Cited by 1,626Cholangiocarcinoma and Gallbladder Cancer StudiesPediatric Hepatobiliary Diseases and TreatmentsLiver Diseases and Immunity

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Cholangiocarcinoma — evolving concepts and therapeutic strategies

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Ilyas, Sumera I., Khan, Shahid A., Hallemeier, Christopher L., Kelley, Robin K., Gores, Gregory J.·2018·Nature Reviews Clinical Oncology

DOI 10.1038/nrclinonc.2017.157PMC PMC5819599

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Abstract

Cholangiocarcinoma is a disease entity comprising diverse epithelial tumours with features of cholangiocyte differentiation: cholangiocarcinomas are categorized according to anatomical location as intrahepatic (iCCA), perihilar (pCCA), or distal (dCCA). Each subtype has a distinct epidemiology, biology, prognosis, and strategy for clinical management. The incidence of cholangiocarcinoma, particularly iCCA, has increased globally over the past few decades. Surgical resection remains the mainstay of potentially curative treatment for all three disease subtypes, whereas liver transplantation after neoadjuvant chemoradiation is restricted to a subset of patients with early stage pCCA. For patients with advanced-stage or unresectable disease, locoregional and systemic chemotherapeutics are the primary treatment options. Improvements in external-beam radiation therapy have facilitated the treatment of cholangiocarcinoma. Moreover, advances in comprehensive whole-exome and transcriptome sequencing have defined the genetic landscape of each cholangiocarcinoma subtype. Accordingly, promising molecular targets for precision medicine have been identified, and are being evaluated in clinical trials, including those exploring immunotherapy. Biomarker-driven trials, in which patients are stratified according to anatomical cholangiocarcinoma subtype and genetic aberrations, will be essential in the development of targeted therapies. Targeting the rich tumour stroma of cholangiocarcinoma in conjunction with targeted therapies might also be useful. Herein, we review the evolving developments in the epidemiology, pathogenesis, and management of cholangiocarcinoma.

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Authors: Ilyas, S. I., Khan, S. A., Hallemeier, C. L., Kelley, R. K., & Gores, G. J.
Affiliations: Mayo Clinic (US)
Hammersmith Hospital (GB)
University of California San Francisco Medical Center (US)
Corresponding author: Gores, Gregory J.
ORCID: Ilyas, Sumera I.
Khan, Shahid A.
Hallemeier, Christopher L.
Kelley, Robin K.
Gores, Gregory J.
Venue: Nature Reviews Clinical Oncology
Publisher: Springer Science and Business Media LLC
Published: 2017-10-10
Volume: 15
Issue: 2
Pages: 95-111
ISSN: 1759-4774 (ISSN-L)
1759-4782
DOI: 10.1038/nrclinonc.2017.157
PMC: PMC5819599
OpenAlex: W2763492611
Cited by: 1,626
References: 175
Citations by year: 2026: 88 · 2025: 164 · 2024: 252 · 2023: 247 · 2022: 253 · 2021: 272 · 2020: 209 · 2019: 82 · 2018: 51 · 2017: 1
FWCI: 19.88
Type: journal article
Language: en
Open access: green
www.ncbi.nlm.nih.gov
Licence: www.springernature.com/gp/researchers/text-and-data-mining (tdm)
www.springernature.com/gp/researchers/text-and-data-mining (vor)
Locations: Nature Reviews Clinical Oncology (publishedVersion)
PubMed (publishedVersion)
PubMed Central (acceptedVersion)
eScholarship (California Digital Library) (submittedVersion)
Funders: Cholangiocarcinoma Foundation
National Institutes of Health
Institute of Chemistry, Chinese Academy of Sciences
National Cancer Institute (R03CA212877)
National Institute of Diabetes and Digestive and Kidney Diseases (R56DK059427, R01DK059427, P30DK084567)
Topics: Health Sciences › Medicine › Surgery › Cholangiocarcinoma and Gallbladder Cancer Studies
Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
Keywords: cholangiocarcinoma, intrahepatic cholangiocarcinoma, hilar cholangiocarcinoma, distal cholangiocarcinoma, pathogenesis, surgical resection, liver transplantation, neoadjuvant chemoradiotherapy, locoregional chemotherapy, external beam radiotherapy, genetic landscape, molecular targets, precision medicine, immunotherapy, tumor stroma, biomarkers
MeSH terms: Adult, Cell Differentiation, Humans, Liver Neoplasms, Prognosis, Liver Transplantation, Cholangiocarcinoma, Neoadjuvant Therapy, Precision Medicine, Molecular Targeted Therapy, Chemoradiotherapy

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