Primary sclerosing cholangitis and ulcerative colitis: Evidence for increased neoplastic potential

Abstract

Primary sclerosing cholangitis (PSC) is a biliary destructive disease mostly affecting patients with ulcerative colitis (UC). PSC has been suggested to be an independent risk factor for the development of colorectal malignancy in UC. Patients with PSC also have an increased risk of developing cholangiocarcinoma. This study aimed at assessing the cumulative risk of colorectal neoplasia in PSC and UC, and also to determine risk factors for the development of cholangiocarcinoma. Fifty-eight PSC patients were included. Forty PSC patients having extensive UC were each matched to two control patients of the same age, with extensive colitis and a comparable duration of the colitis, but without PSC. All UC patients had been under colonoscopic surveillance with multiple biopsies. Among the 40 PSC patients with UC, 16 developed colonic dysplasia or carcinoma, versus 10 in the control group (P < .001). The absolute cumulative risk of developing colorectal dysplasia/carcinoma in the PSC/UC groups was 9%, 31%, and 50% after 10, 20, and 25 years of disease duration. In the group with UC only, the corresponding risk was 2%, 5%, and 10%, respectively (P < .001). Ten patients with PSC developed cholangiocarcinoma, all but one having UC. In the control group, no cholangiocarcinoma occurred. Patients with PSC and UC with colorectal neoplasia developed cholangiocarcinoma significantly more often compared with patients with UC and PSC without colonic dysplasia/carcinoma (P < .02). This study demonstrates not only that patients with PSC and UC have a significantly higher risk of developing colorectal neoplasia compared with patients having UC only, but also that patients with PSC and UC having colorectal neoplasia are more prone to develop cholangiocarcinoma.

Primary sclerosing cholangitis

“…The cumulative risk of developing colorectal dysplasia/ cancer has been reported in a case-control study to be five times higher in patients with PSC plus ulcerative colitis compared with patients with ulcerative colitis alone after 20 to 25 years of ulcerative colitis duration. 45 A prospective study also found a significant increase in the development of dysplasia up to 9 years of follow-up in patients with ulcerative colitis and PSC (45%) compared with carefully matched controls with ulcerative colitis alone (16%). 61 Similarly, a historical cohort study found that dysplasia, as an indication for proctocolectomy, was significantly more common in the group of patients with ulcerative colitis and PSC compared with patients with ulcerative colitis alone.…”

“…[41][42][43][44][45] The autopsy prevalence is much higher with cholangiocarcinoma, it being reported in 30% to 42% of patients with PSC undergoing autopsy. [45][46][47][48] Cholangiocarcinoma may develop in one third of patients with PSC if follow-up is extended long enough. 29 A large clinical experience has reported an annual incidence of less than 1%.…”

“…25,45,48,53 However, patients with incidental microscopic cholangiocarcinoma in the explanted liver in whom the spread to regional lymph nodes has been carefully excluded during the liver transplantation procedure can have a posttransplantation long-term survival similar to those transplanted PSC patients without cholangiocarcinoma. 34 Thus, PSC patients with known cholangiocarcinoma undergoing liver transplantation can be expected to have a poor prognosis, and these patients may be refused transplantation.…”

Relationship between clinical parameters and the colitis-colorectal cancer interval in a cohort of patients with colorectal cancer in inflammatory bowel disease

“…The risk of neoplasia in patients with IBD combined with PSC is controversial. Several studies have indicated that patients with IBD and PSC are at a higher risk of acquiring colorectal neoplasia [54][55][56][57], whereas larger studies from the Mayo clinic reported no risk [58] or only a borderline increased risk [59].…”

Prolonged disease-free survival after orthotopic liver transplantation plus adjuvant chemoirradiation for cholangiocarcinoma

“…1 Worldwide, cholangiocarcinoma is associated with hepatolithiasis, liver fluke infestation, choledochal cysts, primary sclerosing cholangitis (PSC), and ulcerative colitis. 2,3 In Western countries, PSC is one of the main identifiable risk factors for the development of cholangiocarcinoma. 4 Presenting symptoms are often jaundice, pruritus, weight loss, anorexia, and vague abdominal discomfort.…”