Skip to main content
SpringerLink
Log in

Cholangiocarcinoma: classification, diagnosis, staging, imaging features, and management

  • Pictorial Essay
  • Published:
Abdominal Radiology Aims and scope Submit manuscript

Abstract

Cholangiocarcinoma is a relatively uncommon malignant neoplasm with poor prognosis. The distinction between extrahepatic and intrahepatic subtypes is important as epidemiological features, biologic and pathologic characteristics, and clinical course are different for both entities. This review study focuses on the role imaging plays in the diagnosis, classification, staging, and post-treatment assessment of cholangiocarcinoma.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4

(Image courtesy Dr.Ujwal Bhure)

Fig. 5

(Image courtesy Dr.Ujwal Bhure)

Fig. 6

(Image courtesy Dr.Ujwal Bhure)

Fig. 7
Fig. 8

(Image courtesy Dr.Ujwal Bhure)

Fig. 9
Fig. 10

(Image courtesy Dr.Ujwal Bhure)

Similar content being viewed by others

References

  1. Khan SA, Toledano MB, Taylor-Robinson SD (2008) Epidemiology, risk factors, and pathogenesis of cholangiocarcinoma. HPB 10(2):77–82

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  2. Saha SK, Zhu AX, Fuchs CS, Brooks GA (2016) Forty-year trend in cholangiocarcinoma incidence in the U.S.: intrahepatic disease in the rise. The Oncologist 21:594–599

    Article  PubMed  PubMed Central  Google Scholar 

  3. Nakanuma Y, Sato Y, Harada K, et al. (2010) Pathological classification of intrahepatic cholangiocarcinoma based on a new concept. World J Hepatol 2(12):419–427

    Article  PubMed  PubMed Central  Google Scholar 

  4. Yamamoto M, Takasaki K, Yoshikawa T, Ueno K, Nakano M (1998) Does gross appearance indicate prognosis in intrahepatic cholangiocarcinoma? J Surg Oncol 69(3):162–167

    Article  CAS  PubMed  Google Scholar 

  5. Sasaki A, Aramaki M, Kawano K, et al. (1998) Intrahepatic peripheral cholangiocarcinoma: mode of spread and choice of surgical treatment. Br J Surg 85(9):1206–1209

    Article  CAS  PubMed  Google Scholar 

  6. Japan LCSGo (1997) Classification of primary liver cancer. Tokyo: Kanehara

    Google Scholar 

  7. Craig JRPR, Edmonson HA (1989) Tumors of the liver and intrahepatic bile ducts. Atlas of Tumor Pathology: Washington, DC, pp 171–179

    Google Scholar 

  8. Odze RD, Goldblum JR (2014) Odze and Goldblum surgical pathology of the GI tract, liver, biliary tract and pancreas. Berlin: Elsevier Health Sciences

    Google Scholar 

  9. Mounajjed T, Chandan VS, Torbenson MS (2015) Surgical pathology of liver tumors. New York: Springer International Publishing

    Book  Google Scholar 

  10. S-i Aishima, Asayama Y, Taguchi K-i, Sugimachi K, Shirabe K, Shimada M, Sugimachi K, Tsuneyoshi M (2002) The utility of keratin 903 as a new prognostic marker in mass-forming-type intrahepatic cholangiocarcinoma. Mod Pathol 15(11):1181–1190

    Article  PubMed  Google Scholar 

  11. Lau SK, Prakash S, Geller SA, Alsabeh R (2002) Comparative immunohistochemical profile of hepatocellular carcinoma, cholangiocarcinoma, and metastatic adenocarcinoma. Hum Pathol 33(12):1175–1181. doi:10.1053/hupa.2002.130104

    Article  PubMed  Google Scholar 

  12. Hamada S, Satoh K, Hirota M, et al. (2011) Calcium-binding protein S100P is a novel diagnostic marker of cholangiocarcinoma. Cancer Sci 102(1):150–156

    Article  CAS  PubMed  Google Scholar 

  13. Kakar S, Gown AM, Goodman ZD, Ferrell LD (2007) Best practices in diagnostic immunohistochemistry: hepatocellular carcinoma versus metastatic neoplasms. Arch Pathol Lab Med 131(11):1648–1654

    PubMed  Google Scholar 

  14. Nagorney DM, Donohue JH, Farnell MB, Schleck CD, Ilstrup DM (1993) Outcomes after curative resections of cholangiocarcinoma. Arch Surg 128(8):871–878

    Article  CAS  PubMed  Google Scholar 

  15. Edge S, Byrd D, Compton C, et al. (2010) American Joint Committee on Cancer AJCC cancer staging manual. Ann Surg Oncol 17(6):1471–1474

    Article  PubMed  Google Scholar 

  16. Roayaie S, Guarrera JV, Michele QY, et al. (1998) Aggressive surgical treatment of intrahepatic cholangiocarcinoma: predictors of outcomes. J Am Coll Surg 187(4):365–372

    Article  CAS  PubMed  Google Scholar 

  17. Jiao Y, Pawlik TM, Anders RA, et al. (2013) Exome sequencing identifies frequent inactivating mutations in BAP1, ARID1A and PBRM1 in intrahepatic cholangiocarcinomas. Nat Genet 45(12):1470–1473

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  18. Isa T, Tomita S, Nakachi A, et al. (2001) Analysis of microsatellite instability, K-ras gene mutation and p53 protein overexpression in intrahepatic cholangiocarcinoma. Hepato-gastroenterology 49(45):604–608

    Google Scholar 

  19. Borger DR, Tanabe KK, Fan KC, et al. (2012) Frequent mutation of isocitrate dehydrogenase (IDH) 1 and IDH2 in cholangiocarcinoma identified through broad-based tumor genotyping. The Oncologist 17(1):72–79

    Article  CAS  PubMed  Google Scholar 

  20. Churi CR, Shroff R, Wang Y, et al. (2014) Mutation profiling in cholangiocarcinoma: prognostic and therapeutic implications. PLoS ONE 9(12):e115383. doi:10.1371/journal.pone.0115383

    Article  PubMed  PubMed Central  Google Scholar 

  21. Geynisman DM, Catenacci DV (2012) Toward personalized treatment of advanced biliary tract cancers. Discov Med 14(74):41–57

    PubMed  Google Scholar 

  22. Allen RA, Lisa JR (1949) Combined liver cell and bile duct carcinoma. Am J Pathol 25:647–655

    CAS  PubMed  PubMed Central  Google Scholar 

  23. Zhang F, Chen XP, Zhang W, et al. (2008) COmbined Heaptocellular cholangiocarcinoma originating from hepatic progenitor cells: immunohistochecmical and double-fluorescence immunostaining evidence. Histopathology 52(2):224–232

    Article  CAS  PubMed  Google Scholar 

  24. The Liver Cancer Study Group of Japan (1989) The general rules for the clinical and pathological study of primary lliver cancer. Jpn J Surg 19:98–129

    Article  Google Scholar 

  25. Fowler KJ, Sheybani A, Parker RA III, et al. (2013) Combined hepatocellular and cholangiocarcinoma (Biphenotypic) tumors: imaging features and diagnostic accuracy of contrast-enhanced CT and MRI. AJR 201:332–339

    Article  PubMed  Google Scholar 

  26. Edmonson HA, Washington DC, Clemett AR. Tumors of the liver and intrahepatic bile ducts: atlas of tumor pathology, sec 7, fasc. 25 Armed Forces Institute of Pathology,. [Cross Reference] Carcinoma of the major bile ducts2000. 894-903 p.

  27. Clemett AR (1965) Carcinoma of the major bile ducts. Radiology 84:894–903. doi:10.1148/84.5.894

    Article  CAS  PubMed  Google Scholar 

  28. Blechacz B, Komuta M, Roskams T, Gores GJ (2011) Clinical Diagnosis and Staging of Cholangiocarcinoma. Nat Rev Gastroenterol Hepatol 8(9):512–522

    Article  PubMed  PubMed Central  Google Scholar 

  29. Farges O, Fuks D, Le Treut YP, et al. (2011) AJCC 7th edition of TNM staging accurately discriminates outcomes of patients with resectable intrahepatic cholangiocarcinoma. Cancer 117:2170–2177

    Article  PubMed  Google Scholar 

  30. Yamasaki S (2003) Intrahepatic cholangiocarcinoma: macroscopyc type and stage classification. J Hepatobiliary Pancreat Surg 10(4):288–291

    Article  PubMed  Google Scholar 

  31. DeOliveira ML, Cunningham SC, Cameron JL, et al. (2007) Cholangiocarcinoma: thirty-one-year experience with 564 patients at a single institution. Ann Surg 245(5):755–762

    Article  PubMed  PubMed Central  Google Scholar 

  32. Khan SA, Davidson BR, Goldin RD, et al. (2012) Guidelines for the diagnosis and treatment of cholangiocarcinoma: an update. Gut 61(12):1657–1669

    Article  CAS  PubMed  Google Scholar 

  33. Tsao JI, Nimura Y, Kamiya J, et al. (2000) Management of hilar cholangiocarcinoma: comparison of an American and a Japanese experience. Ann Surg 232(2):166–174

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  34. van Gulik TM, Gouma DJ (2007) Changing perspectives in the assessment of resectability of hilar cholangiocarcinoma. Ann Surg Oncol. 14(7):1969–1971. doi:10.1245/s10434-007-9394-8

    Article  PubMed  PubMed Central  Google Scholar 

  35. Lee HY, Kim SH, Lee JM, et al. (2006) Preoperative assessment of resectability of hepatic hilar cholangiocarcinoma: combined CT and cholangiography with revised criteria. Radiology. 239(1):113–121

    Article  PubMed  Google Scholar 

  36. Hong SM, Presley AE, Stelow EB, Frierson HF Jr, Moskaluk CA (2006) Reconsideration of the histologic definitions used in the pathologic staging of extrahepatic bile duct carcinoma. Am J Surg Pathol. 30(6):744–749

    Article  PubMed  Google Scholar 

  37. Hong SM, Kang GH, Lee HY, Ro JY, Nishi M, Omori Y, Okabayashi T, Yamamoto J, Kosuge T, Shimada K, Yamasaki S, Takayama T, Makuuchi M. Smooth muscle distribution in the extrahepatic bile duct: histologic and immunohistochemical studies of 122 cases. Am Pathol 24 Cross ref Miwa Research Society for Gastric Cancer JRSGC Japanese classification of gastric carcinoma Kanehara Tokyo Cross ref A new staging system for massforming intrahepatic cholangiocarcinoma analysis of preoperative and postoperative variables Cancer 23742383. 2001;92(9 SRC— GoogleScholar):660–7

  38. Jarnagin WR, Fong Y, DeMatteo RP, et al. (2001) Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma. Ann Surg 234(4):507–517

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  39. Honickman SP, Mueller PR, Wittenberg J, et al. (1983) Ultrasound in obstructive jaundice: prospective evaluation of site and cause. Radiology 147(2):511–515

    Article  CAS  PubMed  Google Scholar 

  40. Neumaier CE, Bertolotto M, Perrone R, et al. (1995) Staging of hilar cholangiocarcinoma with ultrasound. J Clin Ultrasound 23(3):173–178

    Article  CAS  PubMed  Google Scholar 

  41. Eloubeidi MA, Chen VK, Jhala NC, et al. (2004) Endoscopic ultrasound-guided fine needle aspiration biopsy of suspected cholangiocarcinoma. Clin Gastroenterol Hepatol 2(3):209–213

    Article  PubMed  Google Scholar 

  42. Zandrino F, Benzi L, Ferretti ML, et al. (2002) Multislice CT cholangiography without biliary contrast agent: technique and initial clinical results in the assessment of patients with biliary obstruction. Eur Radiol 12(5):1155–1161

    Article  CAS  PubMed  Google Scholar 

  43. Ahmetoglu A, Kosucu P, Kul S, et al. (2004) MDCT cholangiography with volume rendering for the assessment of patients with biliary obstruction. Am J Roentgenol 183(5):1327–1332

    Article  Google Scholar 

  44. Yoon HJ, Kim YK, Jang KT, et al. (2013) Intraductal papillary neoplasm of the bile ducts: description of MRI and added value of diffusion-weighted MRI. Abdom Imaging 38(5):1082–1090

    Article  PubMed  Google Scholar 

  45. Lee NK, Kim S, Kim GH, et al. (2012) Diffusion-weighted imaging of biliopancreatic disorders: correlation with conventional magnetic resonance imaging. World J Gastroenterol 18(31):4102–4117. doi:10.3748/wjg.v18.i31.4102

    Article  PubMed  PubMed Central  Google Scholar 

  46. Kim JY, Kim MH, Lee TY, et al. (2008) Clinical role of 18F-FDG PET-CT in suspected and potentially operable cholangiocarcinoma: a prospective study compared with conventional imaging. Am J Gastroenterol 103(5):1145–1151. doi:10.1111/j.1572-0241.2007.01710.x

    Article  PubMed  Google Scholar 

  47. Petrowsky H, Wildbrett P, Husarik DB, et al. (2006) Impact of integrated positron emission tomography and computed tomography on staging and management of gallbladder cancer and cholangiocarcinoma. J Hepatol 45(1):43–50. doi:10.1016/j.jhep.2006.03.009

    Article  PubMed  Google Scholar 

  48. Breitenstein S, Apestegui C, Clavien PA (2008) Positron emission tomography (PET) for Cholangiocarcinoma. HPB 10(2):120–121

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  49. Jadvar H, Henderson RW, Conti PS (2007) [F-18]fluorodeoxyglucose positron emission tomography and positron emission tomography: computed tomography in recurrent and metastatic cholangiocarcinoma. J Comput Assist Tomogr 31(2):223–228. doi:10.1097/01.rct.0000237811.88251.d7

    Article  PubMed  Google Scholar 

  50. Kluge R, Schmidt F, Caca K, et al. (2001) Positron emission tomography with [(18)F]fluoro-2-deoxy-d-glucose for diagnosis and staging of bile duct cancer. Hepatology 33(5):1029–1035. doi:10.1053/jhep.2001.23912

    Article  CAS  PubMed  Google Scholar 

  51. Seo S, Hatano E, Higashi T, et al. (2008) Fluorine-18 fluorodeoxyglucose positron emission tomography predicts lymph node metastasis, P-glycoprotein expression, and recurrence after resection in mass-forming intrahepatic cholangiocarcinoma. Surgery 143(6):769–777. doi:10.1016/j.surg.2008.01.010

    Article  PubMed  Google Scholar 

  52. Chung YE, Kim MJ, Park YN, et al. (2009) Varying appearances of cholangiocarcinoma: radiologic-pathologic correlation. Radiographics 29(3):683–700. doi:10.1148/rg.293085729

    Article  PubMed  Google Scholar 

  53. Wernecke K, Henke L, Vassallo P, et al. (1992) Pathologic explanation for hypoechoic halo seen on sonograms of malignant liver tumors: an in vitro correlative study. AJR Am J Roentgenol 159(5):1011–1016

    Article  CAS  PubMed  Google Scholar 

  54. Yoshida Y, Imai Y, Murakami T, et al. (1999) Intrahepatic cholangiocarcinoma with marked hypervascularity. Abdom Imaging 24(1):66–68

    Article  CAS  PubMed  Google Scholar 

  55. Nakanuma Y (2010) A novel approach to biliary tract pathology based on similarities to pancreatic counterparts: is the biliary tract an incomplete pancreas? Pathol Int 60(6):419–429

    Article  PubMed  Google Scholar 

  56. Nakanuma Y, Harada K, Sasaki M, Sato Y (2014) Proposal of a new disease concept “biliary diseases with pancreatic counterparts”. Anatomical and pathological bases. Histol Histopathol 29(1):1–10

    PubMed  Google Scholar 

  57. Kawakatsu M, Vilgrain V, Zins M, et al. (1997) Radiologic features of papillary adenoma and papillomatosis of the biliary tract. Abdom Imaging 22(1):87–90

    Article  CAS  PubMed  Google Scholar 

  58. Gonda TA, Glick MP, Sethi A, et al. (2012) Polysomy and p16 deletion by fluorescence in situ hybridization in the diagnosis of indeterminate biliary strictures. Gastrointest Endosc 75(1):74–79

    Article  PubMed  Google Scholar 

  59. Deshpande V, Konstantinidis IT, Castillo CF, et al. (2016) Intra-pancreatic distal bile duct carcinoma is morphologically, genetically, and clinically distinct from pancreatic ductal adenocarcinoma. J Gastrointest Surg . doi:10.1007/s11605-016-3108-0

    PubMed  Google Scholar 

  60. Poggi G, Amatu A, Montagna B, et al. (2009) OEM-TACE: a new therapeutic approach in unresectable intrahepatic cholangiocarcinoma. Cardiovasc Interv Radiol 32(6):1187–1192

    Article  Google Scholar 

  61. Ruys AT, van Haelst S, Busch OR, et al. (2012) Long-term survival in hilar cholangiocarcinoma also possible in unresectable patients. World J Surg 36:2179–2186

    Article  PubMed  PubMed Central  Google Scholar 

  62. Tan JCC, Coburn NG, Baxter NN, Kiss A, Law CHL (2008) Surgical management of intrahepatic cholangiocarcinoma–a population-based study. Ann Surg Oncol 15(2):600–608

    Article  PubMed  Google Scholar 

  63. Shaib YH, Davila JA, Henderson L, McGlynn KA, El-Serag HB (2007) Endoscopic and surgical therapy for intrahepatic cholangiocarcinoma in the united states: a population-based study. J Clin Gastroenterol 41(10):911–917

    Article  PubMed  Google Scholar 

  64. Nakeeb A, Tran KQ, Black MJ, et al. (2002) Improved survival in resected biliary malignancies. Surgery 132(4):555–563 (discission 63)

    Article  PubMed  Google Scholar 

  65. Meyer CG, Penn I, James L (2000) Liver transplantation for cholangiocarcinoma: results in 207 patients. Transplantation 69(8):1633–1637

    Article  CAS  PubMed  Google Scholar 

  66. De Vreede I, Steers JL, Burch PA, et al. (2000) Prolonged disease-free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma. Liver Transplant 6(3):309–316

    Article  Google Scholar 

  67. Thongprasert S (2005) The role of chemotherapy in cholangiocarcinoma. Ann Oncol 16(Suppl 2):93–96

    Google Scholar 

  68. Vauthey JN, Blumgart LH (1994) Recent advances in the management of cholangiocarcinomas. Semin Liver Dis 14(2):109–114

    Article  CAS  PubMed  Google Scholar 

  69. Chari RS, Rose BD, Wellesley MA. Treatment of cholangiocarcinoma II 2003.

Download references

Acknowledgements

Aoife Kilcoyne would like to acknowledge the support of the Mac Erlaine Scholarship, from the Academic Radiology Research Trust, St. Vincent’s Radiology Group, Dublin, Ireland. Irai S Oliveira would like to acknowledge the support from Hospital Sírio-Libanês, São Paulo, Brazil.

Author information

Authors and Affiliations

  1. Division of Abdominal Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA

    Irai S. Oliveira, Aoife Kilcoyne & Mukesh G. Harisinghani

  2. Department of Radiology, Hospital Sírio-Libanês, São Paulo, SP, 01308-050, Brazil

    Irai S. Oliveira

  3. Department of Pathology, Massachusetts General Hospital Harvard Medical School, Boston, MA, 02114, USA

    Jamie M. Everett & Mari Mino-Kenudson

  4. Department of Radiology, Sir HN – Reliance Foundation Hospital, Mumbai, 400004, India

    Karthik Ganesan

Authors
  1. Irai S. Oliveira
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Aoife Kilcoyne
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Jamie M. Everett
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Mari Mino-Kenudson
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Mukesh G. Harisinghani
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Karthik Ganesan
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Irai S. Oliveira.

Ethics declarations

Funding

No funding was received for this study.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards. This article does not contain any studies with animals performed by any of the authors. For this type of study formal consent is not required.

Informed consent

Statement of informed consent was not applicable since the manuscript does not contain any patient data.

Additional information

CME activity

This article has been selected as the CME activity for the current month. Please visit https://ce.mayo.edu/node/44446 and follow the instructions to complete this CME activity.

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Oliveira, I.S., Kilcoyne, A., Everett, J.M. et al. Cholangiocarcinoma: classification, diagnosis, staging, imaging features, and management. Abdom Radiol 42, 1637–1649 (2017). https://doi.org/10.1007/s00261-017-1094-7

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00261-017-1094-7

Keywords

Search

Navigation