Tumour stage and overall survival in patients with intrahepatic cholangiocarcinoma and primary sclerosing cholangitis – a retrospective cohort study

Abstract

Background Patients with primary sclerosing cholangitis (PSC) carry increased risks for malignancy, among which cholangiocarcinoma (CCA) is the most frequent. We aimed to characterise a cohort of patients with PSC and intrahepatic CCA (iCCA) and to compare this cohort with CCA in different localisations.

Methods We performed a retrospective analysis of our medical database from 01.01.2007 to 30.06.2023 and differentiated CCA according to its localisation within the biliary tract into iCCA, perihilar CCA (pCCA), distal CCA (dCCA), and gallbladder carcinoma (GBC).

Results We identified 8 (28%) patients with iCCA, 14 (48%) patients with pCCA, 6 (21%) patients with GBC, and 1 (3%) patient with dCCA without significant differences in gender distribution and mean age. Mean time between diagnosis of PSC and CCA was 158±84 months for iCCA, 93±94 months for pCCA, and 77±69 months for GBC (p=0.230). At the time of CCA diagnosis, advanced-stage disease was present in 6 (75%) patients with iCCA, 13 (93%) patients with pCCA, and 2 (40%) patients with GBC (p=0.050). Only 5 (63%) patients with iCCA received curatively intended surgery, of whom 4 (80%) patients developed recurrence after a mean time of 38±31 months. Mean survival time in patients with iCCA (35±33 months) lay between patients with pCCA (14±8 months) and patients with GBC (57±58 months), but the difference was not statistically significant (p=0.131).

Conclusion Patients with PSC and iCCA showed an advanced tumour stage at diagnosis and limited long-time survival, which was classified between pCCA with worse prognosis and GBC with better prognosis.

Zusammenfassung

Hintergrund Patienten mit primär sklerosierender Cholangitis (PSC) weisen ein erhöhtes Risiko für Malignität auf, wobei das Cholangiokarzinom (CCA) die häufigste Entität darstellt. Wir untersuchten eine Kohorte von Patienten mit PSC und intrahepatischem CCA (iCCA) und verglichen diese mit CCA in anderen Lokalisationen.

Methodik Wir führten eine respektive Datenbankauswertung vom 01.01.2007 bis zum 30.06.2023 durch und teilten das CCA anhand der Lokalisation im Gallengangsystem in iCCA, perihiläres CCA (pCCA), distales CCA (dCCA) und Gallenblasenkarzinom (GBC) ein.

Ergebnisse Wir identifizierten 8 (28%) Patienten mit iCCA, 14 (48%) Patienten mit pCCA, 6 (21%) Patienten mit GBC und 1 (3%) Patient mit dCCA ohne signifikante Unterschiede in der Geschlechterverteilung und im mittleren Alter. Die mittlere Zeit zwischen PSC- und CCA-Diagnose betrug 158±84 Monate für das iCCA, 93±94 Monate für das pCCA und 77±69 Monate für das GBC (p=0.230). Zum Zeitpunkt der CCA-Diagnose bestand eine fortgeschrittene Tumorerkrankung bei 6 (75%) Patienten mit iCCA, 13 (93%) Patienten mit pCCA und 2 (40%) Patienten mit GBC (p=0.050). Nur 5 (63%) Patienten mit iCCA erhielten eine kurativ intendierte Therapie, von denen 4 (80%) Patienten ein Rezidiv nach im Mittel 38±31 Monaten entwickelten. Die mittlere Überlebenszeit bei Patienten mit iCCA (35±33 Monate) lag zwischen der von Patienten mit pCCA (14±8 Monate) und Patienten mit GBC (57±58 Monate), jedoch ohne statistisch signifikanten Unterschied (p=0.131).

Schlussfolgerung Patienten mit PSC und iCCA weisen zum Diagnosezeitpunkt häufig eine fortgeschrittene Tumorerkrankung und ein eingeschränktes Langzeitüberleben auf, das sich zwischen der schlechten Prognose des pCCA und der besseren Prognose des GBC positioniert.

Keywords

Primary sclerosing cholangitis - cholangiocarcinoma - survival - tumour stage

Schlüsselwörter

Primär sklerosierende Cholangitis - Cholangiokarzinom - Überleben - Tumorstadium

Publication History

Received: 26 September 2023

Accepted after revision: 06 November 2023

Article published online:
09 January 2024

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