Incidence and Risk Factors for Cholangiocarcinoma in Primary Sclerosing Cholangitis

Burak, Kelly W.; Angulo, Paul; Pasha, Tousif M.; Egan, Kathleen M.; Petz, Janice L.; Lindor, Keith D.

doi:10.1111/j.1572-0241.2004.04067.x

Cited by 506 publications

(340 citation statements)

References 24 publications

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“…Patients with PSC are at increased risk for hepatobiliary cancers, especially CCA,31 with recent studies reporting a 10‐year cumulative incidence of 7%‐9% 32, 33, 34. As CCA may present morphologically and clinically as a DS, such lesions must be thoroughly investigated to rule out underlying malignancy.…”

Section: Discussionmentioning

confidence: 99%

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Hilscher

Tabibian

Carey

et al. 2018

Hepatology Communications

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Dominant strictures (DSs) of the biliary tree occur in approximately 50% of patients with primary sclerosing cholangitis (PSC) and may cause significant morbidity. Nevertheless, the definition and management of DSs lacks consensus. We aimed to better understand current perceptions and practices regarding PSC‐associated DSs. We conducted an anonymous, 23‐question, survey‐based study wherein electronic surveys were distributed to 131 faculty in the Division of Gastroenterology and Hepatology at the three Mayo Clinic campuses (Rochester, Scottsdale, and Jacksonville) as well as the affiliated practice network. Responses were aggregated and compared, where applicable, to practice guidelines of the American Association for the Study of Liver Diseases and European Association for the Study of the Liver. A total of 54 faculty (41.2%) completed the survey, of whom 24 (44.4%) were hepatologists, 21 (38.9%) gastroenterologists, and 9 (16.7%) advanced endoscopists. One of the major study findings was that there was heterogeneity among participants' definition, evaluation, management, and follow‐up of DSs in PSC. The majority of participant responses were in accordance with societal practice guidelines, although considerable variation was noted. Conclusion: Despite the prevalence and morbidity of DSs in PSC, clinical perceptions and practices vary widely among hepatologists, gastroenterologists, and advanced endoscopists who manage these patients, even within a single health care system. Further studies are needed to address these variations, develop general and evidence‐based consensus, and increase adherence to societal guidelines. (Hepatology Communications 2018;2:836‐844)

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Section: Discussionmentioning

confidence: 99%

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Hilscher

Tabibian

Carey

et al. 2018

Hepatology Communications

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“…Infection with liver flukes (Opisthorchis viverrini and Clonorchis sinensis) is a common risk factor in East Asia where iCCA represents a large proportion (~85%) of primitive liver cancers [23][24][25] . The association between CCA (mainly pCCA) and PSC is well established especially in Europe, but PSC is a rare disease 23 . More rele vant from epidemiological and clinical points of view is the association with HBV-related and HCVrelated liver diseases that have been identified as definitive risk factors, with a stronger association for iCCA than pCCA 26 .…”

Section: Risk Factorsmentioning

confidence: 99%

Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA)

Bañales

Cardinale

Carpino

et al. 2016

Nat Rev Gastroenterol Hepatol

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1,139

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“…4,5 However, quality-of-life issues (often related to pruritus) and an increased risk of cholangiocarcinoma have often been noted as reasons for seeking MELD exceptions. 6,7 It must be noted that the consensus recommendation regarding MELD exception points for PSC patients with recurrent bacterial cholangitis or septic complications is not evidence-based. Therefore, in PSC patients, the question remains: what role does sepsis play with respect to wait-list mortality and posttransplant survival?…”

Section: See Article On Page 250mentioning

confidence: 99%

Moving toward evidence-based liver allocation in patients with primary sclerosing cholangitis

Wiesner

2013

Liver Transpl

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The prevailing perception promoted by patient advocate groups is that patients with cholestatic liver disease, including patients with primary sclerosing cholangitis (PSC), are disadvantaged by the Model for End-Stage Liver Disease (MELD) allocation scheme. Indeed, recent studies have shown that PSC patients more frequently received MELD exception points than patients with all other liver disease etiologies. 1 Furthermore, 75% of these PSC patients applying for MELD exception points did not meet the criteria recommended at the 2006 consensus meeting on MELD exceptions ( 2 episodes of bacteremia or 1 septic complication). 2,3 Nevertheless, 80% of PSC patients not meeting MELD exception criteria were approved by the regional review board, and they were more likely to undergo transplantation than those PSC patients meeting the criteria. 1 Furthermore, PSC patients were found to undergo living donor transplantation at a higher rate than patients with other etiologies. 4 In reality, in the MELD era, PSC patients have the best waiting-list survival and have one of the best survival rates after transplantation. 4,5 However, quality-of-life issues (often related to pruritus) and an increased risk of cholangiocarcinoma have often been noted as reasons for seeking MELD exceptions. 6,7 It must be noted that the consensus recommendation regarding MELD exception points for PSC patients with recurrent bacterial cholangitis or septic complications is not evidence-based. Therefore, in PSC patients, the question remains: what role does sepsis play with respect to wait-list mortality and posttransplant survival?This question of wait-list mortality risk in PSC patients with bacterial cholangitis is addressed in this issue of Liver Transplantation by Goldberg et al. 8 The authors found that transplant candidates on the wait list with PSC who had episodes of bacterial cholangitis did not have an increased risk of wait-list mortality. They also found that none of 30 patients who were removed from the wait list for death or clinical deterioration had known or reported bacterial cholangitis as the reason for delisting. In addition, the MELD scores at transplantation and at wait-list removal were not different for PSC patients with bacterial cholangitis and PSC patients without bacterial cholangitis; this suggests that bacterial cholangitis alone does not result in a significantly increased MELD score that would change wait-list outcomes.There were several limitations to the study: it was retrospective, was performed at 2 centers (both in a region providing transplantation for patients with high MELD scores), and was based on relatively few patients. Furthermore, the definition of cholangitis was based on physician documentation rather than blood cultures or cholangiographic data. In this study, there was no single clinical finding that was considered confirmatory for bacterial cholangitis, and the diagnosis was based on an individual physician's clinical judgment. In addition, it is unknown how many of the PSC patients were on prophyla...

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Incidence and Risk Factors for Cholangiocarcinoma in Primary Sclerosing Cholangitis

Cited by 506 publications

References 24 publications

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Dominant strictures in primary sclerosing cholangitis: A multicenter survey of clinical definitions and practices

Cholangiocarcinoma: current knowledge and future perspectives consensus statement from the European Network for the Study of Cholangiocarcinoma (ENS-CCA)

Moving toward evidence-based liver allocation in patients with primary sclerosing cholangitis

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