Download PDF
Semin Liver Dis 2004; 24(2): 189-199
DOI: 10.1055/s-2004-828895
Copyright © 2004 by Thieme Medical Publishers, Inc., 333 Seventh Avenue, New York, NY 10001, USA.

Surgical Management of Cholangiocarcinoma

William R. Jarnagin1 , 2 , Margo Shoup2 , 3
  • 1Assistant Professor, Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York
  • 2Department of Surgery, Memorial Sloan-Kettering Cancer Center, New York, New York
  • 3Department of Surgery, Loyola Medical School, Maywood, Illinois
Further Information

Publication History

Publication Date:
11 June 2004 (online)

    Permissions and Reprints

Biliary tract cancer affects approximately 7500 Americans each year. Tumors arising from the gallbladder are the most common; those of bile duct origin, or cholangiocarcinoma, are less frequently encountered, constituting approximately 2% of all reported cancers. Although cholangiocarcinoma can arise anywhere within the biliary tree, tumors involving the biliary confluence (i.e., hilar cholangiocarcinoma) represent the majority, accounting for 40 to 60% of all cases. Twenty to 30% of cholangiocarcinomas originate in the lower bile duct, and approximately 10% arise within the intrahepatic biliary tree and will present as an intrahepatic mass. Complete resection remains the most effective and only potentially curative therapy for cholangiocarcinoma. For all patients with intrahepatic cholangiocarcinoma and nearly all patients with hilar tumors, complete resection requires a major partial hepatectomy. Distal cholangiocarcinomas, on the other hand, are treated like all periampullary malignancies and typically require pancreaticoduodenectomy. Most patients with cholangiocarcinoma present with advanced disease that is not amenable to surgical treatment, and even with a complete resection, recurrence rates are high. Adjuvant therapy (chemotherapy and radiation therapy) has not been shown clearly to reduce recurrence risk.

KEYWORDS

Cholangiocarcinoma - resection - surgery

REFERENCES

  • 1 Carriaga M T, Henson D E. Liver, gallbladder, extrahepatic bile ducts, and pancreas.  Cancer. 1995;  75 171-190
    CrossrefPubMedGoogle Scholar
  • 2 Kuwayti K, Baggenstoss A H, Stauffer M H, Priestly J I. Carcinoma of the major intrahepatic and extrahepatic bile ducts exclusive of the papilla of Vater.  Surg Gynecol Obstet. 1957;  104 357-366
    PubMedGoogle Scholar
  • 3 Burke E C, Jarnagin W R, Hochwald S N et al.. Hilar cholangiocarcinoma: patterns of spread, the importance of hepatic resection for curative operation, and a presurgical clinical staging system.  Ann Surg. 1998;  228 385-394
    CrossrefPubMedGoogle Scholar
  • 4 Fong Y, Blumgart L H, Lin E, Fortner J G, Brennan M F. Outcome of treatment for distal bile duct cancer.  Br J Surg. 1996;  83 1712-1715
    CrossrefPubMedGoogle Scholar
  • 5 Nagorney D M, Donohue J H, Farnell M B, Schleck C D, Ilstrup D M. Outcomes after curative resections of cholangiocarcinoma.  Arch Surg. 1993;  128 871-879
    CrossrefPubMedGoogle Scholar
  • 6 Nakeeb A, Pitt H A, Sohn T A et al.. Cholangiocarcinoma. A spectrum of intrahepatic, perihilar, and distal tumors.  Ann Surg. 1996;  224 463-473
    CrossrefPubMedGoogle Scholar
  • 7 Tompkins R K, Thomas D, Wile A, Longmire W P. Prognostic factors in bile duct carcinoma. Analysis of 96 cases.  Ann Surg. 1981;  194 447-457
    PubMedGoogle Scholar
  • 8 Yeo C J, Cameron J L, Sohn T A et al.. Six hundred fifty consecutive pancreaticoduodenectomies in the 1990s: pathology, complications, and outcomes.  Ann Surg. 1997;  226 248-257
    CrossrefPubMedGoogle Scholar
  • 9 Berdah S V, Delpero J R, Garcia S, Hardwigsen J, Le Treut Y P. A western surgical experience of peripheral cholangiocarcinoma.  Br J Surg. 1996;  83 1517-1521
    CrossrefPubMedGoogle Scholar
  • 10 Chu K M, Lai E C, al-Hadeedi S Y et al.. Intrahepatic cholangiocarcinoma.  World J Surg. 1997;  21 301-306
    CrossrefPubMedGoogle Scholar
  • 11 Harrison L E, Fong Y, Klimstra D S, Zee S Y, Blumgart L H. Surgical treatment of 32 patients with peripheral intrahepatic cholangiocarcinoma.  Br J Surg. 1998;  85 1068-1070
    CrossrefPubMedGoogle Scholar
  • 12 Shaib Y, El-Serag H B. The epidemiology of cholangiocarcinoma.  Semin Liver Dis. 2004;  24 115-126
    Article in Thieme ConnectPubMedGoogle Scholar
  • 13 Berthiaume E P, Wands J. The molecular pathogenesis of cholangiocarcinoma.  Semin Liver Dis. 2004;  24 127-138
    Article in Thieme ConnectPubMedGoogle Scholar
  • 14 Broome U, Olsson R, Loof L et al.. Natural history and prognostic factors in 305 Swedish patients with primary sclerosing cholangitis.  Gut. 1996;  38 610-615
    PubMedGoogle Scholar
  • 15 Pitt H A, Dooley W C, Yeo C J, Cameron J L. Malignancies of the biliary tree.  Curr Probl Surg. 1995;  32 1-90
    PubMedGoogle Scholar
  • 16 Lipsett P A, Pitt H A, Colombani P M, Boitnott J K, Cameron J L. Choledochal cyst disease. A changing pattern of presentation.  Ann Surg. 1994;  220 644-652
    PubMedGoogle Scholar
  • 17 Kubo S, Kinoshita H, Hirohashi K, Hamba H. Hepatolithiasis associated with cholangiocarcinoma.  World J Surg. 1995;  19 637-641
    CrossrefPubMedGoogle Scholar
  • 18 Watanapa P. Cholangiocarcinoma in patients with opisthorchiasis.  Br J Surg. 1996;  83 1062-1064
    PubMedGoogle Scholar
  • 19 Okuda K, Kubo Y, Okazaki N et al.. Clinical aspects of intrahepatic bile duct carcinoma including hilar carcinoma. A study of 57 autopsy proven cases.  Cancer. 1977;  39 232-246
    CrossrefPubMedGoogle Scholar
  • 20 Sako S, Seitzinger G L, Garside E. Carcinoma of the extrahepatic ducts. Review of the literature and report of six cases.  Surgery. 1957;  41 416-417
    PubMedGoogle Scholar
  • 21 Weinbren K, Mutum S S. Pathological aspects of cholangiocarcinoma.  J Pathol. 1983;  139 217-238
    PubMedGoogle Scholar
  • 22 Guthrie J A, Ward J, Robinson P J. Hilar cholangiocarcinomas: T2-weighted spin-echo and gadolinium-enhanced FLASH MR imaging.  Radiology. 1996;  201 347-351
    PubMedGoogle Scholar
  • 23 Itoh K, Fujita N, Kubo K et al.. MR imaging of hilar cholangiocarcinoma-comparative study with CT [in Japanese].  Nippon Igaku Hoshasen Gakkai Zasshi. 1992;  52 443-451
    PubMedGoogle Scholar
  • 24 Lee M G, Lee H J, Kim M H et al.. Extrahepatic biliary diseases: 3D MR cholangiopancreatography compared with endoscopic retrograde cholangiopancreatography.  Radiology. 1997;  202 663-669
    CrossrefPubMedGoogle Scholar
  • 25 Schwartz L H, Coakley F V, Sun Y et al.. Neoplastic pancreaticobiliary duct obstruction: evaluation with breath-hold MR cholangiopancreatography.  AJR Am J Roentgenol. 1998;  170 1491-1495
    PubMedGoogle Scholar
  • 26 Wetter L A, Ring E J, Pellegrini C A, Way L W. Differential diagnosis of sclerosing cholangiocarcinomas of the common hepatic duct (Klatskin tumors).  Am J Surg. 1991;  161 57-62
    CrossrefPubMedGoogle Scholar
  • 27 Lai E C, Mok F P, Fan S T et al.. Preoperative endoscopic drainage for malignant obstructive jaundice.  Br J Surg. 1994;  81 1195-1198
    CrossrefPubMedGoogle Scholar
  • 28 Pitt H A, Gomes A S, Lois J F et al.. Does preoperative percutaneous biliary drainage reduce operative risk or increase hospital cost?.  Ann Surg. 1985;  201 545-553
    PubMedGoogle Scholar
  • 29 Hochwald S N, Burke E C, Jarnagin W R, Fong Y, Blumgart L H. Association of preoperative biliary stenting with increased postoperative infectious complications in proximal cholangiocarcinoma.  Arch Surg. 1999;  134 261-266
    CrossrefPubMedGoogle Scholar
  • 30 Sarmiento J M, Nagorney D M. Hepatic resection in the treatment of perihilar cholangiocarcinoma.  Surg Oncol Clin N Am. 2002;  11 893-908
    PubMedGoogle Scholar
  • 31 Bismuth H, Nakache R, Diamond T. Management strategies in resection for hilar cholangiocarcinoma.  Ann Surg. 1992;  215 31-38
    CrossrefPubMedGoogle Scholar
  • 32 Jarnagin W R, Fong Y, DeMatteo R P et al.. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma.  Ann Surg. 2001;  234 507-519
    CrossrefPubMedGoogle Scholar
  • 33 Weber S M, DeMatteo R P, Fong Y, Blumgart L H, Jarnagin W R. Staging laparoscopy in patients with extrahepatic biliary carcinoma. Analysis of 100 patients.  Ann Surg. 2002;  235 392-399
    CrossrefPubMedGoogle Scholar
  • 34 Cameron J L, Pitt H A, Zinner M J, Kaufman S L, Coleman J. Management of proximal cholangiocarcinomas by surgical resection and radiotherapy.  Am J Surg. 1990;  159 91-97
    PubMedGoogle Scholar
  • 35 Gerhards M F, van Gulik T M, de Wit L T, Obertop H, Gouma D J. Evaluation of morbidity and mortality after resection for hilar cholangiocarcinoma-a single center experience.  Surgery. 2000;  127 395-404
    CrossrefPubMedGoogle Scholar
  • 36 Su C H, Tsay S H, Wu C C et al.. Factors influencing postoperative morbidity, mortality, and survival after resection for hilar cholangiocarcinoma.  Ann Surg. 1996;  223 384-394
    CrossrefPubMedGoogle Scholar
  • 37 Hadjis N S, Blenkharn J I, Alexander N, Benjamin I S, Blumgart L H. Outcome of radical surgery in hilar cholangiocarcinoma.  Surgery. 1990;  107 597-604
    PubMedGoogle Scholar
  • 38 Klempnauer J, Ridder G J, von Wasielewski R et al.. Resectional surgery of hilar cholangiocarcinoma: a multivariate analysis of prognostic factors.  J Clin Oncol. 1997;  15 947-954
    PubMedGoogle Scholar
  • 39 Neuhaus P, Jonas S, Bechstein W O et al.. Extended resections for hilar cholangiocarcinoma.  Ann Surg. 1999;  230 808-818
    CrossrefPubMedGoogle Scholar
  • 40 Kosuge T, Yamamoto J, Shimada K, Yamasaki S, Makuuchi M. Improved surgical results for hilar cholangiocarcinoma with procedures including major hepatic resection.  Ann Surg. 1999;  230 663-671
    CrossrefPubMedGoogle Scholar
  • 41 Nimura Y, Hayakawa N, Kamiya J, Kondo S, Shionoya S. Hepatic segmentectomy with caudate lobe resection for bile duct carcinoma of the hepatic hilus.  World J Surg. 1990;  14 535-543
    CrossrefPubMedGoogle Scholar
  • 42 Mizumoto R, Suzuki H. Surgical anatomy of the hepatic hilum with special reference to the caudate lobe.  World J Surg. 1988;  12 2-10
    CrossrefPubMedGoogle Scholar
  • 43 Kawasaki S, Makuuchi M, Miyagawa S, Kakazu T. Radical operation after portal embolization for tumor of hilar bile duct.  J Am Coll Surg. 1994;  178 480-486
    PubMedGoogle Scholar
  • 44 Makuuchi M, Thai B L, Takayasu K et al.. Preoperative portal embolization to increase safety of major hepatectomy for hilar bile duct carcinoma: a preliminary report.  Surgery. 1990;  107 521-527
    Google Scholar
  • 45 Nagino M, Nimura Y, Kamiya J et al.. Right or left trisegment portal vein embolization before hepatic trisegmentectomy for hilar bile duct carcinoma.  Surgery. 1995;  117 677-681
    PubMedGoogle Scholar
  • 46 Kitagawa Y, Nagino M, Kamiya J et al.. Lymph node metastasis from hilar cholangiocarcinoma: audit of 110 patients who underwent regional and paraaortic node dissection.  Ann Surg. 2001;  233 385-392
    CrossrefPubMedGoogle Scholar
  • 47 Kamada T, Saitou H, Takamura A, Nojima T, Okushiba S I. The role of radiotherapy in the management of extrahepatic bile duct cancer: an analysis of 145 consecutive patients treated with intraluminal and/or external beam radiotherapy.  Int J Radiat Oncol Biol Phys. 1996;  34 767-774
    PubMedGoogle Scholar
  • 48 McMasters K M, Tuttle T M, Leach S D et al.. Neoadjuvant chemoradiation for extrahepatic cholangiocarcinoma.  Am J Surg. 1997;  174 605-608
    CrossrefPubMedGoogle Scholar
  • 49 Pitt H A, Nakeeb A, Abrams R A et al.. Perihilar cholangiocarcinoma. Postoperative radiotherapy does not improve survival.  Ann Surg. 1995;  221 788-797
    PubMedGoogle Scholar
  • 50 Jarnagin W R, Burke E, Powers C, Fong Y, Blumgart L H. Intrahepatic biliary enteric bypass provides effective palliation in selected patients with malignant obstruction at the hepatic duct confluence.  Am J Surg. 1998;  175 453-460
    CrossrefPubMedGoogle Scholar
  • 51 Kuvshinoff B W, Armstrong J G, Fong Y et al.. Palliation of irresectable hilar cholangiocarcinoma with biliary drainage and radiotherapy.  Br J Surg. 1995;  82 1522-1525
    PubMedGoogle Scholar
  • 52 Bowling T E, Galbraith S M, Hatfield A R, Solano J, Spittle M F. A retrospective comparison of endoscopic stenting alone with stenting and radiotherapy in non-resectable cholangiocarcinoma.  Gut. 1996;  39 852-855
    CrossrefPubMedGoogle Scholar
  • 53 Wade T P, Prasad C N, Virgo K S, Johnson F E. Experience with distal bile duct cancers in U.S. Veterans Affairs hospitals: 1987-1991.  J Surg Oncol. 1997;  64 242-245
    CrossrefPubMedGoogle Scholar
  • 54 Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States.  Hepatology. 2001;  33 1353-1357
    CrossrefPubMedGoogle Scholar
  • 55 Kokudo N, Makuuchi M. Extent of resection and outcome after curative resection for intrahepatic cholangiocarcinoma.  Surg Oncol Clin N Am. 2002;  11 969-983
    PubMedGoogle Scholar
  • 56 Primary liver cancer in Japan. Clinicopathologic features and results of surgical treatment.  Liver Cancer Study Group of Japan . Ann Surg. 1990;  211 277-287
    PubMedGoogle Scholar
  • 57 Kobayashi M, Ikeda K, Saitoh S et al.. Incidence of primary cholangiocellular carcinoma of the liver in japanese patients with hepatitis C virus-related cirrhosis.  Cancer. 2000;  88 2471-2477
    CrossrefPubMedGoogle Scholar
  • 58 Yamasaki S. Intrahepatic cholangiocarcinoma: macrosopic type and stage classification.  J HBP Surg. 2003;  10 288-291
    PubMedGoogle Scholar
  • 59 Ohtsuka M, Ito H, Kimura F et al.. Results of surgical treatment for intrahepatic cholangiocarcinoma and clinicopathological factors influencing survival.  Br J Surg. 2002;  89 1525-1531
    CrossrefPubMedGoogle Scholar
  • 60 Okabayashi T, Yamamoto J, Kosuge T et al.. A new staging system for mass-forming intrahepatic cholangiocarcinoma: analysis of preoperative and postoperative variables.  Cancer. 2001;  92 2374-2383
    CrossrefPubMedGoogle Scholar
  • 61 Weber S M, Jarnagin W R, Klimstra D et al.. Intrahepatic cholangiocarcinoma: resectability, recurrence pattern, and outcomes.  J Am Coll Surg. 2001;  193 384-391
    CrossrefPubMedGoogle Scholar
  • 62 Pichlmayr R, Lamesch P, Weimann A, Tusch G, Ringe B. Surgical treatment of cholangiocellular carcinoma.  World J Surg. 1995;  19 83-88
    CrossrefPubMedGoogle Scholar
  • 63 Jan Y Y, Jeng L B, Hwang T L et al.. Factors influencing survival after hepatectomy for peripheral cholangiocarcinoma.  Hepatogastroenterology. 1996;  43 614-619
    PubMedGoogle Scholar
  • 64 Casavilla F A, Marsh J W, Iwatsuki S et al.. Hepatic resection and transplantation for peripheral cholangiocarcinoma.  J Am Coll Surg. 1997;  185 429-436
    CrossrefPubMedGoogle Scholar
  • 65 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 70-79
    CrossrefPubMedGoogle Scholar
  • 66 Valverde A, Bonhomme N, Farges O et al.. Resection of intrahepatic cholangiocarcinoma: a western experience.  J Hepatobiliary Pancreat Surg. 1999;  6 122-127
    CrossrefPubMedGoogle Scholar
  • 67 Inoue K, Makuuchi M, Takayama T et al.. Long-term survival and prognostic factors in the surgical treatment of mass-forming type cholangiocarcinoma.  Surgery. 2000;  127 498-505
    PubMedGoogle Scholar
  • 68 Isa T, Kusano T, Shimoji H et al.. Predictive factors for long-term survival in patients with intrahepatic cholangiocarcinoma.  Am J Surg. 2001;  181 507-511
    CrossrefPubMedGoogle Scholar

William R JarnaginM.D. 

Department of Surgery, Memorial Sloan-Kettering Cancer Center

1275 York Ave

New York, NY 10021

Email: jarnagiw@mskcc.org