What is the management approach for a patient with colorectal cancer (CRC) associated with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC)?

Management of Colorectal Cancer with Ulcerative Colitis and Primary Sclerosing Cholangitis

Patients with colorectal cancer (CRC) associated with ulcerative colitis (UC) and primary sclerosing cholangitis (PSC) require aggressive surgical management with close surveillance for both biliary and colorectal malignancies. 1

Surgical Management

Colorectal Cancer Component

• Proctocolectomy is the preferred surgical approach for patients with CRC in the setting of UC and PSC
• Ileal pouch-anal anastomosis (IPAA) is preferred over ileostomy when possible because:
  • IPAA has better functional results in PSC patients 1
  • IPAA is less complicated with variceal formation compared to ileostomy 1
  • Patients with ileostomy who develop portal hypertension are prone to peristomal varices that can cause recurrent bleeding difficult to treat 1

Timing Considerations

• If liver function is severely compromised, consider:
  • Liver transplantation before or simultaneously with colorectal surgery
  • For patients with recurrent peristomal variceal bleeding, liver transplantation may be necessary 1

Post-Surgical Surveillance

Colorectal Surveillance

• Annual surveillance colonoscopy is mandatory after surgery if any rectal mucosa remains 2
• For patients with IPAA:
  • Continue surveillance of the pouch as there is potential risk of dysplasia in the ileal pouch mucosa 1
  • Risk of neoplasia persists even after liver transplantation at approximately 1% per person per year 2

Biliary Tract Surveillance

• Regular monitoring for cholangiocarcinoma is essential as:
  • PSC patients have 7-9% 10-year cumulative incidence of cholangiocarcinoma 1
  • CRC/dysplasia is a risk factor for cholangiocarcinoma development 1
• Evaluate with:
  • Serum CA 19-9 (using cutoff of 130 U/mL for symptomatic patients) 1
  • Imaging studies for early detection of biliary malignancy

Medical Management

Chemoprevention

• UDCA is not recommended for chemoprevention of colorectal cancer in patients with UC and PSC 1
  • Despite some studies showing decreased risk of colorectal dysplasia with UDCA (risk 0.26; 95% CI, 0.06-0.92), other studies showed no benefit 1
  • High-dose UDCA can be problematic in PSC patients 1

Inflammatory Bowel Disease Management

• Treat according to standard IBD guidelines 1
• Note that UC associated with PSC often has:
  • More extensive colitis (pancolitis in 87% of cases) 1
  • Higher frequency of rectal sparing (52%) 1
  • Higher frequency of "backwash ileitis" (51%) 1
  • Right-sided predominance of colorectal neoplasia (up to 76%) 1

Special Considerations

Post-Liver Transplantation

• Continue annual colonoscopic surveillance after liver transplantation 2
• Risk of colorectal neoplasia persists and may even increase after transplantation 2
• Prophylactic proctocolectomy is not routinely necessary post-transplant 2

Disease Severity Relationship

• Interestingly, patients with more severe PSC (requiring liver transplantation) tend to have milder UC with:
  • Fewer UC flare-ups 3
  • Less need for steroids and immunosuppressants 3
  • Lower rates of colonic inflammation on histology 3
  • Lower incidence of dysplasia and colon cancer 3

Pitfalls to Avoid

• Do not neglect right-sided colon during surveillance as neoplasia in PSC-UC has predilection for proximal colon 1
• Do not assume that liver transplantation eliminates the need for colorectal surveillance 2
• Do not rely solely on clinical symptoms to guide surveillance as UC in PSC patients can be clinically mild despite ongoing risk of malignancy 3
• Do not delay surveillance colonoscopy beyond 1-2 year intervals as recommended by guidelines 1