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Guidelines for the Diagnosis and Treatment of Cholangiocarcinoma: an Update

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Guidelines for the Diagnosis and Treatment of Cholangiocarcinoma: an Update Guidelines Guidelines for the diagnosis and treatment of cholangiocarcinoma: an update Shahid A Khan,1 Brian R Davidson,2 Robert D Goldin,3 Nigel Heaton,4 John Karani,4 Stephen P Pereira,5 William M C Rosenberg,5 Paul Tait,6 Simon D Taylor-Robinson,1 Andrew V Thillainayagam,1 Howard C Thomas,1 Harpreet Wasan7 1Department of Hepatology and ABSTRACT C: level 4 studies or extrapolations from level 2 or 3 Gastroenterology Section, The British Society of Gastroenterology guidelines on the studies. Imperial College London, D: level 5 evidence or inconsistent or inconclusive London, UK management of cholangiocarcinoma were originally 2Department of Hepatobiliary published in 2002. This is the first update since then and studies of any level. Surgery, Division of Medicine, is based on a comprehensive review of the recent University College London, literature, including data from randomised controlled EPIDEMIOLOGY London, UK trials, systematic reviews, meta-analyses, cohort, 3Department of Medicine and CC is the second commonest primary liver Department of Histopathology, prospective and retrospective studies. tumour worldwide, after hepatocellular carcinoma Imperial College London, (HCC).1 245Incidence and mortality rates for London, UK 4 intrahepatic CC have risen steeply and steadily Department of Hepatobiliary BACKGROUND across the world over the past few decades with and Pancreatic Surgery, King’s e College Hospital, London, UK Development of guidelines concomitant falls in extrahepatic CC rates.4 14 5 UCL Institute of Liver and These guidelines on the management of chol- Since the mid-1990s, more deaths have been coded Digestive Health, Division of in England and Wales due to CC than to HCC.45 Medicine, University College angiocarcinoma (CC) were originally published in 1 fi London, London, UK 2002. This is the rst update since and is based on CC kills approximately 1500 people annually in 6Department of Radiology, a comprehensive review of the recent literature. the UK, with approximately equal numbers of men Imperial College London, The recent European HepatoPancreatoBiliary and women.12 The cause of the rise in CC is London, UK Association Consensus Conference on Chol- unknown and is not explained by improvements in 7 e Department of Oncology, diagnosis.612 15 There is debate as to whether the Imperial College London angiocarcinoma guidelines have also been used as 2 Hammersmith Hospital Campus, a source. Specific recommendations have been rise in intrahepatic CC represents a genuine increase London, UK graded based on the quality of evidence available. In in true parenchymal primary CC. Recent evidence the absence of significant data, evidence was based from USA and UK data suggest that rising intra- Correspondence to on expert opinion. This manuscript has been hepatic CC rates partly reflects misclassification of Dr Shahid A Khan, Division of developed with the support of The British Liver perihilar (‘Klatskin’) tumours being incorrectly Medicine, Imperial College 12 London, Liver Unit, 10th Floor, Trust and the UK cholangiocarcinoma charity, the coded as intrahepatic instead of extrahepatic. The QEQM Wing, St Mary’s Hospital Alan Morement Memorial Fund. overall incidence and mortality from all CC, Campus, South Wharf Road, however, does appear to be increasing.12 London W2 1NY, UK and Department of Intent Gastroenterology, 3rd Floor, These guidelines are intended to bring consistency Risk factors Hammersmith House, and improvement in the management from first Established risk factors Hammersmith Hospital Campus, suspicion of CC through to diagnosis and subse- There are several established risk factors for CC, but Du Cane Road, London W12 < 15e19 0HS, UK; quent treatment. As stated in other British Society these account for 30% of all cases. Most cases [email protected] of Gastroenterology guidelines, patient prefer- of CC are sporadic. Primary sclerosing cholangitis ences must be sought and decisions made jointly (PSC), with or without ulcerative colitis, is the Revised 10 July 2012 by the patient and health professional, based on commonest known predisposing factor for CC in the Accepted 13 July 2012 the risks and benefits of any intervention. A Western world (lifetime risk 5e35%).18 In a study of Published Online First multidisciplinary team approach is recommended, 211 patients with PSC of whom 60% had inflam- 15 August 2012 and these often complex cases should be managed matory bowel disease (IBD), malignancies were the in specialist centres with the relevant experience. most frequent cause of death (44%);18 41% of The guidelines should not necessarily be regarded patients developed colorectal cancer (CRC) and 15 as the standard of care for all patients. Each case (39%) developed CC. Other malignancies included must be managed on the basis of individual clin- gall bladder cancer (GBC, n¼2), pancreatic cancer ical data. (n¼1), lymphoma (n¼3), melanoma (n¼1) and gastric cancer (n¼1). Median interval between PSC Levels of evidence diagnosis and CC was 2.5 years (range 0e9.8 years). Studies used as a basis for these guidelines are The estimated risk of CC after 10 years was 9% with graded according to the quality of evidence using no significant differences in patients with and the Oxford Centre for Evidence-based Medicine without IBD.18 In patients with IBD the 10- and 20- levels of evidence (table 1).3 Grading of recom- year risks for CRC were 14% and 31%, respectively, mendations is as follows: significantly higher than for non-IBD patients (2% A: consistent level 1 studies. and 2%). CC, cholangitis and age at entry were B: consistent level 2 or 3 studies or extrapolations independent risk factors for the combined endpoint from level 1 studies. of death or liver transplantation.18 Gut 2012;61:1657–1669. doi:10.1136/gutjnl-2011-301748 1657 Guidelines Table 1 Levels of evidence Level Therapy/prevention, aetiology/harm Prognosis Diagnosis DDX/symptom prevalence study 1a SR (with homogeneity*) of SR (with homogeneity*) of inception SR (with homogeneity*) of Level 1 SR (with homogeneity*) of randomised controlled trial (RCT) cohort studies; CDRy validated in diagnostic studies; CDRy with 1b prospective cohort studies different populations studies from different clinical centres 1b Individual RCT (with narrow CI) Individual inception cohort study Validatingz cohort study with goodx Prospective cohort study with with $ 80% follow-up; CDRy validated reference standards; or CDRy tested good follow-up{ in a single population within one clinical centre 1c All or none** All or none case-series Absolute SpPins and SnNoutsyy All or none case-series 2a SR (with homogeneity*) of SR (with homogeneity*) of either SR (with homogeneity*) of level >2 SR (with homogeneity*) of 2b cohort studies retrospective cohort studies or diagnostic studies and better studies untreated control groups in RCTs 2b Individual cohort study (including Retrospective cohort study or follow-up Exploratoryz cohort study with goodx Retrospective cohort study, or low-quality RCT; eg, <80% of untreated control patients in an RCT; reference standards; CDRy after poor follow-up follow-up) derivation of CDRy or validated on derivation, or validated only on split-samplezz only split-samplezz or databases 2c ‘Outcomes’ research; ecological ‘Outcomes’ research Ecological studies studies 3a SR (with homogeneity*) of SR (with homogeneity*) of 3b and SR (with homogeneity*) of 3b case-control studies better studies and better studies 3b Individual case-control study Non-consecutive study; or without Non-consecutive cohort study consistently applied reference or very limited population standards 4 Case series (and poor quality Case series (and poor quality prognostic Case-control study, poor or Case series or superseded cohort and case-control cohort studies{{) non-independent reference standard reference standards studiesxx) 5 Expert opinion without explicit Expert opinion without explicit critical Expert opinion without explicit Expert opinion without explicit critical appraisal or based on appraisal or based on physiology, critical appraisal or based on critical appraisal or based on physiology, bench research or bench research or ‘first principles’ physiology, bench research or physiology, bench research or ‘first principles’ ‘first principles’ ‘first principles’ *Homogeneity means a systematic review (SR) that is free of worrisome variations (heterogeneity) in the directions and degrees of results between individual studies. Not all SRs with statistically significant heterogeneity need be worrisome, and not all worrisome heterogeneity need be statistically significant. yCDR, Clinical Decision Rule (algorithms or scoring systems which lead to a prognostic estimation or a diagnostic category). zValidating studies test the quality of a specific diagnostic test based on prior evidence. An exploratory study collects information and trawls the data (eg, using a regression analysis) to find which factors are ‘significant’. xGood reference standards are independent of the test, and applied blindly or objectively to applied to all patients. Poor reference standards are haphazardly applied, but still independent of the test. Use of a non-independent reference standard (where the ‘test’ is included in the ‘reference’, or where the ‘testing’ affects the ‘reference’) implies a level 4 study. {Good follow-up in a differential diagnosis study is >80%, with adequate time for alternative diagnoses to emerge (eg, 1e6 months acute, 1e5 years chronic). **Met when all patients died before the treatment
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