Nationwide treatment and outcomes of perihilar cholangiocarcinoma

Anne-Marleen van Keulen, Stijn Franssen, Lydia G. van der Geest, Marieke T. de Boer, Minneke Coenraad, Lydi M. J. W. van Driel, Joris I. Erdmann, Nadia Haj Mohammad, Lara Heij, Heinz-Josef Klumpen, Eric Tjwa, Liselot Valkenburg-van Iersel, Joanne Verheij, Bas Groot Koerkamp, Pim B. Olthof*, Dutch Hepatocellular & Cholangiocarcinoma Group (DHCG)

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Background Perihilar cholangiocarcinoma (pCCA) is a rare tumour that requires complex multidisciplinary management. All known data are almost exclusively derived from expert centres. This study aimed to analyse the outcomes of patients with pCCA in a nationwide cohort.

Methods Data on all patients diagnosed with pCCA in the Netherlands between 2010 and 2018 were obtained from the Netherlands Cancer Registry. Data included type of hospital of diagnosis and the received treatment. Outcomes included the type of treatment and overall survival.

Results A total of 2031 patients were included and the median overall survival for the overall cohort was 5.2 (95% CI 4.7-5.7) months. Three-hundred-ten (15%) patients underwent surgical resection, 271 (13%) underwent palliative systemic treatment, 21 (1%) palliative local anti-cancer treatment and 1429 (70%) underwent best supportive care. These treatments resulted in a median overall survival of 29.6 (95% CI 25.2-34.0), 12.2 (95% CI 11.0-13.3), 14.5 (95%CI 8.2-20.8) and 2.9 (95% CI 2.6-3.2) months respectively. Resection rate was 13% in patients who were diagnosed in non-academic and 32% in academic centres (P ), which resulted in a survival difference in favour of academic centres. Median overall survival was 9.7 (95% CI 7.7-11.7) months in academic centres compared to 4.9 (95% CI 4.3-5.4) months in non-academic centres (P ).

Conclusions In patients with pCCA, resection rate and overall survival were higher for patients who were diagnosed in academic centres. These results show population-based outcomes of pCCA and highlight the importance of regional collaboration in the treatment of these patients.

Original languageEnglish
Pages (from-to)1945-1953
Number of pages9
JournalLiver International
Issue number8
Publication statusPublished - Aug 2021


  • cholangiocarcinoma
  • klatskin tumour
  • treatment outcome

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