@article{4f01289d7bd546a2a3891e8f1d77baf5,
title = "Systemic therapies for intrahepatic cholangiocarcinoma",
abstract = "Intrahepatic cholangiocarcinoma (iCCA) is a highly lethal hepatobiliary neoplasm whose incidence is increasing. Largely neglected for decades as a rare malignancy and frequently misdiagnosed as carcinoma of unknown primary, considerable clinical and investigative attention has recently been focused on iCCA worldwide. The established standard of care includes first-line (gemcitabine and cisplatin), second-line (FOLFOX) and adjuvant (capecitabine) systemic chemotherapy. Compared to hepatocellular carcinoma, iCCA is genetically distinct with several targetable genetic aberrations identified to date. Indeed, FGFR2 and NTRK fusions, and IDH1 and BRAF targetable mutations have been comprehensively characterised and clinical data is emerging on targeting these oncogenic drivers pharmacologically. Also, the role of immunotherapy has been examined and is an area of intense investigation. Herein, in a timely and topical manner, we will review these advances and highlight future directions of research.",
keywords = "Adjuvant therapy, Checkpoint inhibitors, FGFR, IDH",
author = "Kelley, {Robin Kate} and John Bridgewater and Gores, {Gregory J.} and Zhu, {Andrew X.}",
note = "Funding Information: RKK reports consulting fees for advisory or steering committee roles from Agios, Astra Zeneca, and Bristol-Myers Squibb (all to institution); consulting fees for IDMC membership from Genentech/Roche (to individual); and research funding (to institution) from Adaptimmune, Agios, Astra Zeneca, Bayer, Bristol-Myers Squibb, Eli Lilly, EMD Serono, Exelixis, Medimmune, Merck, Novartis, Partner Therapeutics, QED, and Taiho. JB is funded in part by the University College London Hospital/University College London biomedical centre and has no conflicts of interest relevant to this manuscript. GJG acknowledges support from Mayo Clinic, and has no conflicts of interest relevant to this manuscript. AXZ has served as a consultant for Eisai, Bristol-Myers Squibb, Merck Sharp & Dohme Corp., Novartis, AstraZeneca, Bayer, Exelisis, Lilly, Roche/Genetech. His institution has received funding from Lilly, Bayer, Bristol-Myers Squibb, Merck Sharp & Dohme Corp., and Novartis. Please refer to the accompanying ICMJE disclosure forms for further details. Funding Information: JB is funded in part by the University College London Hospital/University College London biomedical centre and GJG acknowledges support from Mayo Clinic. The authors received no financial support to produce this manuscript. Funding Information: JB is funded in part by the University College London Hospital/University College London biomedical centre and GJG acknowledges support from Mayo Clinic. The authors received no financial support to produce this manuscript. Publisher Copyright: {\textcopyright} 2019 European Association for the Study of the Liver",
year = "2020",
month = feb,
doi = "10.1016/j.jhep.2019.10.009",
language = "English (US)",
volume = "72",
pages = "353--363",
journal = "Journal of Hepatology",
issn = "0168-8278",
publisher = "Elsevier",
number = "2",
}