Cholangiocarcinoma — evolving concepts and therapeutic strategies

By Sumera I. Ilyas, Shahid A. Khan, C. Hallemeier, R. Kelley, G. Gores. Nature Reviews Clinical Oncology, 2017

1,379 citations recorded by the source corpus.

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.

Subfields: Oncology, Surgery

View Paper: https://www.semanticscholar.org/paper/1460d99a0d512ae50b74641598a34a97f39acceb

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Citations in the corpus

Cholangiocarcinoma 2020: the next horizon in mechanisms and management (2020, neutral): “The three subtypes of CCA can have different risk factors, pathobiology, clinical presentations, management and prognosis, as well as distinct epidemiological trends 1,2 .”
FGF/FGFR signaling in health and disease (2020, background): “FGFR2 fusions were reported to be present in up to 13% of liver cancers such as intrahepatic cholangiocarcinoma.(638,639) FGFR2 amplifications occur in triple-negative breast cancer, and are associated with high sensitivity to FGFR inhibitors.”