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 a comprehensive surgical approach:

1. Surgical intervention:

   • Proctocolectomy is strongly recommended over segmental resection due to the high risk of metachronous lesions
   • 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
     • Patient has decompensated cirrhosis
     • Patient has recurrent bacterial cholangitis
     • Severe pruritus or jaundice persists despite therapy
     • High-grade biliary dysplasia is present 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 and UC following PSC diagnosis 1
  • Pay particular attention to the right side of the colon, as up to 76% of neoplasias in PSC-UC patients affect the right colon 1
  • Perform surveillance during disease remission to better distinguish 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

Medical therapy should focus on controlling inflammation and monitoring for complications:

1. Inflammatory control:

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

2. Medication considerations:

   • Ursodeoxycholic acid (UDCA) is not recommended for routine treatment of PSC or prevention of colorectal cancer/cholangiocarcinoma
   • Despite some studies showing decreased risk of colorectal dysplasia with UDCA, high-dose UDCA can be problematic in PSC patients 1
   • Regular 5-aminosalicylic acid (5-ASA) therapy may reduce cancer risk in UC patients 2, though this must be balanced against the specific needs of patients with PSC

Risk Factors and Monitoring

Understanding the risk profile helps guide management:

• PSC significantly increases colorectal cancer risk in UC patients, with an absolute risk up to 31% 1
• The risk of colorectal neoplasia after liver transplantation in patients with PSC and UC remains clinically important 3
• Family history of colorectal cancer increases risk fivefold 2
• The incidence of colorectal cancer post-liver transplantation is approximately 1% per person per year 3

Pitfalls and Caveats

• Do not delay surveillance colonoscopy beyond 1-2 year intervals as recommended by guidelines 1
• Do not neglect the right-sided colon during surveillance as neoplasia in PSC-UC has a predilection for the proximal colon 1
• Avoid high-dose UDCA in PSC patients despite potential benefits for colorectal cancer prevention 1
• Consider cholecystectomy in PSC patients with gallbladder polyps ≥8 mm or smaller polyps that are growing in size 1
• Patients with PSC concomitant with UC should be considered a high-risk group for both colorectal cancer and cholangiocarcinoma 4