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What is CCA? — 2 of 2
The incidence and mortality of CCA are rising
The epidemiological profile of CCA varies with geographical location. In Western countries, incidence of CCA has increased over the past few decades5,8
In most countries, CCA is a rare cancer with an incidence of <6 cases per 100,000 people8
Over the past few decades in Western Europe:
- Age-adjusted incidence rates of iCCA have increased5
- Incidence of eCCA has followed a stable-to-decreasing trend5,8
The EU mortality from iCCA increased by ~9% in both sexes from 1990 to 2008, accounting for:9
~50%
of liver cancer deaths
in women9
25%
of liver cancer deaths
in men9
Date of preparation: April 2023
References
- Swiss Professional Information PEMAZYRE® (pemigatinib) on www.swissmedicinfo.ch.
- Rizvi S and Borad MJ. The rise of the FGFR inhibitor in advanced biliary cancer: the next cover of Time magazine? J Gastrointest Oncol. 2016;7:789–96.
- Ghouri YA, et al. Cancer review: Cholangiocarcinoma. J Carcinog. 2015;14:1.
- Sharma P and Yadav S. Demographics, tumor characteristics, treatment, and survival of patients with Klatskin tumors. Ann Gastroenterol. 2018;31:231–6.
- Blechacz B. Cholangiocarcinoma: Current knowledge and new developments. Gut Liver. 2017;11:13–26.
- Valle JW, et al. New horizons for precision medicine in biliary tract cancers. Cancer Discov. 2017;7:943–62.
- Bañales JM, et al. Cholangiocarcinoma 2020: The next horizon in mechanisms and management. Nat Rev Gastroenterol Hepatol. 2020;17:557–88.
- Bañales JM, 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;13:261–80.
- Bertuccio P, et al. A comparison of trends in mortality from primary liver cancer and intrahepatic cholangiocarcinoma in Europe. Ann Oncol. 2013;24:1667–74.
- Blechacz B, et al. Clinical diagnosis and staging of cholangiocarcinoma. Nat Rev Gastroenterol Hepatol. 2011;8:512–22.
- Forner A, et al. Clinical presentation, diagnosis and staging of cholangiocarcinoma. Liver Int. 2019;39(suppl 1):98–107.
- Vogel A, et al. Biliary tract cancer: ESMO Clinical Practice Guideline for diagnosis, treatment and follow-up. Ann Oncol. 2023;34:127–40.
- Lowery MA, et al. Comprehensive molecular profiling of intrahepatic and extrahepatic cholangiocarcinomas: potential targets for intervention. Clin Cancer Res. 2018;24:4154–61.
- Jain A, et al. Cholangiocarcinoma with FGFR genetic aberrations: A unique clinical phenotype. JCO Precis Oncol. 2018;2:1–12.
- Ross JS, et al. New routes to targeted therapy of intrahepatic cholangiocarcinomas revealed by next-generation sequencing. Oncologist. 2014;19:235–42.
- Farshidfar F, et al. Integrative genomic analysis of cholangiocarcinoma identifies distinct IDH-mutant molecular profiles. Cell Rep. 2017;18:2780–94.
- Graham RP, et al. Fibroblast growth factor receptor 2 translocations in intrahepatic cholangiocarcinoma. Hum Pathol. 2014;45:1630–8.
- Churi CR, et al. Mutation profiling in cholangiocarcinoma: Prognostic and therapeutic implications. PLoS One. 2014;9:e115383.
- Arai Y, et al. Fibroblast growth factor receptor 2 tyrosine kinase fusions define a unique molecular subtype of cholangiocarcinoma. Hepatology. 2014;59:1427–34.
- Borad MJ, et al. Fibroblast growth factor receptor 2 fusions as a target for treating cholangiocarcinoma. Curr Opin Gastroenterol. 2015;31:264–68.
- Silverman IM, et al. Clinicogenomic analysis of FGFR2-rearranged cholangiocarcinoma identifies correlates of response and mechanisms of resistance to pemigatinib. Cancer Discov. 2021;11:326–39.
- Barr FG. Fusion genes in solid tumors: The possibilities and the pitfalls. Expert Rev Mol Diagn. 2016;16:921–3.
- Liu PCC, et al. INCB054828 (pemigatinib), a potent and selective inhibitor of fibroblast growth factor receptors 1, 2, and 3, displays activity against genetically defined tumor models. PLoS One. 2020;15:e0231877.
- Abou-Alfa GK, et al. Pemigatinib for previously treated, locally advanced or metastatic cholangiocarcinoma: A multicentre, open-label, phase 2 study. Lancet Oncol. 2020;21:671–84.
All references are available upon request
Abbreviations
Abbreviated Prescribing Information
▼ This medicinal product is subject to additional monitoring. For further information, see product information Pemazyre on www.swissmedicinfo.ch.
Revision date: January 2023. Marketing authorisation holder: Incyte Biosciences International Sàrl, CH-1110 Morges. Refer to www.swissmedicinfo.ch for detailed information.
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