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Surveillance for cholangiocarcinoma in patients with primary sclerosing cholangitis: Can we be more proactive?
  1. Dermot Gleeson1,
  2. Martine Walmsley2,
  3. Palak J Trivedi3,
  4. Deepak Joshi4,
  5. Ben Rea5
  1. 1 Liver Unit, Sheffield Teaching Hospitals Sheffield UK, Sheffield, UK
  2. 2 PSC Support, Oxford, UK
  3. 3 National Institute for Health Research Birmingham Biomedical Research Centre, Centre for Liver and Gastroenterology Research, University of Birmingham, Birmingham, United Kingdom, Birmingham, UK
  4. 4 Institute of Liver Studies, King’s College Hospital NHS Foundation Trust, London, UK
  5. 5 Department of Clinical Radiology, Sheffield Teaching Hospitals NHS Foundation Trust, Sheffield, UK
  1. Correspondence to Professor Dermot Gleeson, Liver Unit, Sheffield Teaching Hospitals Sheffield UK, Sheffield S10 2JF, UK; Dermot.gleeson{at}

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The standardised mortality ratio of primary sclerosing cholangitis (PSC) is 3.55 (2.94–4.28)1 with most excess deaths due to malignancy. Patients with PSC often express fear of developing cancer.2 For many patients, this is the most difficult aspect of PSC.3 4

68%–80% of patients with PSC develop inflammatory bowel disease (IBD),5–8 which confers an increased risk of colorectal cancer (CRC). Thus, annual surveillance colonoscopy is recommended in UK, American and European Guidelines.9–11 This is alongside 6–12 monthly ultrasound surveillance for hepatocellular carcinoma and to detect potentially malignant gallbladder polyps.12 Some studies also report an increased incidence of pancreatic cancer,1 5 although inconsistently.12

However, most cancer-related deaths in PSC result from cholangiocarcinoma (CCA).6 7 13–16 The overall annual incidence of CCA in PSC is 1.1 (0.5–2.7)%17; 160–1600 times that of the general population.1 5 6 12 18 Up to 60% of patients with PSC with CCA die within a year of diagnosis,6 15 and despite resection, 5-year survival is ≤40% (20% with tumours >3 cm).19 Thus, CCA accounts for 24%–58% of deaths in PSC,6 7 13 16 which exceeds combined deaths attributable to CRC (2%–11%),6 13 16 gallbladder cancer7 12 13 15 and hepatocellular carcinoma.1 7 12 15 Despite these poor outcomes, guidance on surveillance for CCA is rather tentative (table 1), often with a ‘suggestion’ or ‘conditional’ recommendation for annual imaging and weak and inconsistent recommendations for biomarker monitoring.

View this table:
Table 1

Current recommendations from guidelines and expert review groups24 regarding cholangiocarcinoma surveillance in PSC

Possibly reflecting this equivocation, clinical practice varies. In a UK multicentre audit20 of 1795 patients with PSC (90% cared for by a hepatologist), 71% were reported as undergoing annual hepatobiliary imaging: most commonly by ultrasound (47%) or alternating ultrasound and …

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  • Contributors DG wrote the initial draft, to which all other authors then contributed substantially.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.