Abstract
Intrahepatic cholangiocarcinoma (ICC) is the second most common primary liver tumor after hepatocellular carcinoma. In recent years, there has been an increasing trend in the incidence and mortality rate of ICC worldwide. In general, the outcome of patients with ICC is dismal, mainly due to late diagnosis and lack of effective treatment options. Detection of the tumor at early stages may make surgical resection, as the only potentially curative treatment, more feasible. Identifying ICC-specific diagnostic and prognostic biomarkers has been a focus of interest among many investigators. Unfortunately, despite recent developments in imaging modalities and laboratory tests, the diagnosis of ICC remains challenging and the majority of patients are not amenable to surgical resection at the time of presentation. Herein, we provide an overview of the current modalities used in the diagnosis of ICC.
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References
Razumilava N, Gores GJ. Cholangiocarcinoma. Lancet. 2014;383(9935):2168–79.
Hill MA, Alexander WB, Guo B, Kato Y, Patra KC, O’Dell MR, et al. Kras and Tp53 mutations cause cholangiocyte-and hepatocyte-derived cholangiocarcinoma. Cancer Res. 2018;78(16):4445–51.
Andersen JB. Molecular pathogenesis of intrahepatic cholangiocarcinoma. J Hepatobiliary Pancreat Sci. 2015;22(2):101–13.
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(12):419.
Banales JM, Cardinale V, Carpino G, Marzioni M, Andersen JB, Invernizzi P, et al. Expert consensus document: cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA). Nat Rev Gastroenterol Hepatol. 2016;13(5):261.
Blechacz B, Komuta M, Roskams T, Gores GJ. Clinical diagnosis and staging of cholangiocarcinoma. Nat Rev Gastroenterol Hepatol. 2011;8:512.
Nathan H, Aloia TA, Vauthey J-N, Abdalla EK, Zhu AX, Schulick RD, et al. A proposed staging system for intrahepatic cholangiocarcinoma. Ann Surg Oncol. 2009;16(1):14–22.
Sasaki A, Aramaki M, Kawano K, Morii Y, Nakashima K, Yoshida T, et al. Intrahepatic peripheral cholangiocarcinoma: mode of spread and choice of surgical treatment. Br J Surg. 1998;85(9):1206–9.
Olnes MJ, Erlich R. A review and update on cholangiocarcinoma. Oncology. 2004;66(3):167–79.
Shaib YH, Davila JA, McGlynn K, El-Serag HB. Rising incidence of intrahepatic cholangiocarcinoma in the United States: a true increase? J Hepatol. 2004;40(3):472–7.
Khan S, Toledano M, Taylor-Robinson S. Epidemiology, risk factors, and pathogenesis of cholangiocarcinoma. HPB. 2008;10(2):77–82.
Alvaro D, Crocetti E, Ferretti S, Bragazzi MC, Capocaccia R. Descriptive epidemiology of cholangiocarcinoma in Italy. Dig Liver Dis. 2010;42(7):490–5.
von Hahn T, Ciesek S, Wegener G, Plentz RR, Weismüller TJ, Wedemeyer H, et al. Epidemiological trends in incidence and mortality of hepatobiliary cancers in Germany. Scand J Gastroenterol. 2011;46(9):1092–8.
Taylor-Robinson S, Toledano M, Arora S, Keegan T, Hargreaves S, Beck A, et al. Increase in mortality rates from intrahepatic cholangiocarcinoma in England and Wales 1968–1998. Gut. 2001;48(6):816–20.
Patel T. Increasing incidence and mortality of primary intrahepatic cholangiocarcinoma in the United States. Hepatology. 2001;33(6):1353–7.
Bridgewater J, Galle PR, Khan SA, Llovet JM, Park J-W, Patel T, et al. Guidelines for the diagnosis and management of intrahepatic cholangiocarcinoma. J Hepatol. 2014;60(6):1268–89.
Jaiswal M, LaRusso N, Gores GJ. Cholangiocarcinoma and pro-inflammatory cytokines. Cancer Alert. 2000;1:141–4.
Alvaro D, Bragazzi MC, Benedetti A, Fabris L, Fava G, Invernizzi P, et al. Cholangiocarcinoma in Italy: a national survey on clinical characteristics, diagnostic modalities and treatment. Results from the “cholangiocarcinoma” committee of the Italian Association for the Study of liver disease. Dig Liver Dis. 2011;43(1):60–5.
Weber SM, Jarnagin WR, Klimstra D, DeMatteo RP, Fong Y, Blumgart LH. Intrahepatic cholangiocarcinoma: resectability, recurrence pattern, and outcomes1. J Am Coll Surg. 2001;193(4):384–91.
Yedibela S, Demir R, Zhang W, Meyer T, Hohenberger W, Schönleben F. Surgical treatment of mass-forming intrahepatic cholangiocarcinoma: an 11-year Western single-center experience in 107 patients. Ann Surg Oncol. 2009;16(2):404.
Shimada K, Sano T, Sakamoto Y, Esaki M, Kosuge T, Ojima H. Surgical outcomes of the mass-forming plus Periductal infiltrating types of intrahepatic cholangiocarcinoma: a comparative study with the typical mass-forming type of intrahepatic cholangiocarcinoma. World J Surg. 2007;31(10):2016–22.
Bartella I, Dufour JF. Clinical diagnosis and staging of intrahepatic cholangiocarcinoma. J Gastrointestin Liver Dis (JGLD). 2015;24(4):481–9.
Leelawat K, Narong S, Wannaprasert J, Ratanashu-ek T. Prospective study of MMP7 serum levels in the diagnosis of cholangiocarcinoma. World J Gastroenterol. 2010;16(37):4697–703.
Singh S, Tang SJ, Sreenarasimhaiah J, Lara LF, Siddiqui A. The clinical utility and limitations of serum carbohydrate antigen (CA19-9) as a diagnostic tool for pancreatic cancer and cholangiocarcinoma. Dig Dis Sci. 2011;56(8):2491–6.
Shen WF, Zhong W, Xu F, Kan T, Geng L, Xie F, et al. Clinicopathological and prognostic analysis of 429 patients with intrahepatic cholangiocarcinoma. World J Gastroenterol. 2009;15(47):5976–82.
Liu SL, Song ZF, Hu QG, Shan D, Hu SB, Li J, et al. Serum carbohydrate antigen (CA) 19-9 as a prognostic factor in cholangiocarcinoma: a meta-analysis. Front Med China. 2010;4(4):457–62.
Tao LY, Cai L, He XD, Liu W, Qu Q. Comparison of serum tumor markers for intrahepatic cholangiocarcinoma and hepatocellular carcinoma. Am Surg. 2010;76(11):1210–3.
Rahnemai-Azar AA, Weisbrod A, Dillhoff M, Schmidt C, Pawlik TM. Intrahepatic cholangiocarcinoma: molecular markers for diagnosis and prognosis. Surg Oncol. 2017;26(2):125–37.
Xu HX, Chen LD, Liu LN, Zhang YF, Guo LH, Liu C. Contrast-enhanced ultrasound of intrahepatic cholangiocarcinoma: correlation with pathological examination. Br J Radiol. 2012;85(1016):1029–37.
D’Onofrio M, Crosara S, De Robertis R, Canestrini S, Mucelli RP. Contrast-enhanced ultrasound of focal liver lesions. AJR Am J Roentgenol. 2015;205(1):W56–66.
Weber SM, Ribero D, O’reilly EM, Kokudo N, Miyazaki M, Pawlik TM. Intrahepatic cholangiocarcinoma: expert consensus statement. HPB. 2015;17(8):669–80.
Miller G, Schwartz LH, D’Angelica M. The use of imaging in the diagnosis and staging of hepatobiliary malignancies. Surg Oncol Clin N Am. 2007;16(2):343–68.
Vilgrain V, Van Beers BE, Flejou J-F, Belghiti J, Delos M, Gautier A-L, et al. Intrahepatic cholangiocarcinoma: MRI and pathologic correlation in 14 patients. J Comput Assist Tomogr. 1997;21(1):59–65.
Corvera CU, Blumgart LH, Akhurst T, DeMatteo RP, D’Angelica M, Fong Y, et al. 18F-fluorodeoxyglucose positron emission tomography influences management decisions in patients with biliary cancer. J Am Coll Surg. 2008;206(1):57–65.
Lee SW, Kim HJ, Park JH, Park DI, Cho YK, Sohn CI, et al. Clinical usefulness of 18F-FDG PET-CT for patients with gallbladder cancer and cholangiocarcinoma. J Gastroenterol. 2010;45(5):560–6.
Maithel SK, Gamblin TC, Kamel I, Corona-Villalobos CP, Thomas M, Pawlik TM. Multidisciplinary approaches to intrahepatic cholangiocarcinoma. Cancer. 2013;119(22):3929–42.
Durot I, Wilson SR, Willmann JK. Contrast-enhanced ultrasound of malignant liver lesions. Abdom Radiol. 2018;43(4):819–47.
Jiang W, Zeng ZC, Tang ZY, Fan J, Sun HC, Zhou J, et al. A prognostic scoring system based on clinical features of intrahepatic cholangiocarcinoma: the Fudan score. Ann Oncol. 2011;22(7):1644–52.
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Abbasi, A., Rahnemai-Azar, A.A., Ronnekleiv-Kelly, S.M., Abbott, D.E., Weber, S.M. (2019). Clinical Presentation and Diagnosis. In: Pawlik, T., Cloyd, J., Dillhoff, M. (eds) Intrahepatic Cholangiocarcinoma. Springer, Cham. https://doi.org/10.1007/978-3-030-22258-1_2
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DOI: https://doi.org/10.1007/978-3-030-22258-1_2
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