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Gut. 2007 December; 56(12): 1755–1756.
PMCID: PMC2095716

Cholangiocarcinoma and its management

This is an introduction to the Gut tutorial “Cholangiocarcinoma and its management” hosted on BMJ Learning—the best available learning website for medical professionals from the BMJ Group.

Cholangiocarcinoma originates in the biliary epithelium. Mortality rates from intrahepatic cholangiocarcinoma appear to have risen steadily over the past 30 years and it now causes more deaths than hepatocellular carcinoma annually in England and Wales. Primary sclerosing cholangitis is the commonest known predisposing factor for cholangiocarcinoma in the UK. The commonest presenting clinical features of perihilar or extrahepatic tumours are those of biliary obstruction. CA 19–9 is elevated in up to 85% of patients with cholangiocarcinoma and has a sensitivity of 75% and specificity of 80% in patients with primary sclerosing cholangitis, but its value in patients without primary sclerosing cholangitis is limited. Good quality magnetic resonance imaging (MRI) is the optimal imaging investigation for suspected cholangiocarcinoma, providing accurate information on biliary anatomy and local invasion, extent of duct involvement with magnetic resonance cholangiopancreatography (MRCP) and vascular involvement by magnetic resonance angiography. Up to 50% of patients are lymph node positive at presentation, which is associated with poor surgical outcome. Perhaps 10–20% of patients have peritoneal involvement and laparoscopy could be considered to determine the presence of peritoneal or superficial liver metastases in those considered resectable on imaging. Surgery is the only curative treatment for patients with cholangiocarcinoma with a 9–18% five year survival for proximal bile duct lesions and 20–30% for distal lesions. Routine preoperative biliary drainage is no longer recommended if there is no undue delay prior to surgery, but in certain patients who are severely malnourished, or who are suffering from acute suppurative cholangitis, preoperative drainage is beneficial.

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Competing interests: None.


1. Taylor‐Robinson S D, Toledano M B, Arora S. et al Increase in mortality rates for intrahepatic cholangiocarcinoma in England and Wales 1968–1998. Gut 2001. 48816–820.820 [PMC free article] [PubMed]
2. Khan S A, Taylor‐Robinson S D, Toledano M B. et al Changing international trends in mortality rates for liver, biliary and pancreatic tumours 1979–1997. J Hepatol 2002. 37806–813.813 [PubMed]
3. Shaib Y, El‐Serag H B. The epidemiology of cholangiocarcinoma. Semin Liver Dis 2004. 24(2)115–125.125 [PubMed]
4. Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology 2001. 331353–1357.1357 [PubMed]
5. O'Grady J G. Treatment options for other malignancies. Liver Transplantation 2000. 6S23–S29.S29 [PubMed]
6. Khan S A, Davidson B R, Goldin R. et al UK guidelines for the diagnosis and treatment of cholangiocarcinoma. Gut. 2002;51(Suppl 6)1–9.9
7. De Groen P C, Gores G J, LaRusso N F. et al Biliary tract cancers. NEJM 1999. 3411368–1379.1379 [PubMed]
8. Bismuth H, Castaing D. Hepatobiliary malignancy. London: Edward Arnold, 1994. 416–424.424
9. Gores G J. Early detection and treatment of cholangiocarcinoma. Liver Transplantation 2000. 6S30–S34.S34 [PubMed]
10. Ramage J K, Donaghy A, Farrant J M. et al Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. Gastroenterology 1995. 108865–869.869 [PubMed]
11. Patel A H, Harnois D M, Klee G G. et al The utility of CA 19–9 in the diagnoses of cholangiocarcinoma in patients without primary sclerosing cholangitis. Am J Gastroenterol 2000. 95204–207.207 [PubMed]
12. Hultcrantz R, Olsson R, Danielsson A. et al A 3‐year prospective study on serum tumor markers used for detecting cholangiocarcinoma in patients with primary sclerosing cholangitis. J Hepatol 1999. 30669–673.673 [PubMed]
13. Kuszyk B S, Soyer P, Bluemke D A. et al Intrahepatic cholangiocarcinoma: the role of imaging in detection and staging. Crit Rev Dian Imaging 1997. 3859–88.88
14. Fulcher A S, Turner M A. HASTE MR cholangiography in the evaluation of hilar cholangiocarcinoma. AJR Am J Roentgenol 1997. 1691501–1505.1505 [PubMed]
15. Cieszanowski A, Chomicka D, Andrzejewska M. et al Imaging techniques in patients with biliary obstruction. Med Sci Monit 2000. 61197–1202.1202 [PubMed]
16. Figueras J, Llado L, Valls C. et al Changing strategies in diagnosis and management of hilar cholangiocarcinoma. Liver Transpl 2000. 6786–794.794 [PubMed]
17. Yeh T S, Jan Y Y, Tseng J H. et al Malignant perihilar biliary obstruction: magnetic resonance cholangiopancreatographic findings. Am J Gastroenterol 2000. 95432–440.440 [PubMed]
18. Zidi S H, Prat F, Le Guen O. et al Performance characteristics of magnetic resonance cholangiography in the staging of malignant hilar strictures. Gut 2000. 46103–106.106 [PMC free article] [PubMed]
19. Magnuson T H, Bender J S, Duncan M D. et al Utility of magnetic resonance cholangiography in the evaluation of biliary obstruction. J Am Coll Surg 1999. 18963–72.72 [PubMed]
20. Chan F L, Chan J K, Leong L L. Modern imaging in the evaluation of hepatolithiasis. Hepatogastroenterology 1997. 44358–369.369 [PubMed]
21. Wiersema M J, Vilmann P, Giovannini M. et al Endosonography‐guided fine‐needle aspiration biopsy: diagnostic accuracy and complication assessment. Gastroenterology 1997. 1121087–1095.1095 [PubMed]
22. In: Sobin L H, Wittekind C. eds. TNM Classification of Malignant Tumours: International Union Against Cancer. 5th ed. New York, NY: Wiley 1997
23. Henson D E, Albores‐Saavedra J, Corle D. Carcinoma of the extrahepatic bile ducts. Histologic types, stage of disease, grade, and survival rates. Cancer 1992. 701498–1501.1501 [PubMed]
24. Hillen H F. Unknown primary tumours. Postgrad Med J 2000. 76690–693.693 [PMC free article] [PubMed]
25. Stevens K J, Smith S L, Denley H. et al Is mammography of value in women with disseminated cancer of unknown origin? Clin Oncol 1999. 1190–92.92
26. Stain S C, Baer H U, Dennison A R. et al Current management of hilar cholangiocarcinoma. Surg Gynecol Obstet 1992. 175579–588.588 [PubMed]
27. Nimura Y, Kamiya J, Nagino M. et al Aggressive surgical treatment of hilar cholangiocarcinoma. J Hepatobiliary Pancreat Surg 1998. 552–61.61 [PubMed]
28. Madariaga J R, Iwatsuki S, Todo S. et al Liver resection for hilar and peripheral cholangiocarcinomas: a study of 62 cases. Ann Surg 1998. 227(1)70–79.79 [PubMed]
29. Iwatsuki S, Todo S, Marsh J W. et al Treatment of hilar cholangiocarcinoma (Klatskin tumors) with hepatic resection or transplantation. J Am Coll Surg 1998. 187358–364.364 [PMC free article] [PubMed]
30. Meyer C G, Penn I, James L. Liver transplantation for cholangiocarcinoma: results in 207 patients. Transplantation 2000. 691633–1637.1637 [PubMed]
31. De Vreede I, Steers J L, Burch P A. et al Prolonged disease‐free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma. Liver Transpl 2000. 6309–316.316 [PubMed]
32. Heimbach J K, Gores G J, Haddock M G. et al Liver transplantation for unresectable perihilar cholangiocarcinoma. Semin Liver Dis 2004. 24201–207.207 [PubMed]
33. Nordback I H, Pitt H A, Coleman J. et al Unresectable hilar cholangiocarcinoma: percutaneous versus operative palliation. Surgery 1994. 115597–603.603 [PubMed]
34. Lammer J, Hausegger K A, Fluckiger F. et al Common bile duct obstruction due to malignancy: treatment with plastic versus metal stents. Radiology 1996. 201167–172.172 [PubMed]
35. Hochwald S N, Burke E C, Jarnagin W R. et al Association of preoperative biliary stenting with increased postoperative infectious complications in proximal cholangiocarcinoma. Arch Surg 1999. 134261–266.266 [PubMed]
36. Nakeeb A, Pitt H A. The role of preoperative biliary decompression in obstructive jaundice. Hepatogastroenterol 1995. 42332–337.337
37. Eckhard R, Abou‐Rebyeh H, Adler A. et al Magnetic resonance cholangiopancreatography‐guided unilateral endoscopic stent placement for Klatskin tumors. Gastrointest Endosc 2001. 5340–46.46 [PubMed]
38. Chang W ‐ H, Kortan P, Haber G B. Outcome in patients with bifurcation tumors who undergo unilateral versus bilateral hepatic duct drainage. Gastrointest Endosc 1998. 47354–362.362 [PubMed]
39. De Palma G D, Galloro G, Sicialiano S. et al Unilateral versus bilateral endoscopic hepatic duct drainage in patients with malignant hilar biliary obstruction: results of a prospective, randomized, and controlled study. Gastrointest Endosc 2001. 53547–553.553 [PubMed]
40. Hejna M, Pruckmayer M, Raderer M. The role of chemotherapy and radiation in the management of biliary cancer: a review of the literature. Eur J Cancer 1998. 34977–986.986 [PubMed]
41. Anderson C D, Pinson C W, Berlin J. et al Diagnosis and treatment of cholangiocarcinoma. Oncologist 2004. 943–57.57 [PubMed]
42. Tsavaris N, Kosmas C, Gouveris P. et al Weekly gemcitabine for the treatment of biliary tract and gallbladder cancer. Invest New Drugs 2004. 22193–198.198 [PubMed]
43. Burris H A, 3rd, Moore M J, Andersen J. et al Improvements in survival and clinical benefit with gemcitabine as first line therapy for patients with advanced pancreatic cancer. J Clin Oncol 1997. 152403–2413.2413 [PubMed]
44. Glimelius B, Hoffman K, Sjoden P O. et al Chemotherapy improves survival and quality of life in advanced biliary and pancreatic cancer. Ann Oncol 1996. 7593–600.600 [PubMed]
45. Ducreux M, Rougier P, Fandi A. et al Effective treatment of advanced bilary tract carcinoma using 5‐FU continuous infusion with cisplatin. Ann Oncol 1998. 9653–656.656 [PubMed]
46. Bowling T E, Galbraith S M, Hatfield A R. et al A retrospective comparison of endoscopic stenting alone with stenting and radiotherapy in non‐resectable cholangiocarcinoma. Gut 1996. 39852–855.855 [PMC free article] [PubMed]
47. Foo M L, Gunderson L L, Bender C E. et al External radiation therapy and transcatheter iridium in the treatment of extrahepatic bile duct carcinoma. Int J Radiat Oncol Biol Phys 1997. 39929–935.935 [PubMed]
48. Pitt H A, Nakeeb A, Abrams R A. et al Peri‐hilar cholangiocarcinoma: Post‐operative radiation therapy does not improve survival. Ann Surg 1995. 221788–798.798 [PubMed]

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