Colorectal Cancer Management in Inflammatory Bowel Disease

Risk Factors and Screening

Primary sclerosing cholangitis (PSC) significantly increases colorectal cancer risk in ulcerative colitis patients, with an absolute risk up to 31% 1
Disease duration, extent of ulcerative colitis, more severe or persistent inflammatory activity, post-inflammatory polyps, and family history of colorectal cancer are risk factors for colorectal cancer in patients with ulcerative colitis 2
Annual surveillance colonoscopy should be performed in all patients with concurrent PSC following PSC diagnosis, regardless of disease activity, extent, and duration, and initial screening colonoscopy should be performed 8 years after symptom onset in patients with UC without PSC 1
Surveillance colonoscopy should be performed during disease remission to better discriminate between dysplasia and inflammation, and chromoendoscopy with targeted biopsies is preferred as it increases dysplasia detection rate 1
Right-sided predominance of colorectal neoplasia is common in ulcerative colitis patients with primary sclerosing cholangitis, with up to 76% of cases affecting the right side of the colon 3

Colectomy is recommended in patients with high-grade colonic dysplasia or neoplasia, persistent symptomatic colonic inflammatory activity despite optimal medical therapy, or confirmed low-grade dysplasia at repeated occasions and/or multiple locations 4
Proctocolectomy is the preferred surgical approach for patients with colorectal cancer in the setting of ulcerative colitis and primary sclerosing cholangitis, with ileal pouch-anal anastomosis (IPAA) preferred over ileostomy when possible due to better functional results and lower risk of variceal formation 3

Liver transplantation may be necessary before or simultaneously with colorectal surgery if liver function is severely compromised, and for patients with recurrent peristomal variceal bleeding or decompensated cirrhosis, recurrent bacterial cholangitis, severe pruritus or jaundice despite endoscopic and pharmacological therapy, or high-grade biliary dysplasia 4, 3
Liver transplantation for early-stage cholangiocarcinoma in PSC can be performed within clinical trials 4

Aim for mucosal healing to reduce inflammation and potentially lower cancer risk 4
Ursodeoxycholic acid (UDCA) is not recommended for routine treatment of PSC or prevention of colorectal cancer or cholangiocarcinoma, despite some studies showing decreased risk of colorectal dysplasia with UDCA, as high-dose UDCA can be problematic in primary sclerosing cholangitis patients 5, 3
Treat according to standard inflammatory bowel disease guidelines, noting that ulcerative colitis associated with primary sclerosing cholangitis often has more extensive colitis, higher frequency of rectal sparing, and higher frequency of "backwash ileitis" 3

Suspect cholangiocarcinoma in newly diagnosed PSC with high-grade stricture(s) or known PSC with worsening signs/symptoms, progressive stricture(s), or new mass lesion, and evaluate with serum CA 19-9 (using cutoff of 130 U/mL for symptomatic patients) and imaging studies for early detection of biliary malignancy 4, 3
Diagnostic workup should include contrast-enhanced cross-sectional imaging as initial test, ERCP with ductal sampling (brush cytology, endobiliary biopsies) for diagnosis and staging 4
Recommend cholecystectomy in PSC patients with gallbladder polyps ≥8 mm in size or smaller polyps that are growing in size 4
Annual surveillance colonoscopy is recommended, and do not delay surveillance colonoscopy beyond 1-2 year intervals as recommended by guidelines, and do not neglect right-sided colon during surveillance as neoplasia in primary sclerosing cholangitis-ulcerative colitis has predilection for proximal colon 3, 6
Regular monitoring for cholangiocarcinoma is essential, as primary sclerosing cholangitis patients have a 7-9% 10-year cumulative incidence of cholangiocarcinoma, and colorectal cancer/dysplasia is a risk factor for cholangiocarcinoma development 3