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1、Guidelines for the diagnosis and treatment of cholangiocarcinoma: an update Shahid A Khan,1Brian R Davidson,2Robert D Goldin,3Nigel Heaton,4John Karani,4 Stephen P Pereira,5William M C Rosenberg,5Paul Tait,6Simon D Taylor-Robinson,1 Andrew V Thillainayagam,1Howard C Thomas,1Harpreet Wasan7 ABSTRACT
2、The British Society of Gastroenterology guidelines on the management of cholangiocarcinoma were originally published in 2002. This is the fi rst update since then and is based on a comprehensive review of the recent literature, including data from randomised controlled trials, systematic reviews, me
3、ta-analyses, cohort, prospective and retrospective studies. BACKGROUND Development of guidelines These guidelines on the management of chol- angiocarcinoma (CC) were originally published in 2002.1 This is the fi rst update since and is based on a comprehensive review of the recent literature. Therec
4、entEuropeanHepatoPancreatoBiliary AssociationConsensusConferenceonChol- angiocarcinoma guidelines have also been used as a source.2 Specifi c recommendations have been graded based on the quality of evidence available. In the absence of signifi cant data, evidence was based on expert opinion. This m
5、anuscript has been developed with the support of The British Liver Trust and the UK cholangiocarcinoma charity, the Alan Morement Memorial Fund. Intent These guidelines are intended to bring consistency and improvement in the management from fi rst suspicion of CC through to diagnosis and subse- que
6、nt treatment. As stated in other British Society of Gastroenterology guidelines, patient prefer- ences must be sought and decisions made jointly by the patient and health professional, based on the risks and benefi ts of any intervention. A multidisciplinary team approach is recommended, and these o
7、ften complex cases should be managed in specialist centres with the relevant experience. The guidelines should not necessarily be regarded as the standard of care for all patients. Each case must be managed on the basis of individual clin- ical data. Levels of evidence Studies used as a basis for th
8、ese guidelines are graded according to the quality of evidence using the Oxford Centre for Evidence-based Medicine levels of evidence (table 1).3Grading of recom- mendations is as follows: A: consistent level 1 studies. B: consistent level 2 or 3 studies or extrapolations from level 1 studies. C: le
9、vel 4 studies or extrapolations from level 2 or 3 studies. D: level 5 evidence or inconsistent or inconclusive studies of any level. EPIDEMIOLOGY CCisthesecondcommonestprimaryliver tumour worldwide, after hepatocellular carcinoma (HCC).1 2 4 5Incidence and mortality rates for intrahepatic CC have ri
10、sen steeply and steadily across the world over the past few decades with concomitant falls in extrahepatic CC rates.4e14 Since the mid-1990s, more deaths have been coded in England and Wales due to CC than to HCC.4 5 CC kills approximately 1500 people annually in the UK, with approximately equal num
11、bers of men and women.12The cause of the rise in CC is unknown and is not explained by improvements in diagnosis.6 12e15There is debate as to whether the rise in intrahepatic CC represents a genuine increase in true parenchymal primary CC. Recent evidence from USA and UK data suggest that rising int
12、ra- hepatic CC rates partly refl ects misclassifi cation of perihilar(Klatskin)tumoursbeingincorrectly coded as intrahepatic instead of extrahepatic.12The overallincidenceandmortalityfromall CC, however, does appear to be increasing.12 Risk factors Established risk factors There are several establis
13、hed risk factors for CC, but these account for 2 diagnostic studies SR (with homogeneity*) of 2b and better studies 2bIndividual cohort study (including low-quality RCT; eg, 80%, with adequate time for alternative diagnoses to emerge (eg, 1e6 months acute, 1e5 years chronic). *Met when all patients
14、died before the treatment became available but some now survive on it; or when some patients died before the treatment became available but none now die on it. yyAn Absolute SpPin: a diagnostic fi nding whose Specifi city is so high that a Positive result rules in the diagnosis. An Absolute SnNout:
15、a diagnostic fi nding whose Sensitivity is so high that a Negative result rules out the diagnosis. zzSplit-sample validation is achieved by collecting all the information in a single tranche, then artifi cially dividing this into derivation and validation samples. xxPoor quality cohort study: one th
16、at failed to clearly defi ne comparison groups and/or failed to measure exposures and outcomes in the same (preferably blinded) objective way in both exposed and non-exposed individuals and/or failed to identify or appropriately control known confounders and/or failed to carry out a suffi ciently long and complete follow-up of patients. Poor quality case-control study: one that failed to clearly defi ne comparison groups and/or failed to measure exposures and outcomes in the same (pre
