BACKGROUND: Patients with primary sclerosing cholangitis (PSC) may be at higher of risk of malignancy after liver transplantation (LT) compared to other LT recipients. We aimed to determine the cumulative incidence of/risk factors for and long-term cancer-related mortality in patients with PSC after LT. METHODS: All adult patients who underwent LT for PSC without cholangiocarcinoma from 1984-2012, with follow-up through June 2015. We estimated cumulative incidence, risk factors and mortality from de novo malignancies after LT RESULTS: 293 patients were identified (mean age, 47±12 years; 63.3% males; 2.4% smoking at LT). Over a median of 11.5 years (range, 6.4-18.6), 64 patients (21.8%) developed 73 nonskin cancers, including 46 solid-organ cancers (11 renal, 11 colorectal, 7 prostate, 5 breast, 5 pancreas, 3 ovarian/endometrial/vulvar, 4 de novo cholangiocarcinoma). Twenty-two patients developed hematological malignancies (18 posttransplant lymphoproliferative diseases [PTLD], 2 Hodgkin’s disease, 2 myelodysplastic syndrome). Five patients developed melanoma. The 1-, 5-, 10- and 20-year cumulative incidences of cancer were 2.1%, 8.6%, 18.7%, and 27%, respectively. Mortality of PSC patients who developed cancer was higher than for PSC patients without cancer (HR 2.2, p<0.01). On multivariate analysis, recipient age and elevated pre-LT INR were associated with increased risk of de novo (nonskin) malignancy. CONCLUSION: The 10-year cumulative risk of cancer after LT for advanced stage PSC was 18.7%, with PTLD, colorectal and renal cell cancers being the most common. Post-LT de novo nonskin cancer decreased overall posttransplant survival. Only recipient age and elevated INR at LT were associated with increased nonskin cancer risk.
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