Liver Cancers pp 187-198 | Cite as

Diagnosis and Staging of Cholangiocarcinoma

  • Jessica R. HaleEmail author
  • Olusola O. Faluyi


Cholangiocarcinoma which is often diagnosed late due to non-specific clinical presentation is the most common biliary tract malignancy. In general, accurate staging is crucial to identifying patients with early disease who may be amenable to surgery with curative intent or locally advanced disease for which locoregional treatment approaches may be relevant. Furthermore, accurate diagnosis is crucial to guiding systemic treatment in the adjuvant or palliative setting. Findings on detailed radiological examination with CT and/or MRI could be suggestive of the diagnosis and sufficient for staging, while laparoscopy is at times necessary to exclude intra-abdominal metastases. However, the role of PET requires further evaluation. Invasive techniques such as ERCP, SpyGlass endoscopy and PTC have been found useful for biliary tract evaluation and for therapeutic relief of biliary tract obstruction. These biliary intervention techniques and EUS-FNA have also been found useful for obtaining a confirmatory histological diagnosis. This chapter subsequently discusses the diagnosis and staging of cholangiocarcinoma in some detail with some future perspective.


Cholangiocarcinoma CT Endoscopy Histology Liver Marker Metastases MRI Radiology Staging 


  1. 1.
    Razumilava N, Gores GJ. Combination of gemcitabine and cisplatin for biliary tract cancer: a platform to build on. J Hepatol. 2011;54:577–8.CrossRefGoogle Scholar
  2. 2.
    Ahrendt SA, Pitt HA. Biliary tract. In: Townsend C, editor. Sabiston textbook of surgery. Philadelphia: W.B. Saunders Company; 2001. p. 1076–111.Google Scholar
  3. 3.
    Bismuth H, Corlette MB. Intrahepatic cholangioenteric anastomosis in carcinoma of the hilus of the liver. Surg Gynecol Obstet. 1975;140:170–8.PubMedGoogle Scholar
  4. 4.
    Hann LE, Greatrex KV, Bach AM, et al. Cholangiocarcinoma at the hepatic hilus: sonographic findings. AJR Am J Roentgenol. 1997;168(4):985–9.CrossRefGoogle Scholar
  5. 5.
    Van Beers BE. Diagnosis of cholangiocarcinoma. HPB (Oxford). 2008;10(2):87–93.CrossRefGoogle Scholar
  6. 6.
    Giorgio A, Montesarchio L, Gatti P, et al. Contrast-enhanced ultrasound: a simple and effective tool in defining a rapid diagnostic work-up for small nodules detected in cirrhotic patients during surveillance. J Gastrointestin Liver Dis. 2016;25(2):205–11.PubMedGoogle Scholar
  7. 7.
    Navaneethan U, Njei B, Venkatesh PG, Lourdusamy V, Sanaka MR. Endoscopic ultrasound in the diagnosis of cholangiocarcinoma as the etiology of biliary strictures: a systematic review and meta-analysis. Gastroenterol Rep (Oxf). 2015;3(3):209–15.CrossRefGoogle Scholar
  8. 8.
    Ruys AT, van Beem BE, Engelbrecht MR, et al. Radiological staging in patients with hilar cholangiocarcinoma: a systematic review and meta-analysis. Br J Radiol. 2012;85(1017):1255–62.CrossRefGoogle Scholar
  9. 9.
    Lee SW, Kim HJ, Park JH, et al. Clinical usefulness of 18F-FDG PET-CT for patients with gallbladder cancer and cholangiocarcinoma. J Gastroenterol. 2010;45(5):560–6.CrossRefGoogle Scholar
  10. 10.
    Lee HY, Kim SH, Lee JM, et al. Preoperative assessment of resectability of hepatic hilar cholangiocarcinoma: combined CT and cholangiography with revised criteria. Radiology. 2006;239(1):113–21.CrossRefGoogle Scholar
  11. 11.
    Tan CH, Low SC, Thng CH. APASL and AASLD consensus guidelines on imaging diagnosis of hepatocellular carcinoma: a review. Int J Hepatol. 2011;2011:519783.CrossRefGoogle Scholar
  12. 12.
    Pugacheva O, Matsui O, Kozaka K, et al. Detection of small hypervascular hepatocellular carcinomas by EASL criteria: comparison with double-phase CT during hepatic arteriography. Eur J Radiol. 2011;80(3):e201–6.CrossRefGoogle Scholar
  13. 13.
    Li R, Cai P, Ma KS, et al. Dynamic enhancement patterns of intrahepatic cholangiocarcinoma in cirrhosis on contrast-enhanced computed tomography: risk of misdiagnosis as hepatocellular carcinoma. Sci Rep. 2016;6:26772. Scholar
  14. 14.
    Vilgrain V, Van Beers BE, Flejou JF, et al. Intrahepatic cholangiocarcinoma: MRI and pathologic correlation in 14 patients. Comput Assist Tomogr. 1997;21(1):59–65.CrossRefGoogle Scholar
  15. 15.
    Huang B, Wu L, Lu XY, et al. Small intrahepatic cholangiocarcinoma and hepatocellular carcinoma in cirrhotic livers may share similar enhancement patterns at multiphase dynamic MR imaging. Radiology. 2016;281(1):150–7.CrossRefGoogle Scholar
  16. 16.
    Forner A, Vilana R, Ayuso C, et al. Diagnosis of hepatic nodules 20 mm or smaller in cirrhosis: prospective validation of the noninvasive diagnostic criteria for hepatocellular carcinoma. Hepatology. 2008;47(1):97–104.CrossRefGoogle Scholar
  17. 17.
    Charatcharoenwitthaya P, Enders FB, Halling KC, Lindor KD. Utility of serum tumor markers, imaging, and biliary cytology for detecting cholangiocarcinoma in primary sclerosing cholangitis. Hepatology. 2008;48(4):1106–17.CrossRefGoogle Scholar
  18. 18.
    Ryoo I, Lee JM, Park HS, Han JK, Choi BI. Preoperative assessment of longitudinal extent of bile duct cancers using MDCT with multiplanar reconstruction and minimum intensity projections: comparison with MR cholangiography. Eur J Radiol. 2012;81(9):2020–6.CrossRefGoogle Scholar
  19. 19.
    Petrowsky H, Wildbrett P, Husarik DB, et al. Impact of integrated positron emission tomography and computed tomography on staging and management of gallbladder cancer and cholangiocarcinoma. J Hepatol. 2006;45:43–50.CrossRefGoogle Scholar
  20. 20.
    Park MS, Kim TK, Kim KW, et al. Differentiation of extrahepatic bile duct cholangiocarcinoma from benign stricture: findings at MRCP versus ERCP. Radiology. 2004;233(1):234–40.CrossRefGoogle Scholar
  21. 21.
    Harewood GC, Baron TH, Stadheim LM, Kipp BR, Sebo TJ, Salomao DR. Prospective, blinded assessment of factors influencing the accuracy of biliary cytology interpretation. Am J Gastroenterol. 2004;99:1464–9.CrossRefGoogle Scholar
  22. 22.
    Navaneethan U, Hasan MK, Lourdusamy V, Njei B, Varadarajulu S, Hawes RH. Single-operator cholangioscopy and targeted biopsies in the diagnosis of indeterminate biliary strictures: a systematic review. Gastrointest Endosc. 2015;82(4):608–14.CrossRefGoogle Scholar
  23. 23.
    Esposito I. Schirmacher pathological aspects of cholangiocarcinoma. HPB (Oxford). 2008;10(2):83–6.CrossRefGoogle Scholar
  24. 24.
    Nakanuma Y, Sato Y, Harada K, Sasaki M, Xu J, Ikeda H. Pathological classification of intrahepatic cholangiocarcinoma based on a new concept. World J Hepatol. 2010;2:419–27.CrossRefGoogle Scholar
  25. 25.
    Blechacz B. Cholangiocarcinoma: current knowledge and new developments. Gut Liver. 2017;11(1):13–26.CrossRefGoogle Scholar
  26. 26.
    Spolverato G, Ejaz A, Kim Y, Sotiropoulos GC, Pau A, Alexandrescu S, et al. Tumor size predicts vascular invasion and histologic grade among patients undergoing resection of intrahepatic cholangiocarcinoma. J Gastrointest Surg. 2014;18:1284–91.CrossRefGoogle Scholar
  27. 27.
    Nagorney DM, Pawlik TM, Chun YS, et al. Perihilar bile ducts. In: Amin MB, editor. AJCC cancer staging manual. 8th ed. Chicago: AJCC; 2017. p. 311.CrossRefGoogle Scholar
  28. 28.
    Spolverato G, Bagante F, Weiss M, et al. Comparative performances of the 7th and the 8th editions of the American Joint Committee on Cancer staging systems for intrahepatic cholangiocarcinoma. J Surg Oncol. 2017; [Epub ahead of print].CrossRefGoogle Scholar
  29. 29.
    Aloia T, Pawlik TM, Taouli B, et al. Intrahepatic bile ducts. In: Amin MB, editor. AJCC cancer staging manual. 8th ed. Chicago: AJCC; 2017. p. 295.CrossRefGoogle Scholar
  30. 30.
    Ercolani G, Zanello M, Grazi GL, et al. Changes in the surgical approach to hilar cholangiocarcinoma during an 18-year period in a Western single center. J Hepatobiliary Pancreat Sci. 2010;17(3):329–37.CrossRefGoogle Scholar
  31. 31.
    Ghouri YA, Mian I, Boris Blechacz B. Cancer review: cholangiocarcinoma. J Carcinog. 2015;14:1. Published online 23 Feb 2015. Scholar
  32. 32.
    Hong SM, Pawlik TM, Cho H, et al. Depth of tumor invasion better predicts prognosis than the current American Joint Committee on Cancer T classification for distal bile duct carcinoma. Surgery. 2009;146(2):250–7.CrossRefGoogle Scholar
  33. 33.
    Krasinskas A, Pawlik TM, Mino-Kenudson M, Vauthey J-N. Distal bile duct. In: Amin MB, editor. AJCC cancer staging manual. 8th ed. Chicago: AJCC; 2017. p. 317.CrossRefGoogle Scholar
  34. 34.
    Goere D, Wagholikar GD, Pessaux P, et al. Utility of staging laparoscopy in subsets of biliary cancers: laparoscopy is a powerful diagnostic tool in patients with intrahepatic and gallbladder carcinoma. Surg Endosc. 2006;20(5):721–5.CrossRefGoogle Scholar
  35. 35.
    Weber SM, Ribero D, O'Reilly EM, Kokudo N, Miyazaki M, Pawlik TM. Intrahepatic cholangiocarcinoma: expert consensus statement. HPB (Oxford). 2015;17(8):669–80.CrossRefGoogle Scholar
  36. 36.
    Bird N, Elmasry M, Jones R, et al. Role of staging laparoscopy in the stratification of patients with perihilar cholangiocarcinoma. Br J Surg. 2017;104(4):418–25.CrossRefGoogle Scholar
  37. 37.
    Joseph S, Connor S, Garden OJ. Staging laparoscopy for cholangiocarcinoma. HPB (Oxford). 2008;10(2):116–9.CrossRefGoogle Scholar
  38. 38.
    Macias RIR, Banales JM, Sangro B, et al. The search for novel diagnostic and prognostic biomarkers in cholangiocarcinoma. Biochim Biophys Acta. 2018;1864(4 Pt B):1468–77. pii: S0925-4439(17):30275-2.CrossRefGoogle Scholar
  39. 39.
    Kirchner J, Sawicki LM, Deuschl C, et al. 18 F-FDG PET/MR imaging in patients with suspected liver lesions: value of liver-specific contrast agent Gadobenate dimeglumine. PLoS One. 2017;12(7):e0180349.CrossRefGoogle Scholar
  40. 40.
    Sia D, Hoshida Y, Villanueva A, et al. Integrative molecular analysis of intrahepatic cholangiocarcinoma reveals 2 classes that have different outcomes. Gastroenterology. 2013;144(4):829–40.CrossRefGoogle Scholar

Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  1. 1.Clatterbridge Cancer CentreWirralUK

Personalised recommendations