Opinion statement
Cholangiocarcinoma is an uncommon malignant tumor arising from biliary epithelium. The incidence increases with age and usually affects individuals in their sixth or seventh decade of life. Most patients clinically present with features of biliary obstruction. Although surgical resection offers the only hope for cure, the majority of patients are found to have unresectable disease on initial presentation and carry extremely grim prognosis. This has lead to an emphasis on the role of palliative care, with the relief of biliary obstruction being the primary goal in the management of these patients. Surgical bypass was once considered as the primary means of palliating biliary obstruction, but nonsurgical placement of biliary stents is associated with lower morbidity and mortality. Newer modalities such as photodynamic therapy, brachytherapy, and high-intensity ultrasound therapy may result in improved survival and play a future role as an adjunctive therapy to surgical resection.
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References and Recommended Reading
Nakeeb A, Pitt HA, Sohn TA, et al.: Cholangiocarcinoma: A spectrum of intrahepatic, perihilar, and distal tumors. Ann Surg 1996, 224: 463.
Bismuth H, Castaing D, Traynor O: Resection or palliation: priority of surgery in the treatment of hilar cancer. World J Surg 1988, 12: 39–47.
Chapman RW: Risk factors for biliary tract carcinogenesis. Ann Oncol 1999, 10(Suppl 4): 308.
de Groen PC, Gores GJ, LaRusso NF, et al.: Biliary tract cancers. N Engl J Med 1999, 341: 1368.
Chamberlain RS, Blumgart LH: Hilar cholangiocarcinoma: a review and commentary. Ann Surg Oncol 2000, 7: 55–66.
Henke AC, Jensen CS, Cohen MB: Cytologic diagnosis of adenocarcinoma in biliary and pancreatic duct brushings. Adv Anat Pathol 2002, 9: 301–308.
Sugiyama M, Atomi Y, Wada N, et al.: Endoscopic transpapillary bile duct biopsy without sphincterotomy for diagnosing biliary strictures: A prospective comparison with bile and brush cytology. Am J Gastroenterol 1996, 91: 465.
Ponchon T, Gagnon P, Berger F, et al.: Value of endobiliary brush cytology and biopsies for the diagnosis of malignant bile duct stenosis: Results of a prospective study. Gastrointest Endosc 1995, 2: 565.
Kubota Y, Takaoka M, Tani K, et al.: Endoscopic transpapillary biopsy for diagnosis of patients with pancreaticobiliary ductal strictures. Am J Gastroenterol 1993, 88: 1700.
Rabinovitz M, Zajko AB, Hassanein T, et al.: Diagnostic value of brush cytology in the diagnosis of bile duct carcinoma: A study in 65 patients with bile duct strictures. Hepatology 1990, 12: 747.
Rumalla A, Baron TH, Leontovich O, et al.: Improved diagnostic yield of endoscopic biliary brush cytology by digital image analysis. Mayo Clin Proc 2001, 76: 29–33.
BaronTH, Harewood GC, Rumalla A, et al.: A prospective comparison of digital image analysis and routine cytology for the identification of malignancy in biliary tract strictures. Clin Gastroenterol Hepatol 2004, 2: 214–219. This study illustrates the effectiveness and efficacy of newer diagnostic techniques.
Khan SA, Davidson BR, Goldin R, et al.: Guidelines for the diagnosis and treatment of cholangiocarcinoma: consensus document. Gut 2002, 51 Suppl 6: Vl1-Vl9.
Ahrendt SA, Nakeeb A, Pitt HA: Cholangiocarcinoma. Clin Liver Dis 2001, 5: 191–218.
Konstantinos NL, Gores GJ: Cholangiocarcinoma. Gastroenterology 2005, 128: 1655–1667.
Manfredi R, Barbaro B, Masselli G, et al.: Magnetic resonance imaging of cholangiocarcinoma. Semin Liver Dis 2004, 24: 155–164. The complementary role of MRI/MRCP in the diagnosis and management of CCA.
Gores GJ: Early detection and treatment of cholangiocarcinoma. Liver Transpl 2000, 6: S30-S34.
Kipp BR, Stadheim LM, Halling SA, et al.: A comparison of routine cytology and fluorescence in situ hybridization for the detection of malignant bile duct strictures. Am J Gastroenterol 2004, 99: 1675–1681. This study illustrates the diagnostic accuracy of newer techniques like FISH.
Eloubeidi MA, Chen VK, Jhala NC, et al.: Endoscopic ultrasound -guided fine needle aspiration biopsy of suspected cholangiocarcinoma. Clin Gastroenterol Hepatol 2004, 2: 209–213. Even though a small study, it illustrates the applicability of EUS in the diagnosis of CCA.
Fritscher-Ravens A, Broering DC, Sriram PV: EUS-guided fine-needle aspiration cytodiagnosis of hilar cholangiocarcinoma: a case series. Gastrointest Endosc 2000, 52: 534–540.
Polydorou AA, Cairns SR, Dowsett JF, et al.: Palliation of proximal malignant biliary obstruction by endoscopic endoprosthesis insertion. Gut 1991, 32: 685.
JarnaginWR, Fong Y, DeMatteo RP, Gonen M, et al.: Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma [discussion 517–519]. Ann Surg 2001, 234: 507–517. This study relates to the surgical therapy, clinical staging criteria and prognostic factors for CCA, and the role of laparoscopy in clinical staging of CCA.
WeberSM, DeMatteo RP, Fong Y, et al.: Staging laparoscopy in patients with extrahepatic biliary carcinoma. Analysis of 100 patients. Ann Surg 2002, 235: 392–399. This study focuses on the surgical therapy, clinical staging criteria, and prognostic factors for CCA, and the role of laparoscopy in clinical staging of CCA.
BurkeEC, Jarnagin WR, Hochwald SN, 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. This study relates to the surgical therapy, clinical staging criteria, and prognostic factors for CCA, and the role of laparoscopy in clinical staging of CCA.
Mizumoto R, Suzuki H: Surgical anatomy of the hepatic hilum with special reference to the caudate lobe. World J Surg 1988, 12: 2–10.
Smith AC, Dowsett JF, Russell RC, et al.: Randomised trial of endoscopic stenting versus surgical bypass in malignant low bile duct obstruction. Lancet 1994, 344: 1655.
Lai EC, Chu KM, Lo CY, et al.: Choice of palliation for malignant hilar biliary obstruction. Am J Surg 1992, 163: 208.
Andersen JR, Sorensen SM, Kruse A, et al.: Randomized trial of endoscopic endoprosthesis versus operative bypass in malignant obstructive jaundice. Gut 1989, 30: 1132.
Shepherd HA, Royle G, Ross AP, et al.: Endoscopic biliary endoprosthesis in the palliation of malignant obstruction of the distal common bile duct: a randomized trial. Br J Surg 1988, 75: 1166.
Stain SC, Baer HU, Dennison AR, Blumgart LH: Current management of hilar cholangiocarcinoma. Surg Gynecol Obstet 1992, 175: 579–88.
Levy MJ, Baron TH, Gostout CJ, et al.: Palliation of malignant extrahepatic biliary obstruction with plastic versus expandable metal stents: an evidence-based approach. Clin Gastroenterol Hepatol 2004, 2: 273–285. A detailed overview of evidence available to date and current recommendations on stent type for palliation therapy in CCA patients.
Raijman I: Biliary and pancreatic stents. Gastrointest Endoscopy Clin N Am 2003, 13: 561–592.
De Palma GD, Pezzullo A, Rega M, et al.: Unilateral placement of metallic stents for malignant hilar obstruction: a prospective study. Gastrointest Endosc 2003, 58: 50–53.
Hintze RE, Abou-Rebyeh H, Adler A, et al.: Magnetic resonance cholangiopancreatography-guided unilateral endoscopic stent placement for Klatskin tumors. Gastrointest Endosc 2001, 53: 40–46.
Freeman ML, Overby C: Selective MRCP and CTtargeted drainage of malignant hilar biliary obstruction with self-expanding metallic stents. Gastrointest Endosc 2003, 58: 41.
Kaassis M, Boyer J, Dumas R, et al.: Plastic or metal stents for malignant stricture of the common bile duct? Results of a randomized prospective study. Gastrointest Endosc 2003, 57: 178–182.
Chang WH, Kortan P, Haber GB: Outcome in patients with bifurcation tumors who undergo unilateral versus bilateral hepatic duct drainage. Gastrointest Endosc 1998, 47: 354–362.
Peters RA, Williams SG, Lombard M, et al.: The management of high-grade hilar strictures by endoscopic insertion of self-expanding metal endoprostheses. Endoscopy 1997, 29: 10–16.
Dowsett JF, Vaira D, Hatfield AR, Cairns SR, et al.: Endoscopic biliary therapy using the combined percutaneous and endoscopic technique. Gastroenterology 1989, 96: 1180–1186.
Deviere J, Baize M, de Toeuf J, Cremer M: Long-term follow-up of patients with hilar malignant stricture treated by endoscopic internal biliary drainage. Gastrointest Endosc 1988, 34: 95–101.
De Palma GD, Galloro G, Siciliano 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, 53: 547–553.
Sherman S: Endoscopic drainage of malignant hilar obstruction: is one biliary stent enough or should we work to place two? Gastrointest Endosc 2001, 53: 681–684.
Bruha R, Petrtyl J, Kubecova M, et al.: Intraluminal brachytherapy and self expandable stents in nonresectable biliary malignancies--the question of long-term palliation. Hepatogastroenterology 2001, 48: 631–637. Role of brachytherapy in the management of CCA.
Montemaggi P, Costamagna G, Dobelbower RR, et al.: Intraluminal brachytherapy in the treatment of pancreas and bile duct carcinoma. Int J Radiat Oncol Biol Phys 1995, 32: 437–443.
Gerhards MF, van Gulik TM, Gonzalez D, et al.: Results of postoperative radiotherapy for resectable hilar cholangiocarcinoma. World J Surg 2003, 27: 173–179.
Rumalla A, Baron TH, Wang KK, et al.: Endoscopic application of photodynamic therapy for cholangiocarcinoma. Gastrointest Endosc 2001, 53: 500–504.
Berr F: Photodynamic therapy for cholangiocarcinoma. Semin Liver Dis 2004, 24: 177–187. This article describes the emerging role of PDT in the management algorithm of CCA.
Ortner ME, Caca K, Berr F, et al.: Successful photodynamic therapy for nonresectable cholangiocarcinoma: a randomized prospective study. Gastroenterology 2003, 125: 1355–1363.
Prat F, Lafon C, Margonari J, et al.: A high-intensity US probe designed for intraductal tumor destruction: experimental results. Gastrointest Endosc 1999, 50: 388–392.
Prat F, Lafon C, Theilliere JY, et al.: Destruction of a bile duct carcinoma by intraductal high intensity ultrasound during ERCP. Gastrointest Endosc 2001, 53: 797–800.
Prat F, Lafon C, De Lima DM, et al.: Endoscopic treatment of cholangiocarcinoma and carcinoma of the duodenal papilla by intraductal high-intensity US: Results of a pilot study. Gastrointest Endosc 2002, 56: 909–915.
Gores GJ: Cholangiocarcinoma:current concepts and insights. Hepatology 2003, 37: 961–969.
Sudan D, DeRoover A, Chinnakotia S, et al.: Radiochemotherapy and transplantation allow long-term survival for nonresectable hilar cholangiocarcinoma. Am J Transplant 2002, 2: 774–779.
De Vreede I, Steers JL, Burch PA, et al.: Prolonged disease-free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma. Liver Transpl 2000, 6: 309–316.
Shimoda M, Farmer DG, Colquhoun SD, et al.: Liver transplantation for cholangiocellular carcinoma:analysis of a single-center experience and review of the literature. Liver Transpl 2001, 7: 1023–1033.
Greene FL, Page DL, Fleming ID, et al., eds: AJCC Cancer Staging Handbook, edn 6. New York: Springer-Verlag, 2002.
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Chahal, P., Baron, T.H. Cholangiocarcinoma. Curr Treat Options Gastro 8, 493–502 (2005). https://doi.org/10.1007/s11938-005-0036-6
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DOI: https://doi.org/10.1007/s11938-005-0036-6