Abstract

Abstract Background Primary sclerosing cholangitis (PSC) is a predisposing factor for cholangiocarcinoma(CCA).Patients with CCA have poor outcomes post-transplant.Intrahepatic CCA is difficult to detect pre-operatively in cirrhosis.Few outcomes are reported among PSC transplant patients where CCA was incidental. Aims To determine the incidence of incidental CCA (iCCA) in PSC liver explants and the impact of iCCA on post-transplant survival for patients with PSC Methods This was a retrospective cohort study in which the medical records of PSC patients who underwent transplant at QEII Health Sciences Center in Halifax between January 1987 to March 2020 were reviewed. The incidence of iCCA was calculated. Survival analysis was performed to determine the mean time to survival among iCCA and non-iCCA groups Results A total of 94 patients were transplanted for PSC during the study period.The mean age was 43 years.Twenty-six percent were women and 74% were men.Three were iCCA, one hilar and two intrahepatic CCA. All iCCAs occurred prior to 2008. All iCCA patients had contrast enhanced CT, ERCP with cholangiogram and MRI.Two of the ERCPs reported CBD related strictures and the brushings had no evidence of malignancy.CA19-9 level was measured in one and reported as normal.Contrast enhanced MRI was not available prior to 2007. The PSC patients had 1, 5, and 10-year survival rates of 93.8%, 88.6% and 81.9% respectively.One patient required re-transplant for graft failure and died within 3 months post-transplant due to complications. In all other cases the cause of death was not identified. Among patients with iCCA, two deaths were due to metastatic cholangiocarcinoma and the other due to surgical complication in the immediate post-transplant period. None survived beyond 2 years. Conclusions Observed PSC survival in this cohort was above national average in the same period.The 3 iCCA cases identified occurred before the availability of contrast enhanced MRI. Patients with iCCA and liver transplant had poor survival at 1 and 2 years.There were no iCCA identified after 2007.These findings suggest incidental cholangiocarcinoma was effectively reduced by the advent of high-resolution imaging modality, and improvements in pre-transplant screening, including tumor marker CA19-9 and stringent patient selection. Further study involving multiple centres is necessary to draw a definite conclusion. Funding Agencies None

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