Cholangiocarcinoma (CCA) is a malignancy of biliary system which occurs due to hyperplasia of bile ducts epithelial cells and also it is the most common malignancy in patients with primary sclerosing cholangitis (PSC) and carries a high rate of mortality. Although the pathogenesis of CCA in PSC is largely unknown, inflammation-driven carcinogenesis concomitant with various genetic and epigenetic abnormalities are underlying factors. The majority of CCA cases develop from a dominant stricture (DS), which is defined as a stricture with a diameter < 1.5 mm in the common bile duct or < 1.0 mm in the hepatic duct. In PSC patients presenting with an abrupt aggravation of jaundice, pain, fatigue, pruritus, weight loss, or worsening liver biochemistries, CCA should be suspected and evaluated utilizing a variety of diagnostic modalities. However, early recognition of CCA in PSC remains a major challenge.Herein, we have survey the effectiveness rate of present treatments and the effect of early diagnosis of CCA in patient’s prognosis.

in this study we gathered the information of 450 liver transplant patient with definite diagnose of PSC with/without CCA from Abu Ali Sina Hospital (the largest center of hepatobiliary surgery in the south of Iran) between 2016-2021. The logistic regression method was used to identify the factors associated with CCA.

We reviewed 450 transplanted cases of PSC. Amongst these, cholangiocarcinoma was 18.2% (n =82). Significant risk factors in univariate analysis included Carbohydrate antigen 19-9 (CA 19.9), Hilar lymph node in CT Scan & MRCP, Stricture in CT scan & MRCP, mass in CT scan & MRCP, and age of PSC diagnosis, taking medication (azaram,sulfasalazine,mesalazine,ursoflor). In our study azaram medicine proved to have a remarkable protective effect among PSC patients against cholangiocarcinoma. Multiple logistic regression analysis showed that factors of CA 19-9(OR: 4.888 ; 95% CI:1.816-13.157), Hilar lymph node in CT Scan & MRCP (OR: 2.652 ; 95% CI:1.406-5.004),Stricture in CT scan (OR: 5.153 ; 95% CI:1.634-16.244) and Mass in CT scan & MRCP (OR: 2.565 ; 95% CI:1.328-4.953) are important predictors of cholangiocarcinoma

Several risk factors for CCA have been identified. Due to the late diagnosis of cholangiocarcinoma by regular examining of PSC patients and considering clinical trials and combining CT scan and MRCP reports, people at higher risk of this cancer can be identified.