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. 2007 Dec;56(12):1755–1756. doi: 10.1136/gut.2007.138859

Cholangiocarcinoma and its management

S A Khan 1, A Miras 1, M Pelling 1, S D Taylor‐Robinson 1
PMCID: PMC2095716  PMID: 17998326

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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Footnotes

Competing interests: None.

References

  • 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 200148816–820. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 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 200237806–813. [DOI] [PubMed] [Google Scholar]
  • 3.Shaib Y, El‐Serag H B. The epidemiology of cholangiocarcinoma. Semin Liver Dis 200424(2)115–125. [DOI] [PubMed] [Google Scholar]
  • 4.Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology 2001331353–1357. [DOI] [PubMed] [Google Scholar]
  • 5.O'Grady J G. Treatment options for other malignancies. Liver Transplantation 20006S23–S29. [DOI] [PubMed] [Google Scholar]
  • 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. [DOI] [PMC free article] [PubMed]
  • 7.De Groen P C, Gores G J, LaRusso N F.et al Biliary tract cancers. NEJM 19993411368–1379. [DOI] [PubMed] [Google Scholar]
  • 8.Bismuth H, Castaing D.Hepatobiliary malignancy. London: Edward Arnold, 1994416–424.
  • 9.Gores G J. Early detection and treatment of cholangiocarcinoma. Liver Transplantation 20006S30–S34. [DOI] [PubMed] [Google Scholar]
  • 10.Ramage J K, Donaghy A, Farrant J M.et al Serum tumor markers for the diagnosis of cholangiocarcinoma in primary sclerosing cholangitis. Gastroenterology 1995108865–869. [DOI] [PubMed] [Google Scholar]
  • 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 200095204–207. [DOI] [PubMed] [Google Scholar]
  • 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 199930669–673. [DOI] [PubMed] [Google Scholar]
  • 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 19973859–88. [PubMed] [Google Scholar]
  • 14.Fulcher A S, Turner M A. HASTE MR cholangiography in the evaluation of hilar cholangiocarcinoma. AJR Am J Roentgenol 19971691501–1505. [DOI] [PubMed] [Google Scholar]
  • 15.Cieszanowski A, Chomicka D, Andrzejewska M.et al Imaging techniques in patients with biliary obstruction. Med Sci Monit 200061197–1202. [PubMed] [Google Scholar]
  • 16.Figueras J, Llado L, Valls C.et al Changing strategies in diagnosis and management of hilar cholangiocarcinoma. Liver Transpl 20006786–794. [DOI] [PubMed] [Google Scholar]
  • 17.Yeh T S, Jan Y Y, Tseng J H.et al Malignant perihilar biliary obstruction: magnetic resonance cholangiopancreatographic findings. Am J Gastroenterol 200095432–440. [DOI] [PubMed] [Google Scholar]
  • 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 200046103–106. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 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 199918963–72. [DOI] [PubMed] [Google Scholar]
  • 20.Chan F L, Chan J K, Leong L L. Modern imaging in the evaluation of hepatolithiasis. Hepatogastroenterology 199744358–369. [PubMed] [Google Scholar]
  • 21.Wiersema M J, Vilmann P, Giovannini M.et al Endosonography‐guided fine‐needle aspiration biopsy: diagnostic accuracy and complication assessment. Gastroenterology 19971121087–1095. [DOI] [PubMed] [Google Scholar]
  • 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 1992701498–1501. [DOI] [PubMed] [Google Scholar]
  • 24.Hillen H F. Unknown primary tumours. Postgrad Med J 200076690–693. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 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 19991190–92. [DOI] [PubMed] [Google Scholar]
  • 26.Stain S C, Baer H U, Dennison A R.et al Current management of hilar cholangiocarcinoma. Surg Gynecol Obstet 1992175579–588. [PubMed] [Google Scholar]
  • 27.Nimura Y, Kamiya J, Nagino M.et al Aggressive surgical treatment of hilar cholangiocarcinoma. J Hepatobiliary Pancreat Surg 1998552–61. [DOI] [PubMed] [Google Scholar]
  • 28.Madariaga J R, Iwatsuki S, Todo S.et al Liver resection for hilar and peripheral cholangiocarcinomas: a study of 62 cases. Ann Surg 1998227(1)70–79. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 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 1998187358–364. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 30.Meyer C G, Penn I, James L. Liver transplantation for cholangiocarcinoma: results in 207 patients. Transplantation 2000691633–1637. [DOI] [PubMed] [Google Scholar]
  • 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 20006309–316. [DOI] [PubMed] [Google Scholar]
  • 32.Heimbach J K, Gores G J, Haddock M G.et al Liver transplantation for unresectable perihilar cholangiocarcinoma. Semin Liver Dis 200424201–207. [DOI] [PubMed] [Google Scholar]
  • 33.Nordback I H, Pitt H A, Coleman J.et al Unresectable hilar cholangiocarcinoma: percutaneous versus operative palliation. Surgery 1994115597–603. [PubMed] [Google Scholar]
  • 34.Lammer J, Hausegger K A, Fluckiger F.et al Common bile duct obstruction due to malignancy: treatment with plastic versus metal stents. Radiology 1996201167–172. [DOI] [PubMed] [Google Scholar]
  • 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 1999134261–266. [DOI] [PubMed] [Google Scholar]
  • 36.Nakeeb A, Pitt H A. The role of preoperative biliary decompression in obstructive jaundice. Hepatogastroenterol 199542332–337. [PubMed] [Google Scholar]
  • 37.Eckhard R, Abou‐Rebyeh H, Adler A.et al Magnetic resonance cholangiopancreatography‐guided unilateral endoscopic stent placement for Klatskin tumors. Gastrointest Endosc 20015340–46. [DOI] [PubMed] [Google Scholar]
  • 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 199847354–362. [DOI] [PubMed] [Google Scholar]
  • 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 200153547–553. [DOI] [PubMed] [Google Scholar]
  • 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 199834977–986. [DOI] [PubMed] [Google Scholar]
  • 41.Anderson C D, Pinson C W, Berlin J.et al Diagnosis and treatment of cholangiocarcinoma. Oncologist 2004943–57. [DOI] [PubMed] [Google Scholar]
  • 42.Tsavaris N, Kosmas C, Gouveris P.et al Weekly gemcitabine for the treatment of biliary tract and gallbladder cancer. Invest New Drugs 200422193–198. [DOI] [PubMed] [Google Scholar]
  • 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 1997152403–2413. [DOI] [PubMed] [Google Scholar]
  • 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 19967593–600. [DOI] [PubMed] [Google Scholar]
  • 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 19989653–656. [DOI] [PubMed] [Google Scholar]
  • 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 199639852–855. [DOI] [PMC free article] [PubMed] [Google Scholar]
  • 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 199739929–935. [DOI] [PubMed] [Google Scholar]
  • 48.Pitt H A, Nakeeb A, Abrams R A.et al Peri‐hilar cholangiocarcinoma: Post‐operative radiation therapy does not improve survival. Ann Surg 1995221788–798. [DOI] [PMC free article] [PubMed] [Google Scholar]

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