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Anatomic and Morphologic Classifications of Cholangiocarcinoma

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Diagnosis and Management of Cholangiocarcinoma
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Abstract

Cholangiocarcinoma (CCA) is a malignant tumor that can arise from any part of the biliary tree. Anatomically, these tumors are separated into three categories based on their site of origin: intrahepatic, perihilar, and distal CCA. The morphologies, risk factors, presentation, treatment, and outcomes for each anatomic category are often variable but sometimes overlap. To that end, the American Joint Committee on Cancer (AJCC) assesses CCA by the three anatomic categories. This chapter explores the anatomic classifications and the common morphologies for each. We also review clinical and pathological staging according to the AJCC staging manual.

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Abbreviations

AJCC:

American Joint Committee on Cancer

CCA:

Cholangiocarcinoma

dCCA:

Distal cholangiocarcinoma

iCCA:

Intrahepatic cholangiocarcinoma

IG:

Intraductal growing

LCSGJ:

The Liver Cancer Study Group of Japan

MF:

Mass-forming

MSKCC:

Memorial Sloan Kettering Cancer Center

pCCA:

Perihilar cholangiocarcinoma

PI:

Periductal infiltrating

TNM:

Tumor, lymph node, metastases

References

  1. Bergquist A, von Seth E. Epidemiology of cholangiocarcinoma. Best Pract Res Clin Gastroenterol. 2015;29(2):221–32.

    Article  Google Scholar 

  2. Wu L, Tsilimigras DI, Paredes AZ, Mehta R, Hyer JM, Merath K, et al. Trends in the incidence, treatment and outcomes of patients with intrahepatic cholangiocarcinoma in the USA: facility type is associated with margin status, use of lymphadenectomy and overall survival. World J Surg. 2019;43(7):1777–87.

    Article  Google Scholar 

  3. Yamasaki S. Intrahepatic cholangiocarcinoma: macroscopic type and stage classification. J Hepato-Biliary-Pancreat Surg. 2003;10(4):288–91.

    Article  Google Scholar 

  4. Zhang XF, Lv Y, Weiss M, Popescu I, Marques HP, Aldrighetti L, et al. Should utilization of lymphadenectomy vary according to morphologic subtype of intrahepatic cholangiocarcinoma? Ann Surg Oncol. 2019;26(7):2242–50.

    Article  Google Scholar 

  5. Bagante F, Weiss M, Alexandrescu S, Marques HP, Aldrighetti L, Maithel SK, et al. Long-term outcomes of patients with intraductal growth sub-type of intrahepatic cholangiocarcinoma. HPB (Oxford). 2018;20(12):1189–97.

    Article  Google Scholar 

  6. Aloia TA, Pawlik TM, Taouli B, Rubbia-Brandt L, Vauthey JN. Intrahepatic bile ducts. In: Amin MB, editor. AJCC cancer staging manual. 8th ed. Chicago: American Joint Committee on Cancer; 2017. p. 295–302.

    Chapter  Google Scholar 

  7. Bagante F, Spolverato G, Weiss M, Alexandrescu S, Marques HP, Aldrighetti L, et al. Impact of morphological status on long-term outcome among patients undergoing liver surgery for intrahepatic cholangiocarcinoma. Ann Surg Oncol. 2017;24(9):2491–501.

    Article  Google Scholar 

  8. Mavros MN, Economopoulos KP, Alexiou VG, Pawlik TM. Treatment and prognosis for patients with intrahepatic cholangiocarcinoma: systematic review and meta-analysis. JAMA Surg. 2014;149(6):565–74.

    Article  Google Scholar 

  9. 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.

    Article  Google Scholar 

  10. Spolverato G, Bagante F, Weiss M, Alexandrescu S, Marques HP, Aldrighetti L, 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;115(6):696–703.

    Article  Google Scholar 

  11. Amin MB; American Joint Committee on Cancer, American Cancer Society. AJCC cancer staging manual. 8th ed. In: Amin MB, Editor-in-chief, Edge SB, et al., Gress DM, RHIT, CTR, Technical editor, Meyer LR, CAPM, Managing editor. Chicago: American Joint Committee on Cancer/Springer; 2017. xvii, 1024 p.

    Google Scholar 

  12. Edge SB; American Joint Committee on Cancer. AJCC cancer staging manual. 7th ed. New York: Springer; 2010. xiv, 648 p.

    Google Scholar 

  13. Nathan H, Aloia TA, Vauthey JN, Abdalla EK, Zhu AX, Schulick RD, et al. A proposed staging system for intrahepatic cholangiocarcinoma. Ann Surg Oncol. 2009;16(1):14–22.

    Article  Google Scholar 

  14. Igami T, Ebata T, Yokoyama Y, Sugawara G, Takahashi Y, Nagino M. Staging of peripheral-type intrahepatic cholangiocarcinoma: appraisal of the new TNM classification and its modifications. World J Surg. 2011;35(11):2501–9.

    Article  Google Scholar 

  15. Ronnekleiv-Kelly SM, Pawlik TM. Staging of intrahepatic cholangiocarcinoma. Hepatobiliary Surg Nutr. 2017;6(1):35–43.

    Article  Google Scholar 

  16. Bagante F, Spolverato G, Merath K, Weiss M, Alexandrescu S, Marques HP, et al. Intrahepatic cholangiocarcinoma tumor burden: a classification and regression tree model to define prognostic groups after resection. Surgery. 2019;166(6):983–90.

    Article  Google Scholar 

  17. Buettner S, Ten Cate DWG, Bagante F, Alexandrescu S, Marques HP, Lamelas J, et al. Survival after resection of multiple tumor foci of intrahepatic cholangiocarcinoma. J Gastrointest Surg. 2019;23(11):2239–46.

    Article  Google Scholar 

  18. Zhang XF, Xue F, Dong DH, Weiss M, Popescu I, Marques HP, et al. Number and station of lymph node metastasis after curative-intent resection of intrahepatic cholangiocarcinoma impact prognosis. Ann Surg. 2020. Ahead of print.

    Google Scholar 

  19. Nagorney DM, Pawlik TM, Chun YS, Ebata T, Vauthey JN. Perihilar bile ducts. In: Amin MB, editor. AJCC cancer staging manual. 8th ed. Chicago: American Joint Committee on Cancer; 2017. p. 311–6.

    Chapter  Google Scholar 

  20. Klatskin G. Adenocarcinoma of the hepatic duct at its bifurcation within the porta hepatis. An unusual tumor with distinctive clinical and pathological features. Am J Med. 1965;38:241–56.

    Article  CAS  Google Scholar 

  21. Matsuo K, Rocha FG, Ito K, D'Angelica MI, Allen PJ, Fong Y, et al. The Blumgart preoperative staging system for hilar cholangiocarcinoma: analysis of resectability and outcomes in 380 patients. J Am Coll Surg. 2012;215(3):343–55.

    Article  Google Scholar 

  22. Bismuth H, Corlette MB. Intrahepatic cholangioenteric anastomosis in carcinoma of the hilus of the liver. Surg Gynecol Obstet. 1975;140(2):170–8.

    CAS  PubMed  Google Scholar 

  23. Bismuth H, Nakache R, Diamond T. Management strategies in resection for hilar cholangiocarcinoma. Ann Surg. 1992;215(1):31–8.

    Article  CAS  Google Scholar 

  24. Deoliveira ML, Schulick RD, Nimura Y, Rosen C, Gores G, Neuhaus P, et al. New staging system and a registry for perihilar cholangiocarcinoma. Hepatology. 2011;53(4):1363–71.

    Article  Google Scholar 

  25. Burke EC, Jarnagin WR, Hochwald SN, Pisters PW, Fong Y, Blumgart LH. Hilar Cholangiocarcinoma: patterns of spread, the importance of hepatic resection for curative operation, and a presurgical clinical staging system. Ann Surg. 1998;228(3):385–94.

    Article  CAS  Google Scholar 

  26. Jarnagin WR, Fong Y, DeMatteo RP, Gonen M, Burke EC, Bodniewicz BJ, et al. Staging, resectability, and outcome in 225 patients with hilar cholangiocarcinoma. Ann Surg. 2001;234(4):507–17; discussion 17–9.

    Article  CAS  Google Scholar 

  27. Tran TB, Ethun CG, Pawlik TM, Schmidt C, Beal EW, Fields RC, et al. Actual 5-year survivors after surgical resection of hilar cholangiocarcinoma. Ann Surg Oncol. 2019;26(2):611–8.

    Article  Google Scholar 

  28. Aoba T, Ebata T, Yokoyama Y, Igami T, Sugawara G, Takahashi Y, et al. Assessment of nodal status for perihilar cholangiocarcinoma: location, number, or ratio of involved nodes. Ann Surg. 2013;257(4):718–25.

    Article  Google Scholar 

  29. Krasinskas A, Pawlik TM, Mino-Kenudson M, Vauthey JN. Distal bile duct. In: Amin MB, editor. AJCC cancer staging manual. 8th ed. Chicago: American Joint Committee on Cancer; 2017. p. 317–25.

    Chapter  Google Scholar 

  30. Hong SM, Presley AE, Stelow EB, Frierson HF Jr, Moskaluk CA. Reconsideration of the histologic definitions used in the pathologic staging of extrahepatic bile duct carcinoma. Am J Surg Pathol. 2006;30(6):744–9.

    Article  Google Scholar 

  31. Hong SM, Pawlik TM, Cho H, Aggarwal B, Goggins M, Hruban RH, 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.

    Article  Google Scholar 

  32. Bledsoe JR, Shinagare SA, Deshpande V. Difficult diagnostic problems in pancreatobiliary neoplasia. Arch Pathol Lab Med. 2015;139(7):848–57.

    Article  CAS  Google Scholar 

  33. Pomianowska E, Grzyb K, Westgaard A, Clausen OP, Gladhaug IP. Reclassification of tumour origin in resected periampullary adenocarcinomas reveals underestimation of distal bile duct cancer. Eur J Surg Oncol. 2012;38(11):1043–50.

    Article  CAS  Google Scholar 

  34. Kiriyama M, Ebata T, Aoba T, Kaneoka Y, Arai T, Shimizu Y, et al. Prognostic impact of lymph node metastasis in distal cholangiocarcinoma. Br J Surg. 2015;102(4):399–406.

    Article  CAS  Google Scholar 

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Correspondence to Mark D. Girgis .

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Mederos, M.A., Girgis, M.D. (2021). Anatomic and Morphologic Classifications of Cholangiocarcinoma. In: Tabibian, J.H. (eds) Diagnosis and Management of Cholangiocarcinoma. Springer, Cham. https://doi.org/10.1007/978-3-030-70936-5_2

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  • DOI: https://doi.org/10.1007/978-3-030-70936-5_2

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