862–9.
Jarnigan, WR. Cholangiocarcinoma of the extrahepatic bile ducts. Semin Surg Oncol 2000; 19: 156–76.
Weber, SM, DeMatteo, RP, Fong, Y, Blumgart, LH, and Jarnagin, WR. Staging laparoscopy in patients with extrahepatic biliary carcinoma. Ann Surg 2002; 235: 392–9.
Vollmer, CM, Drebin, JA, Middleton, WD, et al. Utility of staging laparoscopy on subsets of peripancreatic and biliary malignancies. Ann Surg 2002; 235: 1–7.
Connor, S, Barron, E, Wigmore, SJ, Madhavan, KK, Parks, RW, and Garden, OJ. The utility of laparoscopic assessment in the preoperative staging of suspected hilar cholangiocarcinoma. J Gastrointest Surg 2005; 9: 476–80.
Sano, T, Shimada, K, Sakamoto, Y, Yamamoto, J, Yamasaki, S, and Kosuge, T. One hundred two consecutive hepatobiliary resections for perihilar cholangiocarcinoma with zero mortality. Ann Surg 2006; 244: 240–7.
Maguchi, H, Takahashi, K, Katanuma, A, et al. Preoperative biliary drainage for hilar cholangiocarcinoma. J Hepatobiliary Pancreat Surg 2007; 14: 441–6.
Heming, AW, Reed, AI, Howard, RJ, et al. Preoperative portal vein embolization for extended hepatectomy. Ann Surg 2003; 237: 686–93.
Kawasaki, S, Imamura, H, Kobayashi, A, Noike, T, Miwa, S, and Miyagawa, S. Results of surgical resection for patients with hilar bile duct cancer: Application of extended hepatectomy after biliary drainage and hemihepatic portal vein embolization. Ann Surg 2003; 238: 84–92.
Yokoyama, Y, Nagino, M, Nishio, H, Ebata, T, Igami, T, and Nimura, Y. Recent advances in the treatment of hilar cholangiocarcinoma: Portal vein embolization. J Hepatobiliary Pancreat Surg 2007; 14: 447–54.
Seyama, Y, Kubota, K, Sano, K, et al. Long-term outcome of extended hemihepatectomy for hilar bile duct cancer with no mortality and high survival rate. Ann Surg 2003; 238: 73–83.
Heming, AL, Reed, AI, Fujita, S, Foley, DP, and Howard, RJ. Surgical management of hilar cholangiocarcinoma. Ann Surg 2005; 241: 693–702.
Miyazaki, M, Kato, A, Ito, H, et al. Combined vascular resection in operative resection for hilar cholangiocarcinoma: Does it work?Surgery 2007; 141: 581–8.
Hirono, S, Tani, M, Kawai, M, Ina, S, Uchiyama, K, and Yamaue, H. Indication of hepatopancreatoduodenectomy for biliary tract cancer. World J Surg 2006; 30: 567–73.
Wakai, T, Shirai, Y, Tsuchiya, Y, Nomura, T, Akazawa, K, and Hatakeyama, K. Combined major hepatectomy and pancreaticoduodenectomy for locally advanced biliary carcinoma: Long term results. World J Surg 2008; 32: 1075–6.
Miyazaki, M, Kimura, F, Shimizu, H, et al. Recent advance in the treatment of hilar cholangiocarcinoma: Hepatectomy with vascular resection. J Hepatobiliary Pancreat Surg 2007; 14: 463–8.
Konstadoulakis, MM, Roayaie, S, Gomatos, IP, et al. Aggressive surgical resection of hilar cholangiocarcinoma: Is it justified? Audit of a single center's experience. Am J Surg 2008; 196: 160–9.
DeOliveira, ML, Cunningham, SC, Cameron, JL, et al. Cholangiocarcinoma: Thirty-one-year experience with 564 patients at a single institution. Ann Surg 2007; 245: 755–62.
Cheng, Q, Luo, X, Zhang, B, Jiang, X, Yi, B, and Wu, M. Predictive factors for prognosis of hilar cholangiocarcinoma: Postresection radiotherapy improves survival. Eur J Surg Oncol 2007; 33: 202–7.
Kayahara, M, Nagakawa, T, Ohta, T, Kitagawa, H, Tajima, H, and Miwa, K. Role of nodal involvement and the periductal soft-tissue margin in middle and distal bile duct cancer. Ann Surg 1999; 229: 76–83.
Todoroki, T, Kawamoto, T, Koike, N, Fukao, K, Shoda, J, and Takahashi, H. Treatment strategy for patients with middle and lower third bile duct cancer. Br J Surg 2001; 88: 364–70.
Sakamoto, Y, Kosuge, T, Shimada, K, et al. Prognostic factors of surgical resection in middle and distal bile duct cancer: An analysis of 55 patients concerning the significance of ductal and radial margins. Surgery 2005; 137: 396–402.
Karanicolas, PJ, Davies, E, Kunz, R, et al. The pylorus: Take it or leave it? Systematic review and meta-analysis of pylorus-preserving versus standard Whipple pancreaticoduodenectomy for pancreatic or periampullary cancer. Ann Surg Oncol 2007; 14: 1825–34.
Cheng, Q, Luo, X, Zhang, B, Jiang, X, Yi, B, and Wu, M. Distal bile duct carcinoma: Prognostic factors after curative surgery. A series of 112 cases. Ann Surg Oncol 2006; 14: 1212–9.
Fong, Y, Blumgart, LH, Lin, E, Fortner, JG, and Brennan, MF. Outcome of treatment for distal bile duct cancer. Br J Surg 1996; 83: 1712–15.
Konstadoulakis, MM, Roayaie, S, Gomatos, IP, et al. Fifteen-year, single-center experience with the surgical management of intrahepatic cholangiocarcinoma: Operative results and long-term outcome. Surgery 2008; 143: 366–74.
Paik, KY, Jung, JC, Heo, JS, Choi, SH, Choi, DW, and Kim, YI. What prognostic factors are important for resected intrahepatic cholangiocarcinoma?J Gastroenterol Hepatol 2008; 23: 766–70.
Endo, I, Gonen, M, Yopp, AC, et al. Intrahepatic cholangiocarcinoma: Rising frequency, improved survival, and determinants of outcome after resection. Ann Surg 2008; 248: 84–96.
Weber, SM, Jarnagin, WR, Klimstra, D, DeMatteo, RP, Fong, Y, and Blumgart, LH. Intrahepatic cholangiocarcinoma: Resectability, recurrence pattern, and outcomes. J Am Coll Surg 2001; 193: 384–91.
Pinson, CW and Moore, . Liver transplantation is not indicated for cholangiocarcinoma. HPB 2003; 5: 203–5.
Callery, M. Transplantation for cholangiocarcinoma: Advance or supply-demand dilemma?Gastroenterology 2006; 130: 2242–4.
Brandsaeter, B, Isoniemi, H, Broome, U, et al. Liver transplantation for primary sclerosing cholangitis; predictors and consequences of hepatobiliary malignancy. J Hepatol 2004; 40: 815–22.
Wu, Y, Johlin, FC, Rayhill, SC, et al. Long-term, tumor-free survival after radiotherapy combining hepatectomy-Whipple en bloc and orthotopic liver transplantation for early-stage hilar cholangiocarcinoma. Liver Transpl 2008; 14: 279–86.
Ghali, P, Marotta, PJ, Yoshida, EM, et al. Liver transplantation for incidental cholangiocarcinoma: Analysis of the Canadian experience. Liver Transpl 2005; 11: 1412–16.
Meyer, CG, Penn, I, and James, L. Liver transplantation for cholangiocarcinoma: Results in 207 patients. Transplantation 2000; 69: 1633–7.
Kaiser, GM, Sotiropoulos, GC, Jauch, KW, et al. Liver transplantation for hilar cholangiocarcinoma: A German survey. Transplant Proc 2008; 40: 3191–3.
Becker, NS, Rodriguez, JA, Barshes, NR, O'Mahony, CA, Goss, JA, and Aloia, TA. Outcomes analysis for 280 patients with cholangiocarcinoma treated with liver transplantation over an 18-year period. J Gastrointest Surg 2008; 12: 117–22.
Robles, R, Figueras, J, Turrión, VS, et al. Liver transplantation for hilar cholangiocarcinoma: Spanish experience. Transplant Proc 2003; 35: 1821–2.
Iwatsuki, S, Todo, S, Marsh, JW, et al. Treatment of hilar cholangiocarcinoma (Klatskin tumors) with hepatic resection or transplantation. J Am Coll Surg 1998; 187: 358–64.
Sotiropoulos, GC, Kaiser, BM, Lang, H, et al. Liver transplantation as a primary indication for intrahepatic cholangiocarcinoma: A single-center experience. Transplant Proc 2008; 40: 3194–5.
Pascher, A, Jonas, S, and Heuhaus, P. Intrahepatic cholangiocarcinoma: Indication for transplantation. J Hepatobiliary Pancreat Surg 2003; 10: 232–7.
Robles, R, Figueras, J, Turrión, VS, et al. Liver transplantation for peripheral cholangiocarcinoma: Spanish experience. Transplant Proc 2003; 35: 1823–4.
Rosen, CB, Heimbach, JK, and Gores, GJ. Surgery for cholangiocarcinoma: The role of liver transplantation. HPB 2008; 10: 186–9.
Gores, GJ, Nagorney, DM, and Rosen, CB. Cholangiocarcinoma: Is transplantation an option? For whom?J Hepatol 2007; 47: 455–9.
Heimbach, JK, Gores, GJ, Haddock, MG, et al. Predictors of disease recurrence following neoadjuvant chemoradiotherapy and liver transplantation for unresectable perihilar cholangiocarcinoma. Transplantation 2006; 82: 1703–7.
Neuhaus, P, Jonas, S, Settmacher, U, et al. Surgical management of proximal bile duct cancer: Extended right lobe resection increases resectability and radicality. Langenbecks Arch Surg 203; 388: 194–200.