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This subset of patients has been proven to be at increased risk for developing cholangiocarcinoma, gallbladder carcinoma and colorectal cancer in those with overlapping inflammatory bowel disease. Herein, we review the prevalence of these malignancies and recommend screening tools and current knowledge to reduce the disease burden in this population. Cholangiocarcinoma is the most dominant malignancy affecting <jats:styled-content style=\"fixed-case\">PSC<\/jats:styled-content> patients, with a lifetime risk ranging from 5% to 20%. We advocate for serial <jats:styled-content style=\"fixed-case\">US<\/jats:styled-content> or <jats:styled-content style=\"fixed-case\">MRI<\/jats:styled-content>\/<jats:styled-content style=\"fixed-case\">MRCP<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">CA<\/jats:styled-content> 19\u20109 to screen for cholangiocarcinoma. Gallbladder cancer has a lifetime risk around 2% in this population and we agree with annual imaging for lesions as recommended by national guidelines. Patients with <jats:styled-content style=\"fixed-case\">PSC<\/jats:styled-content> and concomitant <jats:styled-content style=\"fixed-case\">IBD<\/jats:styled-content> are at increased risk of colorectal carcinoma from time of diagnosis and therefore should likely undergo annual surveillance. The low rates of hepatocellular cancer and pancreatic cancer indicate surveillance for these malignancies is less advantageous.<\/jats:p>","DOI":"10.1111\/liv.13354","type":"journal-article","created":{"date-parts":[[2016,12,28]],"date-time":"2016-12-28T02:03:04Z","timestamp":1482890584000},"page":"1103-1109","source":"Crossref","is-referenced-by-count":41,"title":["An update on cancer risk and surveillance in primary sclerosing cholangitis"],"prefix":"10.1111","volume":"37","author":[{"given":"Jennifer L.","family":"Horsley\u2010Silva","sequence":"first","affiliation":[{"name":"Division of Gastroenterology and Hepatology Mayo Clinic  Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eduardo A.","family":"Rodriguez","sequence":"additional","affiliation":[{"name":"Division of Gastroenterology and Hepatology Mayo Clinic  Phoenix AZ USA"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diana 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