Epidemiology of cholangiocarcinoma

Maria Qurashi; Mathew Vithayathil; Shahid A Khan

doi:10.1016/j.ejso.2023.107064

Epidemiology of cholangiocarcinoma

Eur J Surg Oncol. 2023 Sep 9:107064. doi: 10.1016/j.ejso.2023.107064. Online ahead of print.

Authors

Maria Qurashi¹, Mathew Vithayathil¹, Shahid A Khan²

Affiliations

¹ Department of Surgery and Cancer, Imperial College London, W12 0NN, UK.
² Liver Unit, Division of Digestive Diseases, Department of Metabolism, Digestion and Reproduction, Imperial College London, UK. Electronic address: shahid.khan@imperial.ac.uk.

PMID: 37709624
DOI: 10.1016/j.ejso.2023.107064

Abstract

Cholangiocarcinoma (CCA) represents a heterogenous set of malignancies arising from the biliary tract. Classification of CCA subdivides tumours into intrahepatic (iCCA) and extrahepatic (eCCA), with eCCA further categorised as perihilar (pCCA) and distal (dCCA) lesions. Tumour subtypes show distinct epidemiological, genetic and clinical characteristics. Global incidence and mortality are rising, with the highest rates seen in Asian populations compared to the West. There has been a divergence in recent mortality trends observed between CCA subtypes, with rising rates of iCCA seen compared with eCCA. There are several drivers for these differing trends, including specific risk factors, misclassification of CCA subtypes and variation in diagnosis and surveillance. Risk factors for CCA can be divided into hepatobiliary, extra-hepatic and environmental, with hepatobiliary diseases conferring the largest risk. Surgery represents the only curative treatment for CCA, but can only be offered to early-stage candidates who are otherwise fit; the majority of patients are therefore treated with chemotherapy and, recently, immunotherapy. Due to late-stage presentation of disease, prognosis is poor, with 5-year survival <20%.

Keywords: Cholangiocarcinoma; Epidemiology; Extrahepatic cholangiocarcinoma; Intrahepatic cholangiocarcinoma; Mortality.