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

Proctocolectomy with ileal pouch-anal anastomosis (IPAA) is the preferred surgical approach for patients with colorectal cancer in the setting of ulcerative colitis and primary sclerosing cholangitis. 1

Surgical Management

The management of patients with this complex triad requires aggressive surgical intervention:

1. Surgical approach:

   • Proctocolectomy is superior to segmental colectomy in this high-risk population
   • IPAA is preferred over permanent ileostomy when technically feasible due to:
     • Better functional outcomes
     • Lower risk of variceal formation 1

2. Timing considerations:

   • Liver transplantation may be necessary before or simultaneously with colorectal surgery if:
     • Liver function is severely compromised
     • Patient has recurrent peristomal variceal bleeding
     • Decompensated cirrhosis is present
     • Patient has recurrent bacterial cholangitis
     • Severe pruritus or jaundice persists despite endoscopic and pharmacological therapy 1

Surveillance Recommendations

Annual surveillance is critical for these high-risk patients:

• Colonoscopy:

  • Annual surveillance colonoscopy should be performed in all patients with concurrent PSC following PSC diagnosis, regardless of disease activity, extent, and duration 1
  • Pay special attention to the right side of the colon, as up to 76% of neoplasia in PSC-UC patients affects the right colon 1
  • Perform surveillance during disease remission to better discriminate between dysplasia and inflammation
  • Use chromoendoscopy with targeted biopsies as it increases dysplasia detection rate 1

• Cholangiocarcinoma surveillance:

  • Regular monitoring is essential as PSC patients have a 7-9% 10-year cumulative incidence of cholangiocarcinoma
  • Colorectal cancer/dysplasia is a risk factor for cholangiocarcinoma development 1
  • Diagnostic workup should include contrast-enhanced cross-sectional imaging and ERCP with ductal sampling when indicated 1

Medical Management

• Inflammation control:

  • Aim for mucosal healing to reduce inflammation and potentially lower cancer risk 1
  • Treat according to standard inflammatory bowel disease guidelines, noting that UC associated with PSC often has more extensive colitis, higher frequency of rectal sparing, and "backwash ileitis" 1

• Chemoprevention:

  • Regular 5-aminosalicylic acid (5-ASA) therapy may reduce cancer risk by up to 75% in UC patients 2
  • However, ursodeoxycholic acid (UDCA) is not recommended for routine treatment of PSC or prevention of colorectal cancer despite some studies showing decreased risk of colorectal dysplasia, as high-dose UDCA can be problematic in PSC patients 1

Post-Treatment Monitoring

• Continue annual surveillance colonoscopy after treatment 1
• Monitor for cholangiocarcinoma development, especially in patients with colorectal cancer/dysplasia 1
• For patients who undergo liver transplantation, continue colonoscopic surveillance as the risk of colorectal neoplasia remains clinically important post-transplantation 3

Important Considerations and Pitfalls

1. Don't delay surveillance:

   • Delaying surveillance colonoscopy beyond 1-2 year intervals significantly increases risk 1
   • The incidence of colorectal cancer post-liver transplantation is approximately 1% per person per year 3

2. Don't neglect the right colon:

   • Right-sided predominance of colorectal neoplasia is common in UC patients with PSC 1

3. Family history matters:

   • A family history of sporadic CRC in any relative increases risk fivefold in UC patients 2

4. Gallbladder surveillance:

   • Recommend cholecystectomy in PSC patients with gallbladder polyps ≥8 mm or smaller polyps that are growing in size 1

The management of this complex condition requires a multidisciplinary approach involving gastroenterologists, hepatologists, colorectal surgeons, and transplant specialists to optimize outcomes and reduce mortality.