Global trends in mortality from intrahepatic and extrahepatic cholangiocarcinoma

J Hepatol. 2019 Jul;71(1):104-114. doi: 10.1016/j.jhep.2019.03.013. Epub 2019 Mar 23.


Background & aims: Intrahepatic (ICC) and extrahepatic cholangiocarcinoma (ECC) have rarely been studied individually, probably due to difficulties in their diagnosis and certification. Mortality trends from these 2 neoplasms have been inconsistent over the last decades. The aim of this study was to analyze worldwide trends in mortality from ICC and ECC in selected countries.

Methods: We extracted death certification data for ICC and ECC, and population estimates from the World Health Organization and Pan American Health Organization databases for 32 selected countries from Europe, the Americas, and Australasia from 1995 to 2016. We computed age-standardized (world population) mortality rates from ICC and ECC, and performed joinpoint regression analysis.

Results: Mortality rates from ICC increased in all countries considered, with a levelling off over recent years in Germany (women), Italy (men), Argentina (men), the USA (men), Hong Kong (men), and Japan (both sexes). The highest rates in 2010-2014 (1.5-2.5/100,000 in men and 1.2-1.7/100,000 in women) were registered in Hong Kong, France, Austria, Spain, the UK, and Australia. The lowest rates (0.2-0.6/100,000 in both sexes) were registered in Latin American and eastern European countries. Mortality from ECC decreased in most of the countries considered, with rates below 1/100,000 in both sexes between 2010 and 2014, with the only exception being Japan (2.8/100,000 in men and 1.4/100,000 in women).

Conclusions: Increasing mortality from ICC was observed globally, due to trends in risk factors and possibly, in part, due to better disease classification. Mortality from ECC levelled off or decreased, most likely following the increased use of laparoscopic cholecystectomy.

Lay summary: Biliary tract cancers include intrahepatic cholangiocarcinoma (ICC) and extrahepatic cholangiocarcinoma (ECC), however there are some differences in their risk factors. Consequently, the distinction between ICC and ECC is important. Over the last few decades, mortality from ICC has tended to rise in several areas of the world, following the increased prevalence of its major risk factors. In contrast, mortality from ECC tended to decrease in most countries, following the increased use of laparoscopic cholecystectomy.

Keywords: Extrahepatic cholangiocarcinoma; Intrahepatic cholangiocarcinoma; Mortality; Trends; Worldwide.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Age Factors
  • Bile Duct Neoplasms* / mortality
  • Bile Duct Neoplasms* / pathology
  • Bile Ducts, Extrahepatic / pathology*
  • Bile Ducts, Intrahepatic / pathology*
  • Cholangiocarcinoma* / mortality
  • Cholangiocarcinoma* / pathology
  • Female
  • Global Health* / statistics & numerical data
  • Global Health* / trends
  • Humans
  • Male
  • Middle Aged
  • Mortality / trends*
  • Prevalence
  • Risk Factors
  • Sex Factors