Skip to main content

Surgical Approach to the Treatment of Cholangiocarcinoma

  • Chapter
  • First Online:
Diagnosis and Management of Cholangiocarcinoma

Abstract

Cholangiocarcinoma is a rare but lethal malignancy arising anywhere along the biliary ductal epithelium from the liver to the duodenum. The disease is classified based on anatomic location, and the mainstay of treatment is surgery. Complete resection is currently the only chance for cure despite significant advances in systemic and other nonsurgical therapies. In this chapter, we present a detailed evaluation of the anatomic classification of cholangiocarcinoma and preoperative evaluation, defining resectability, types of resections, and postoperative outcomes. Special attention is given to advances in the field and the future of surgery in the multidisciplinary management of cholangiocarcinoma.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Abbreviations

AFP:

Alpha fetoprotein

AJCC:

American Joint Committee on Cancer

BSA:

Body surface area

CCA:

Cholangiocarcinoma

CEA:

Carcinoembryonic antigen

CT:

Computed tomography

dCCA:

distal cholangiocarcinoma

eCCA:

extrahepatic cholangiocarcinoma

ERC:

Endoscopic retrograde cholangiography

FGFR:

Fibroblast growth factor receptor

FLR:

Future liver remnant

GEMOX:

Gemcitabine with oxaliplatin

iCCA:

intrahepatic cholangiocarcinoma

IDH:

Isocitrate dehydrogenase

KGR:

Kinetic growth rate

NCCN:

National Comprehensive Cancer Network

OLT:

Orthotopic liver transplantation

pCCA:

perihilar cholangiocarcinoma

PET:

Positron emission tomography

PVE:

Portal vein embolization

RLV:

Remnant liver volume

SEER:

Surveillance, Epidemiology, and End Results

TFLV:

Total functional liver volume

TLV:

Total liver volume

TNM:

Tumor Node Metastasis

References

  1. Yamasaki S. Intrahepatic cholangiocarcinoma: macroscopic type and stage classification. J Hepato-Biliary-Pancreat Surg. 2003;10:288–91.

    Article  Google Scholar 

  2. National Comprehensive Cancer Network. 2019. Hepatobiliary cancers.

    Google Scholar 

  3. Nakeeb A, Pitt HA, Sohn TA, Coleman J, Abrams RA, Piantadosi S, Hruban RH, Lillemoe KD, Yeo CJ, Cameron JL. Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg. 1996;224:463–75.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  4. de Groen PC, Gores GJ, LaRusso NF, Gunderson LL, Nagorney DM. Biliary tract cancers. N Engl J Med. 1999;341:1368–78.

    Article  PubMed  Google Scholar 

  5. Klatskin G. Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis: an unusual tumor with distinctive clinical and pathological features. Am J Med. 1965;38:241–56.

    Article  CAS  PubMed  Google Scholar 

  6. Patel T. Worldwide trends in mortality from biliary tract malignancies. BMC Cancer. 2002;2:10.

    Article  PubMed  PubMed Central  Google Scholar 

  7. DeOliveira ML, Cunningham SC, Cameron JL, Kamangar F, Winter JM, Lillemoe KD, Choti MA, Yeo CJ, Schulick RD. Cholangiocarcinoma: thirty-one-year experience with 564 patients at a single institution. Ann Surg. 2007;245:755–62.

    Article  PubMed  PubMed Central  Google Scholar 

  8. Saha SK, Zhu AX, Fuchs CS, Brooks GA. Forty-year trends in cholangiocarcinoma incidence in the u.s.: intrahepatic disease on the rise. Oncologist. 2016;21:594–9.

    Article  PubMed  PubMed Central  Google Scholar 

  9. Bertuccio P, Malvezzi M, Carioli G, Hashim D, Boffetta P, El-Serag HB, La Vecchia C, Negri E. Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma. J Hepatol. 2019;71:104–14.

    Article  PubMed  Google Scholar 

  10. Mansour JC, Aloia TA, Crane CH, Heimbach JK, Nagino M, Vauthey J-N. Hilar Cholangiocarcinoma: expert consensus statement. HPB. 2015;17:691–9.

    Article  PubMed  PubMed Central  Google Scholar 

  11. Vilgrain V. Staging cholangiocarcinoma by imaging studies. HPB (Oxford). 2008;10:106–9.

    Article  CAS  Google Scholar 

  12. Breitenstein S, Apestegui C, Clavien P-A. Positron emission tomography (PET) for cholangiocarcinoma. HPB (Oxford). 2008;10:120–1.

    Article  CAS  Google Scholar 

  13. Okabayashi T, Yamamoto J, Kosuge T, Shimada K, Yamasaki S, Takayama T, Makuuchi M. A new staging system for mass-forming intrahepatic cholangiocarcinoma. Cancer. 2001;92:2374–83.

    Article  CAS  PubMed  Google Scholar 

  14. Bismuth H, Corlette M. Intrahepatic cholangioenteric anastomosis in carcinoma of the hilus of the liver. Surgery. Gynecol Obstet. 1975;140:170–8.

    CAS  Google Scholar 

  15. Burke EC, Jarnagin WR, Hochwald SN, Pisters PW, Fong Y, Blumgart LH. Hilar Cholangiocarcinoma: patterns of spread, the importance of hepatic resection for curative operation, and a presurgical clinical staging system. Ann Surg. 1998;228:385–94.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  16. Jarnagin WR, Fong Y, DeMatteo RP, Gonen M, Burke EC, Jessica Bodniewicz BS, Miranda Youssef BA, Klimstra D, Blumgart LH. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma. Ann Surg. 2001;234:507–19.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  17. Postlewait LM, Ethun CG, Le N, Pawlik TM, Buettner S, Poultsides G, Tran T, Idrees K, Isom CA, Fields RC, et al. Proposal for a new T-stage classification system for distal cholangiocarcinoma: a 10-institution study from the U.S. Extrahepatic Biliary Malignancy Consortium. HPB (Oxford). 2016;18:793–9.

    Article  Google Scholar 

  18. Vauthey J-N, Abdalla EK, Doherty DA, Gertsch P, Fenstermacher MJ, Loyer EM, Lerut J, Materne R, Wang X, Encarnacion A, et al. Body surface area and body weight predict total liver volume in Western adults. Liver Transpl. 2002;8:233–40.

    Article  PubMed  Google Scholar 

  19. Guglielmi A, Ruzzenente A, Conci S, Valdegamberi A, Iacono C. How much remnant is enough in liver resection? Dig Surg. 2012;29:6–17.

    Article  PubMed  Google Scholar 

  20. Narula N, Aloia TA. Portal vein embolization in extended liver resection. Langenbeck's Arch Surg. 2017;402:727–35.

    Article  Google Scholar 

  21. Rous P, Larimore LD. Relation of the portal blood to liver maintenance: a demonstration of liver atrophy conditional on compensation. J Exp Med. 1920;31:609–32.

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  22. Fausto N, Campbell JS, Riehle KJ. Liver regeneration. Hepatology. 2006;43:S45–53.

    Article  CAS  PubMed  Google Scholar 

  23. Shindoh J, Truty MJ, Aloia TA, Curley SA, Zimmitti G, Huang SY, Mahvash A, Gupta S, Wallace MJ, Vauthey J-N. Kinetic growth rate after portal vein embolization predicts posthepatectomy outcomes: toward zero liver-related mortality in patients with colorectal liver metastases and small future liver remnant. J Am Coll Surg. 2013;216:201–9.

    Article  PubMed  Google Scholar 

  24. Abulkhir A, Limongelli P, Healey AJ, Damrah O, Tait P, Jackson J, Habib N, Jiao LR. Preoperative portal vein embolization for major liver resection: a meta-analysis. Ann Surg. 2008;1:247.

    Google Scholar 

  25. Panzeri F, Crippa S, Castelli P, Aleotti F, Pucci A, Partelli S, Zamboni G, Falconi M. Management of ampullary neoplasms: a tailored approach between endoscopy and surgery. World J Gastroenterol. 2015;21:7970–87.

    Article  PubMed  PubMed Central  Google Scholar 

  26. Weber SM, Jarnagin WR, Klimstra D, DeMatteo RP, Fong Y, Blumgart LH. Intrahepatic Cholangiocarcinoma: resectability, recurrence pattern, and outcomes. J Am Coll Surg. 2001;193:384–91.

    Article  CAS  PubMed  Google Scholar 

  27. Matsuo K, Rocha FG, Ito K, D’Angelica MI, Allen PJ, Fong Y, DeMatteo RP, Gonen M, Endo I, Jarnagin WR. The blumgart preoperative staging system for hilar cholangiocarcinoma: analysis of resectability and outcomes in 380 patients. J Am Coll Surg. 2012;215:343–55.

    Article  PubMed  Google Scholar 

  28. Ruys AT, Busch OR, Gouma DJ, van Gulik TM. Staging laparoscopy for hilar cholangiocarcinoma: is it still worthwhile? Ann Surg Oncol. 2011;18:2647–53.

    Article  PubMed  PubMed Central  Google Scholar 

  29. Reames BN, Ejaz A, Koerkamp BG, Alexandrescu S, Marques HP, Aldrighetti L, Maithel SK, Pulitano C, Bauer TW, Shen F, et al. Impact of major vascular resection on outcomes and survival in patients with intrahepatic cholangiocarcinoma: a multi-institutional analysis. J Surg Oncol. 2017;116:133–9.

    Article  PubMed  Google Scholar 

  30. Levi Sandri GB, Spoletini G, Mascianà G, Colasanti M, Lepiane P, Vennarecci G, D’Andrea V, Ettorre GM. The role of minimally invasive surgery in the treatment of cholangiocarcinoma. Eur J Surg Oncol. 2017;43:1617–21.

    Article  CAS  PubMed  Google Scholar 

  31. van Vugt JLA, Gaspersz MP, Coelen RJS, Vugts J, Labeur TA, de Jonge J, Polak WG, Busch ORC, Besselink MG, IJzermans JNM, et al. The prognostic value of portal vein and hepatic artery involvement in patients with perihilar cholangiocarcinoma. HPB. 2018;20:83–92.

    Article  PubMed  Google Scholar 

  32. Hemming AW, Mekeel K, Khanna A, Baquerizo A, Kim RD. Portal Vein Resection in Management of Hilar Cholangiocarcinoma. Journal of the American College of Surgeons. 2011;212:604–13.

    Google Scholar 

  33. Maeta T, Ebata T, Hayashi E, Kawahara T, Mizuno S, Matsumoto N, Ohta S, Nagino M, the Nagoya Surgical Oncology Group. Pancreatoduodenectomy with portal vein resection for distal cholangiocarcinoma. Br J Surg. 2017;104:1549–57.

    Article  CAS  PubMed  Google Scholar 

  34. Peng C, Zhou D, Meng L, Cao Y, Zhang H, Pan Z, Lin C. The value of combined vein resection in pancreaticoduodenectomy for pancreatic head carcinoma: a meta-analysis. BMC Surg. 2019;19:84.

    Article  PubMed  PubMed Central  Google Scholar 

  35. Tseng J, Raut C, Lee J, Pisters P, Vauthey J, Abdalla E, Gomez H, Sun C, Crane C, Wolff R. Pancreaticoduodenectomy with vascular resection: margin status and survival duration. Journal of Gastrointestinal Surgery. 2004;8:935–50.

    Article  PubMed  Google Scholar 

  36. Meyer CG, Penn I, James L. Liver transplantation for cholangiocarcinoma: results in 207 patients. Transplantation. 2000;8:69.

    Google Scholar 

  37. Rosen CB. Transplantation versus resection for hilar cholangiocarcinoma: an argument for shifting paradigms for resectable disease in annals of surgery 2018. Ann Surg. 2018;5:267.

    Google Scholar 

  38. Ethun CG, Lopez-Aguiar AG, Anderson DJ, Adams AB, Fields RC, Doyle MB, Chapman WC, Krasnick BA, Weber SM, Mezrich JD, et al. Transplantation versus resection for hilar cholangiocarcinoma: an argument for shifting treatment paradigms for resectable disease. Ann Surg. 2018;5:267.

    Google Scholar 

  39. Spolverato G, Yakoob MY, Kim Y, Alexandrescu S, Marques HP, Lamelas J, Aldrighetti L, Gamblin TC, Maithel SK, Pulitano C, et al. Impact of complications on long-term survival after resection of intrahepatic cholangiocarcinoma. Cancer. 2015;121:2730–9.

    Article  PubMed  Google Scholar 

  40. Ali SM, Clark CJ, Zaydfudim VM, Que FG, Nagorney DM. Role of Major Vascular Resection in Patients with Intrahepatic Cholangiocarcinoma. Annals of Surgical Oncology. 2013;20:2023–8.

    Article  PubMed  Google Scholar 

  41. Ohtsuka M, Ito H, Kimura F, Shimizu H, Togawa A, Yoshidome H, Shimamura F, Shimizu Y, Miyazaki M. Extended hepatic resection and outcomes in intrahepatic cholangiocarcinoma. Journal of Hepato-Biliary-Pancreatic Surgery. 2003;10:259–64.

    Article  PubMed  Google Scholar 

  42. Allen PJ, Reiner AS, Gonen M, Klimstra DK, Blumgart LH, Brennan MF, D’Angelica M, Dematteo R, Fong Y, Jarnagin WR. Extrahepatic cholangiocarcinoma: a comparison of patients with resected proximal and distal lesions. HPB. 2008;10:341–6.

    Google Scholar 

  43. Hasegawa S, Ikai I, Fujii H, Hatano E, Shimahara Y. Surgical Resection of Hilar Cholangiocarcinoma: Analysis of Survival and Postoperative Complications. World Journal of Surgery. 2007;31:1258–65.

    Article  Google Scholar 

  44. Nagino M, Kamiya J, Uesaka K, Sano T, Yamamoto H, Hayakawa N, Kanai M, Nimura Y. Complications of Hepatectomy for Hilar Cholangiocarcinoma. World Journal of Surgery. 2001;25:1277–83.

    Article  CAS  PubMed  Google Scholar 

  45. Gerhards MF, van Gulik TM, de Wit LT, Obertop H, Gouma DJ. Evaluation of morbidity and mortality after resection for hilar cholangiocarcinoma—a single center experience. Surgery. 2000;127:395–404.

    Article  CAS  PubMed  Google Scholar 

  46. Andrianello S, Paiella S, Allegrini V, Ramera M, Pulvirenti A, Malleo G, Salvia R, Bassi C. Pancreaticoduodenectomy for distal cholangiocarcinoma: surgical results, prognostic factors, and long-term follow-up. Langenbeck’s Archives of Surgery. 2015;400:623–8.

    Article  PubMed  Google Scholar 

  47. Valle J, Wasan H, Palmer DH, Cunningham D, Anthoney A, Maraveyas A, Madhusudan S, Iveson T, Hughes S, Pereira SP, et al. Cisplatin plus Gemcitabine versus Gemcitabine for Biliary Tract Cancer. N Engl J Med. 2010;362:1273–81.

    Article  CAS  PubMed  Google Scholar 

  48. Ebata T, Hirano S, Konishi M, Uesaka K, Tsuchiya Y, Ohtsuka M, Kaneoka Y, Yamamoto M, Ambo Y, Shimizu Y, et al. Randomized clinical trial of adjuvant gemcitabine chemotherapy versus observation in resected bile duct cancer. Br J Surg. 2018;105:192–202.

    Article  CAS  PubMed  Google Scholar 

  49. Edeline J, Benabdelghani M, Bertaut A, Watelet J, Hammel P, Joly J-P, Boudjema K, Fartoux L, Bouhier-Leporrier K, Jouve J-L, et al. Gemcitabine and oxaliplatin chemotherapy or surveillance in resected biliary tract cancer (PRODIGE 12-ACCORD 18-UNICANCER GI): a randomized phase III study. J Clin Oncol. 2019;37:658–67.

    Article  CAS  PubMed  Google Scholar 

  50. Primrose JN, Fox RP, Palmer DH, Malik HZ, Prasad R, Mirza D, Anthony A, Corrie P, Falk S, Finch-Jones M, et al. Capecitabine compared with observation in resected biliary tract cancer (BILCAP): a randomised, controlled, multicentre, phase 3 study. Lancet Oncol. 2019;20:663–73.

    Article  CAS  PubMed  Google Scholar 

  51. Shroff RT, Kennedy EB, Bachini M, Bekaii-Saab T, Crane C, Edeline J, El-Khoueiry A, Feng M, Katz MHG, Primrose J, et al. Adjuvant therapy for resected biliary tract cancer: ASCO clinical practice guideline. J Clin Oncol. 2019;37:1015–27.

    Article  PubMed  Google Scholar 

  52. Ben-Josef E, Guthrie KA, El-Khoueiry AB, Corless CL, Zalupski MM, Lowy AM, Thomas CR, Alberts SR, Dawson LA, Micetich KC, et al. SWOG S0809: A phase II intergroup trial of adjuvant capecitabine and gemcitabine followed by radiotherapy and concurrent capecitabine in extrahepatic cholangiocarcinoma and gallbladder carcinoma. J Clini Oncol. 2014;33:2617–22.

    Article  CAS  Google Scholar 

  53. Rizvi S, Khan SA, Hallemeier CL, Kelley RK, Gores GJ. Cholangiocarcinoma — evolving concepts and therapeutic strategies. Nat Rev Clin Oncol. 2018;15:95–111.

    Article  CAS  PubMed  Google Scholar 

  54. Groot Koerkamp B, Wiggers JK, Allen PJ, Besselink MG, Blumgart LH, Busch ORC, Coelen RJ, D’Angelica MI, DeMatteo RP, Gouma DJ, et al. Recurrence rate and pattern of perihilar cholangiocarcinoma after curative intent resection. J Am Coll Surg. 2015;221:1041–9.

    Article  PubMed  PubMed Central  Google Scholar 

  55. Wang C, Pang S, Si-Ma H, Yang N, Zhang H, Fu Y, Yang G. Specific risk factors contributing to early and late recurrences of intrahepatic cholangiocarcinoma after curative resection. World J Surg Oncol. 2019;17:2–2.

    Article  PubMed  PubMed Central  Google Scholar 

  56. El-Gendi A, El-Shafei M, El-Gendi S, Shawky A. Laparoscopic Versus Open Hepatic Resection for Solitary Hepatocellular Carcinoma Less Than 5 cm in Cirrhotic Patients: A Randomized Controlled Study. Journal of Laparoendoscopic & Advanced Surgical Techniques. 2017;28:302–10.

    Article  Google Scholar 

  57. Fretland ÅA, Dagenborg VJ, Bjørnelv GMW, Kazaryan AM, Kristiansen R, Fagerland MW, Hausken J, Tønnessen TI, Abildgaard A, Barkhatov L, et al. Laparoscopic Versus Open Resection for Colorectal Liver Metastases: The OSLO-COMET Randomized Controlled Trial. Annals of Surgery. 2018:267.

    Google Scholar 

  58. Massard C, Michiels S, Ferté C, Le Deley M-C, Lacroix L, Hollebecque A, Verlingue L, Ileana E, Rosellini S, Ammari S, et al. High-throughput genomics and clinical outcome in hard-to-treat advanced cancers: results of the MOSCATO 01 trial. Cancer Discov. 2017;7:586–95.

    Article  CAS  PubMed  Google Scholar 

  59. Verlingue L, Malka D, Allorant A, Massard C, Ferté C, Lacroix L, Rouleau E, Auger N, Ngo M, Nicotra C, et al. Precision medicine for patients with advanced biliary tract cancers: an effective strategy within the prospective MOSCATO-01 trial. Eur J Cancer. 2017;87:122–30.

    Article  PubMed  Google Scholar 

  60. Simile MM, Bagella P, Vidili G, Spanu A, Manetti R, Seddaiu MA, Babudieri S, Madeddu G, Serra PA, Altana M, et al. Targeted Therapies in Cholangiocarcinoma: Emerging Evidence from Clinical Trials. Medicina (Kaunas). 2019;55:42.

    Article  Google Scholar 

  61. Javle M, Lowery M, Shroff RT, Weiss KH, Springfeld C, Borad MJ, Ramanathan RK, Goyal L, Sadeghi S, Macarulla T, et al. Phase II study of BGJ398 in patients with fgfr-altered advanced cholangiocarcinoma. J Clin Oncol. 2018;36:276–82.

    Article  CAS  PubMed  Google Scholar 

  62. Borger DR, Tanabe KK, Fan KC, Lopez HU, Fantin VR, Straley KS, Schenkein DP, Hezel AF, Ancukiewicz M, Liebman HM, et al. Frequent mutation of isocitrate dehydrogenase (IDH)1 and IDH2 in cholangiocarcinoma identified through broad-based tumor genotyping. Oncologist. 2012;17:72–9.

    Article  CAS  PubMed  Google Scholar 

  63. Abou-Alfa GK, Macarulla Mercade T, Javle M, Kelley RK, Lubner S, Adeva J, Cleary JM, Catenacci DV, Borad MJ, Bridgewater JA, et al. LBA10_PR - ClarIDHy: A global, phase III, randomized, double-blind study of ivosidenib (IVO) vs placebo in patients with advanced cholangiocarcinoma (CC) with an isocitrate dehydrogenase 1 (IDH1) mutation. Ann Oncol. 2019;30:872–3.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Corresponding author

Correspondence to Sepideh Gholami .

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2021 Springer Nature Switzerland AG

About this chapter

Check for updates. Verify currency and authenticity via CrossMark

Cite this chapter

Judge, S.J., Loehfelm, T.W., Gholami, S. (2021). Surgical Approach to the Treatment of Cholangiocarcinoma. In: Tabibian, J.H. (eds) Diagnosis and Management of Cholangiocarcinoma. Springer, Cham. https://doi.org/10.1007/978-3-030-70936-5_14

Download citation

  • DOI: https://doi.org/10.1007/978-3-030-70936-5_14

  • Published:

  • Publisher Name: Springer, Cham

  • Print ISBN: 978-3-030-70935-8

  • Online ISBN: 978-3-030-70936-5

  • eBook Packages: MedicineMedicine (R0)

Publish with us

Policies and ethics