The impact of primary sclerosing cholangitis or inflammatory bowel disease on cholangiocarcinoma phenotype, therapy, and survival

Abstract

Background and Aim Primary sclerosing cholangitis (PSC), with or without inflammatory bowel disease (IBD), confers the risk of cholangiocarcinoma. Isolated IBD may be an independent risk factor for cholangiocarcinoma. We sought to compare cholangiocarcinoma phenotype and outcomes between patients with PSC, IBD, and neither. Methods Patients with malignancy were separated into cohorts by the presence of PSC and IBD. Data regarding demographics, clinical presentation, therapeutic regimens, and survival were collected. Statistical analysis was carried out using GraphPad and R‐Studio. Results Of 946 patients, 22 had PSC, and 18 had isolated IBD. PSC and IBD patients were younger than controls ( P < 0.001, P = 0.01). Cholangiocarcinoma prevalence was estimated at 0.01% for IBD patients, 0.6% for PSC patients, and 0.002% for all other patients. All cohorts most often presented at stage 4. PSC patients presented more often at stage 3 ( P = 0.04) and with perihilar disease ( P = 0.001). Patients with PSC or IBD received less chemotherapy ( P = 0.004, 0.01). Median overall survivals were 15 months (PSC), 11 months (IBD), and 10 months (controls) ( P = 0.79). Patients with intrahepatic tumors had longer survival ( P < 0.001). Curative intent resection improved survival in all cohorts ( P < 0.001). Multivariate regression identified resection as a predictor of improved survival. Extrahepatic, perihilar, gallbladder, and unspecified biliary tumors were predictors of death. Conclusions Cholangiocarcinoma presents at a late stage and portends dismal survival regardless of PSC or IBD status. Survival was dependent on tumor location and surgical resection. These data suggest that efforts should focus on developing protocols that are able to detect and treat cholangiocarcinoma in high‐risk populations (PSC) at an early stage.

Treatment of cholangiocarcinoma in patients with primary sclerosing cholangitis: a comprehensive review

“…94 95 Patients with PSC are less likely to receive active palliative therapy, which might be ascribed to late diagnosis, rapid deterioration and underlying liver dysfunction. 19 Patients with locally advanced, unresectable disease, limited to the liver locoregional therapies (LRTs), have been evaluated. Transarterial chemoembolisation (TACE), EBRT, hepatic artery infusion (HAI) and transarterial radioembolisation (TARE) have reported an OS ranging from 13 to 21 months, but as of yet, LRT is not considered a standard therapy for unresectable iCCA.…”