Cholangiocarcinoma: evolving concepts and therapeutic strategies

Cholangiocarcinoma – evolving concepts and therapeutic strategies.

Nat Rev Clin Oncol. 2017 Oct 10. doi: 10.1038/nrclinonc.2017.157. [Epub ahead of
print]

Rizvi S(1), Khan SA(2)(3), Hallemeier CL(4), Kelley RK(5), Gores GJ(1).

Author information:
(1)Division of Gastroenterology and Hepatology, Mayo Clinic, 200 First Street
Southwest, Rochester, Minnesota 55905, USA.
(2)Department of Hepatology, St Mary’s Hospital, Imperial College London, Praed
Street, London W2 1NY, UK.
(3)Department of Hepatology, Hammersmith Hospital, Imperial College London,
Ducane Road, London W12 0HS, UK.
(4)Department of Radiation Oncology, Mayo Clinic, 200 First Street Southwest,
Rochester, Minnesota 55905, USA.
(5)The University of California, San Francisco Medical Center, 505 Parnassus
Avenue, San Francisco, California 94143, USA.

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.

DOI: 10.1038/nrclinonc.2017.157
PMID: 28994423

https://www.ncbi.nlm.nih.gov/pubmed/?term=28994423