Diseases of the Gallbladder and Biliary Tract - Digi-ED的胆囊和胆道疾病-数码

A95year-oldwomanpresentedwithupperabdominalpainandjaundice;ultrasounddemonstratedgallstones.Symptomswereshortinduration,andjaundicebegantoclearrapidly.Inviewofherageandimprovingcondition,shewastreatedconservativelyforcholecystitis.However,shethendevelopedprotractedvomiting;endoscopydemonstratedseveralstonesintheduodenum. Whatisthemostlikelycausethistypeofcomplication? a.Malignancyextendingintoduodenum b.Perforationofgallbladder c.Caroli’ssyndrome d.MetastasisfromanotherprimarytumorDiseasesoftheGallbladderandBiliaryTractDrissRaissi,MDNewYorkStateUniversityDownstateLectureSeriesNormalBiliaryPhysiologyLiverproduces500-1500mLofbile/dayMajorphysiologicroleofbiliarytractandGBistoconcentratebileandconductitinwell-timedaliquotstotheintestine.Intheintestine:bileacidsparticipateinnormalfatdigestionCholesterolandotherendogenous/exogenouscmpdsinbileexcretedinfeces.BiliaryPhysiologyComplexfluidsecretedbyhepatocytesPassesthroughhepaticbileductsintocommonhepaticductToniccontractionofsphincterofOddiduringfastingdiverts~1/2ofbilethroughthecysticductintotheGB–storedandconcentrated.CCK–releasedafterfoodingestion

GBcontracts,sphincterofOddirelaxesAllowsdeliveryoftimedbolusofbileintointestine.Bileacids–detergentmoleculesHavebothfatandwatersolublemoietiesConveyphospholipidsandcholesterolfromlivertointestine

CholesterolundergoesfecalexcretionEnterohepaticcirculationBileacidssolubilizedietaryfatandpromoteitsdigestionandabsorptionEnterohepaticcirculation:BileacidsefficientlyreabsorbedbySImucosa(terminalileum)recycledtoliverforre-excretionCholelithiasisNormalGallbladderVelvetygreenmucosaThinwallTallcolumnarcellsliningmucosalfolds(right)Submucosaandmuscularisattheleft.CholelithiasisGallstones:MCCbiliarytractdiseaseinUS(20-35%byage75)2types:Cholesterol(75%)Pigment

CalciumbilirubinateandothercalciumsaltsCholesterolStonesCholesterol:InsolubleinwaterNormallycarriedinbilesolubilizedbybileacidsandphospholipidsInmostindividuals,bilecontains>cholesterolthancanbemaintainedinstablesolution“supersaturated〞withcholesterolmicroscopiccholesterolcrystalsformInterplayofnucleation(mucus,stasis)and“anti-nucleating〞(apolipoproteinA-I)factorsdeterminewhethercholesterolgallstonesformGradualdepositionofcholesterollayersmacroscopiccholesterolstonesCholesterolStonesGallbladder:keytostoneformationAreaofbilestasisslowcrystalgrowthProvidesmucusorothermaterialtoactasanidusforinitiatingcholesterolcrystal.MexicanAmericansandseveralAmericanIndiantribes,particularlythePimaIndiansintheSouthwesthighprevalenceratesofcholesterolgallstones↓bileacidsecretionisbelievedtobethecommondenominatorintheseethnicgroupsPigmentstonesPathophysiologylesswellunderstood

productionofbilirubinconjugates(hemolyticstates)

biliaryCa2+andCO32-CirrhosisBacterialdeconjugationofbilirubintolesssolubleformPredisposingFactorsFactorsthatincreasebiliarycholesterolsaturation:EstrogensMultiparityOCP’sObesityRapidweightlossTerminalilealdisease(decreasesbileacidpool)Factorsthatincreasebilestasis:BileductstricturesParenteralhyperalimentationFastingCholedochalcystsPregnancy–(GBhypomotility)ClinicalManifestationsMostareasymptomaticDuctobstruction-underlyingcauseofallmanifestationsCysticductobstructiondistendsGBbiliarypainSuperimposedinflamm/ifx

acutecholecystitisCommonductobstructionpain,jaundice,ifx(cholangitis),pancreatitis,and/orhepaticdamage2°tobiliarycirrhosisAsymptomaticGallstones60-80%patientswithgallstonesinUSOver20-yearperiod:18%ofthesedevelopbiliarypain3%requirecholecystectomyProphylacticcholecystectomyconsideredin3high-riskgroups:1.Diabetics–10-15%greatermortality2.Calcified(porcelain)GB–Associatedw/CAofGB3.Sicklecellanemia–hepaticcrisisdifficulttodifferentiatevs.acutecholecystitisPorcelainGallbladderTreatmentofAsxGallstonesChenodeoxycholicacidorUrsodeoxycholicacidDissolutionofcholesterolstonesExpectantmanagementthencholecystectomyifsymptomaticdiseasedevelops=morecosteffectiveAlternatives:Dissovecholesterolstones:InstillMethyl-tert-butyl-etherorethylpropionateintoGBFragmentstones:extracorporealshockwavelithotripsyExtracorporealShockwaveLithotripsyChronicCholecystitisandBiliaryPainNonacutesx.causedbypresenceofgallstonesBiliaryPain(misnamedbiliarycolic)GBfromsymptomaticpatientsmaybegrosslynormalMildhistologicinflammationwithfibrosisandthickeningoftenfrompreviousattacksofacutecholecystitis.Symptoms:FromcontractionofGBduringtransientobstructionofcysticductbygallstones.SteadyacheinepigastriumorRUQ

comesonquickly

plateauoverafewminutessubsidesgraduallyover30min-severalhoursReferredpainattipofscapulaorrightshoulderN/Vcanaccompany.(nofever,leukocytosis,orpalpablemass)Attacksoccuratvariableintervals(days–years)Nonspecificsymptoms:Dyspepsia,fattyfoodintolerance,bloatingandflatulence,heartburn,belchingDiagnosisUltrasonographySensitivityandspecificity>95%Oralcholecystograpy90%sensitivity,75%specificityReservedforensuringcysticductpatencyinptswhomdissolutiontherapyorextracorporealshockwavelithotripsyisplannedTreatmentLaparoscopiccholecystectomyTreatmentofchoiceforrecurrentbiliarypainMayneedpreoperativeendoscopicorradiologicexaminationofCBDforconcomitantcholedocholithiasisOpencholecystectomyMortalityrate<0.5%Mightberequiredifdifficultiesduringprocedurei.e.adhesions,obesityNSAIDSSeveralreortsandtrialssuggestthatuseduringbiliarypainprovidesadequatepainreliefand↓progressiontoacutecholecystitisAcuteCholecystitisAcuteCholecystitisAcuterightsubcostalpainandtendernessfromobstructionofcysticduct

Distension,inflammation,and2°ifxofGBAcalculouscholecystitis(5%)

Triad-Prolongedfasting,immobility,hemodynamicinstabilityCriticallyillpatients(burns,trauma,sepsis)ParenteralhyperalimentationAcuteCholecystitisEpigastricorRUQpainGraduallyinseverityandlocalizestoGBareaUnlikebiliarypain,doesnotspontaneouslyresolveLowgradefever,anorexia,n/v,rightsubcostaltenderness(+)Murphy’ssignsubhepatictendernessandinspiratoryarrestduringdeepbreathTenderenlargedgallbladder(1/3)Mildjaundice(20%)–concomitantCBDstonesorBDedemaMurphy’sSignComplicationsOnsetoffever,shakingchills,leukocytosis,abdominalpainortenderness,orpersistentseveresymptoms=progressionofdiseaseanddevelopmentofcomplicationsEmphysematouscholecystitisDiabeticswithbacterialgaspresentinGBlumenandwallEmpyemaofgallbladderGangrenePerforationMirizzi’ssyndromeProfoundjaundiceinwhichextrinsicCBDcompressionoccursfromimpactedstoneinGBneckEmphysematous

CholecystitisDiagnosisAcuteCholecystitisRadionuclidescanningafteradministrationof99mTc-DISIDAorHIDAMostaccuratetesttoconfirmcysticductobstructionIfGBfillswithisotope

acutecholecystitisunlikelyIfbileductvisualizedbutgallbladdernotClinicaldiagnosisstronglysupportedImagestakenshortlyafterinjectionoftheradiolabeledtracer.Gallbladder(blackspot)fillsasradioactivematerialissecretedintobileandfloodsin.Imagesaftergallbladderfilled.EmptyingstimulatedbyaninjectionofCCKEnlargingblackstreakrepresentingtheCBDappearsbelowthegallbladder.Asstreakbecomesvisible,blackspotrepresentingtheGB↓insizeandalmostdisappearsasbileissqueezedintothesmallintestine.DiagnosisAcuteCholecystitisUltrasonagraphy:Gallstones(orsludgeinacalculous)alongwithlocalizedtendernessovertheGB,pericholecysticfluid,andGBwallthickening

strongsupportiveevidenceforacutecholecystitisOralcholecystograms=noclinicaluseUnreliableinacutelyillpatientManagementofAcuteCholecystitisPatientsmayimproveover1-7dayswithexpectantmanagementNGsuctionforprofoundvomiting,and/orabdominaldistensionIVfluids,ABX,andanalgesicsCholecystectomyBecauseofhighriskofrecurrentacutecholecystitisWithinfirst24-48hoursafteracuteepisodeEmergencysurgeryifadvanceddiseaseandcomplications,usuallyassociatedwithinfectionandsepsis.Cholecystostomy(operativeorpercutaneous)AlternativetocholecystectomyinpatientswithhighoperativeriskPrognosisMortalityofacutecholecystitis=5-10%Almostentirelyconfinedtopatients>60withseriousassociateddiseasesandthosewithsupparativecomplicationsComplicationsInfectionCholecystoentericfistula

resultsingallstoneileus.Choledocholithiasis

andCholangitisCholedocholithiasis&AcuteCholangitis~15%ofptswithgallstoneshaveCBDstones(choledocholithiasis)CDstonesusuallyoriginatefromGBLesscommonly

stonesformdenovointhebiliarytreeInternationalincidenceratehigherb/cprimaryCBDstonescausedbyparasitesAsiansAscarislumbricoidesandClonorchissinensis

Biliarytractlithiasismostoftenbeginswithacalculus(stone)inthegallbladder.Asmallenoughcalculus(orpartofacalculus)maybecomeimpactedintheneckofthegallbladderorcysticduct

acutecholecystitis.Thestonemaytravelfurtherdownintothecommonbileduct,andimpactioninthisduct(choledocholithiasis)mayproduceobstructionwithjaundice.Thestonemaytravelfurtherdownand,neartheampulla,obstructthepancreaticduct,leadingtoacutepancreatitis.Thestonemaypassthroughtheampullaandoutintotheduodenum.

SymptomsandSignsBiliarycolicFromrapidinCBDpressureduetoobstructedbileflowCharcot’sTriad=classiccholangitis1.RUQpain–frequentlyrecurring,severe,persistsforseveralhours2.ChillsandFever-associatedwithseverecolic3.Jaundice-associatedwithabdominalpainHepatomegaly–incalculousbiliaryobstructionTenderness–RUQandepigastriumPain=MCpresentingsymptomcolickyinnature,moderateinseverity,andlocatedintheRUQintermittent,transient,andrecurrentandmaybeassociatedwithnauseaandvomiting.Jaundice

CBDbecomesobstructedandconjugatedbilirubinentersthebloodstream.Historyofclay-coloredstoolsandtea-coloredurineisobtainedfromsuchpatientsinapproximately50%Thejaundicecanbeepisodic.Fever

IndicationofcholangitisCharcottriad:fever,jaundice,andRUQpainstronglyfavorsthediagnosis.Pancreatitis

Gallstonesareresponsiblefor50%ofallcasesConversely,4-8%ofpatientswithgallstonesdeveloppancreatitis.PancreatitiscanbeprecipitatedifCBDobstructionoccursattheleveloftheampullaofVater.PresentationofCholedocholithiasisPrimaryCBDStonesCausedbyconditionsleadingtobilestasisandchronicbactibilia.Upto90%ofpatientswithbrownpigmentCBDstoneshave(+)bilecultureresultsUsuallybrownpigmentstones.Brownstonesdifferfromblackpigmentstonesbyhavingahighercontentofcholesterol.Brownstonesaresoftandearthyinconsistencyandtaketheshapeoftheduct.InWesternpopulations,biliarystasisissecondarytofactorssuchas:sphincterofOddidysfunction,benignbiliarystrictures,sclerosingcholangitis,andcysticdilatationofthebileducts.InAsianpopulations,AlumbricoidesandCsinensispromotestasis:EitherblockingthebiliaryductsorbydamagingtheductwallsResultsinstrictureformation.Bactibiliaisalsocommonintheseinstances,probablysecondarytoepisodicportalbacteremia.SecondaryCBDStonesArisefromthegallbladder

migratetotheCBDHaveatypicalspectrumofcholesterolstonesandblackpigmentstones.Bacteriacanbeculturedfromthesurfaceofcholesterolandpigmentstonesbutnotfromthecore,suggestingthatbacteriadonotplayaroleintheirformation.LaboratoryDiagnosis

WBC

nonspecific.

Serumandurinebilirubin-indicateobstructionoftheCBDthehigherthebilirubinlevel,thegreaterthepredictivevalue.CBDstonesarepresentinapproximately60%ofpatientswithserumbilirubinlevelsgreaterthan3mg/dL.

Serumamylaseandlipase

acutepancreatitiscomplicatingcholedocholithiasis.

Alkalinephosphataseandgamma-glutamyltranspeptidase

obstructivecholedocholithiasisgoodpredictivevalueforthepresenceofCBDstones.

Prothrombintime

InprolongedCBDobstruction,secondarytodepletionofvitaminK(theabsorptionofwhichisbile-dependent).

Livertransaminases

choledocholithiasiscomplicatedbycholangitis,pancreatitis,orboth.Bloodculture

positivein30-60%ofpatientswithcholangitis.PreoperativeDiagnosisTransabdominalultrasonography

Itisusuallythefirstmodalityusedinthediagnosisofpatientswithbiliary-relatedsymptoms.Ultrasonographyfindingsareaccurateinthediagnosisofgallbladderstones,butCBDstonesaremissedfrequently(sensitivity15-40%).Ontheotherhand,CBDdilatationisidentifiedaccurately,withupto90%accuracy.EndoscopicultrasonographyIntroductionofahigh-frequency(7.5-12MHz)ultrasonicprobeadvancedintotheduodenumunderendoscopicguidance.Awater-filledballoonisusedtoprovideanacousticwindow.SensitivityandspecificityofCBDstonedetectionarereportedinrangeof85-100%.Invasive,$$$,needexperiencedenoscopist/ultrasonographerComputedtomographyscanveryaccurateinthedetectionofbiliarytreeobstructionandductaldilatationsensitivityof75-90%inthedetectionofCBDstones=essentialinevaluationofjaundice.Capableofdefiningtheleveloftheobstructionandprovidesinformationaboutthesurroundingstructures,especiallythepancreas.MRCPnoninvasivetoolwith97%accuracy,92%sensitivity,and100%specificity.$$$,inconvenience,andlimitations(eg,obesity,presenceofmetalobjects,eg,pacemakers)EndoscopicUltrasound(EUS)MRCPCholangiographyCriterionstandardforthedetectionofCBDstonesEndoscopicRetrogradeCholangiopancreatography(ERCP)TheCBDiscannulatedthroughtheampulla,contrastinjected,andfilmsareobtained.Experienceoftheendoscopistisbestpredictorofsuccess,(90-95%inexperthands)Complications=hyperamylasemiaandcholangitis.PercutaneousTranshepaticCholangiography(PTC)

maybethemodalityofchoiceinpatientsinwhomERCPisdifficult(eg,previousgastricsurgery)percutaneouslyandtranshepaticallyintoanintrahepaticduct,andcholangiographyisperformed.ComplicationsBiliaryCirrhosis:CBDobstruction>30daysliverdamagecirrhosisHypoprothrombinemia:Ptsmaybleedexcessivelyd/tPTRespondsto10mgparenteralvitaminKorwatersolubleoralvitaminKwithin24-36h.TreatmentofCholedocholithiasisCBDstoneinptwithcholelithiasisandcholecystitis:endoscopicpapillotomyandstoneextractionfollowedbylaparoscopiccholecystectomy.ERCPbeforecholecystectomyinpatientswith:Gallstonesandjaundice(serumbili>2mg/dL)DilatedCBD(>7mm)StonesinbileductseenonultrasoundorCTPrimaryBiliaryCirrhosisPrimaryBiliaryCirrhosisChronicdiseaseofliverwithautoimmunedestructionofintrahepaticbileductsandcholestasisInsidiousonsetOftendetectedbychancefindingofAlkalinePhosphataseWomenaged40-60Diseaseisprogressiveandcomplicatedby:Steatorrhea,xanthomas,xanthelasma,osteoporosis,osteomalacia,andportalhypertensionAssociatedwithSjögren’ssyndrome,scleroderma,hypothyroidism,andceliacdiseaseInfectionwithChlamydiapneumoniaemaybetriggerorcausativeagentXanthomainPBCClinicalFindingsManyasymptomaticforyearsFatigueandpruritisHepatomegalywithprogressionXanthomatouslesionsInskinandtendonsandaroundeyelidsJaundiceandsignsofportalHTN(late)RiskofosteoporosisincreasedLaboratoryfindingsinPBCSignsofcholestasisAlkPhos,cholesterol(HDL),laterbilirubinAnti-mitochondrialAntibodies(95%)DirectedagainstPDHinmitochondriaSerumIgMDiagnosisofPBCBasedoncholestaticliverchemistriesandanti-mitochondrialantibodiesinserumcombinedwithcharacteristichistologyinliverbiopsyLiverbiopsyPermitshistologicstagingStageI:PortalinflammationwithgranulomasStageII:BileductproliferationStageIII:InterlobularfibrousseptaStageIV:CirrhosisBiliaryFibrosis

Portalareawithmarkedductularproliferationandminimalinflammationinacaseofchronicbiliaryobstruction.TreatmentofPBCUrsodeoxycholicAcidPreferredmedicaltreatmentslowsprogression,improveslong-termsurvival,↓riskofesophagealvaricesSymptomaticTreatmentCholestyramineorColestipol-forpruritisCanaggravatesteatorrhaleadingtovitaminA,D,KdeficiencyRifampininconsistentlybeneficialOpiodantagonistsNaloxone,naltrexone–showpromisefortreatingpruritis5-HT3antagonistsOndansetronCalciumsupplementationHelpspreventosteomalciaColchicineandMethotrexateSomebenefitimprovingsymptomsandserumlevelsofAPLivertransplant TreatmentofchoiceforadvanceddiseasePrognosisofPBCWithouttransplant,survival=7-10yearsoncesymptomsdevelopAdverseprognosticindicators:OlderageHighserumbilirubinEdemaLowserumalbuminProlongedPTVaricealhemorrhageTheMayoriskscore:

R=0.871loge(bilirubininmg/dL)+(–2.53)loge(albumining/dL)+0.039ageinyears+2.38loge(prothrombintimeinseconds)+0.859(edemascoreof0,0.5,or1)PrimarySclerosingCholangitisPrimarySclerosingCholangitisUncommondiseasecharacterizedbydiffuseinflammationofbiliarytractleadingtofibrosisandstricturesofbiliarysystem.Mostcommoninmenage20-40andcloselyassociatedwithulcerativecolitis(presentin~2/3ofptswithPSC)Only1-4%ofpatientswithUCdevelopPSC.LikeUC,smokingisassociatedwitha↓riskofPSCAssociatedwithHLA-B8andDR3orDR4ANCA(70%),withfluorescentstainingcharacteristicsandtargetantigensdistinctfromthoseinWegener’sInAIDS,PSCmayresultfrominfectionscausedbyCMV,cryptosporidium,ormicrosporum.PSCisusuallyprogressive,leadingtocirrhosis,portalhypertension,andliverfailure.

SymptomsandSignsProgressiveobstructivejaundiceFrequentlyassociatedwithmalaise,pruritus,anorexia,andindigestion.ComplicationsofchroniccholestasisOsteoporosisMalabsorptionoffatsolublevitaminsLaboratoryDiagnosisofPSC

APorGGT–MCabnormalitySerumtransaminasescanbenormalor

serumbilirubininadvancedPSCHepaticsynthetictests(albumin,PT,etc)abnormalinadvancedPSCSerumcholylglycine(bilesalt)outofproportiontotheelevationofserumbilirubin.p-ANCAsin

60-82%ofpatientswithPSC.(FrequencyinUCissimilar.)CA19-9levelgreaterthan100U/mLhas75%sensitivityand80%specificityinidentifyingPSCpatientswithcholangiocarcinoma.ImagingDiagnosisofPSCERCPCholangiographyremainscriterionstandardCholangiographyremainsthecriterionstandardforestablishingthediagnosisofPSC.irregularlydistributed,multifocalstricturesanddilatationsoftheintrahepaticandextrahepaticbileducts=beadingMRCPNoninvasive,butlesssensitive(90%)thanERCP(97%)forvisualizingintrahepaticductsImagingofPSCTheradiographicpatternofPSCisthatofstricturesofvaryinglengthsintheintrahepaticandextrahepaticducts.Theremayinterveningareasofminimaldilatationoftheductswitharesulting"beaded"appearance.Usuallytherearemultipleareasofinvolvement.Inthiscasealmostallofthevisualizedductsareabnormalincontour.ERCPERCPimageshowsmultifocalstricturesandirregularityoftherightintrahepaticbileducts.TreatmentofPSC NoeffectivemedicaltherapiesexistCiprofloxacinEpisodesofacutebacterialcholangitisUrsodeoxycholicacid(UDCA)improvessymptomsandLFTsinadultpatientswithPSC.ERCPBalloondilationoflocalizedstrictures.Repeatedproceduresimprovessurvival.Ifmajorstricture–shorttermstentrelievessymptomsandimprovesLFTsSurgicalresectionInpatientswithoutcirrhosis,resectionofdominantbileductstricturemayimprovesurvivalvs.ERCPbecauseof↓riskcholangiocarcinoma.PrognosisofPSCAverages10yearsoncesymptomsappearAdverseprognosticmarkers:OlderageHigherserumbilirubinandASTLoweralbuminlevelsHistoryofvaricealbleedingComplications:Cholangiocarcinoma(10-15%)ofadultswithPSC.ColonCA/dysplasiaInpatientswithulcerativecolitis,PSCisindependentriskfactorStrictadherencetocolonoscopicsurveillanceprogramavisedNeoplasmsinthe

BiliaryTractCarcinomasofBiliaryTract

Manifestationsweightloss(77%)nausea(60%)anorexia(56%)abdominalpain(56%)fatigue(63%)pruritus(51%)fever(21%)malaise(19%)diarrhea(19%)constipation(16%)abdominalfullness(16%)Symptomaticpatientsusuallyhaveadvanceddisease,withspreadtohilarlymphnodesbeforeobstructivejaundiceoccurs.Itisassociatedwithapoorprognosis.CarcinomaoftheGallbladderUncommonmalignancy–2.5/100,000Mostcommonofbiliarytractcancers(54%)>90%areadenocarcinomasInNativeAmericans,GBcarcinomaisthemostcommonlyseenGImalignancyMale:Female=1:3Overallmeansurvivalrate=6months,5-yearsurvivalrateis5%Atdiagnosis,mostoftheGBisreplacedordestroyedbythecancerRiskFactorsforGBCancerCholelithiasisoftenlargeandsymptomaticstonespresentChronicinfectionofgallbladderSalmonellaTyphiGeneticFactorsGBpolyps>1cmindiameterMucosalcalcificationofGB(PorcelainGB)carcinomain25%AnomalouspancreaticobiliaryductaljunctionCongenitalbiliarycystsEnvironmentalcarcinogensAnabolicSteroidsNohabloingles!BaseballbeverygoodtomeI'mnotacrazyperson.I'mnotstupid.SymptomsandSignsJaundice

skinorictericscleraeEarly

PaininRUQwithradiationintobackAnorexia,weightloss,feverandchills(cholangitis),supraclavicularLNCourvoisier’sLawPalpableGBwithobstructivejaundicesignifiesmalignantdiseaseThisgeneralizationaccurateonly50%oftimeHepatomegalyUsuallypresentandassociatedwithlivertendernessAscitesCanoccurwithperitonealimplantsHematemesisormelenaFromerosionoftumorintobloodvessel(hemobilia)CarcinomaofGallbladderLocation:fundus(60%),body(30%),neck(10%)NotoriouslyinsidiousDiagnosismadeincidentlyatsurgerySpread

Earlylymphaticspread

retroperitoneal,rightceliac,andpancreaticoduodenalnodes.Directinvasionoftheliver,extrahepaticbiliaryducts,andduodenumandcolon(lesscommon)occurs.Intraperitonealseedingmayoccur.TNMStagingTis =CarcinomainsituT1a=GBwall:invadeslaminapropriaT1b=

GBwall:invadesmusclelayerT2=PerimuscularconnectivetissueT3=PerforatesserosaordirectlyinvadesliveroradjacentorganT4=InvadesmainportalveinorhepaticarteryormultipleorgansN1a=HepatoduodenalligamentnodesN1b=OtherregionallymphnodesM0=NodistantmetastasesM1=DistantmetastasesStage0:Tis N0 M0StageI:T1 N0 M0StageII:T2 N0 M0StageIII:T1-2 N1 M0 T3N0-1M0StageIVA:T4 N0-1 M0StageIVB:T1-4N2 M0 T1-4N0-2M1

CarcinomaofBileDucts(Cholangiocarcinoma)Tumorthatarisesfromtheintrahepaticorextrahepaticbiliaryepithelium3%ofallcancerdeathsintheUS>90%areadenocarcinomas,remainderaresquamouscellCA3GeographicLocations:IntrahepaticLeastcommonExtrahepatic(ie,perihilar)Perihilar(Klatskintumors)=MostcommonAtbifurcationofRandLhepaticductsDistalextrahepaticUpperborderofpancreasampullaTheetiologyofmostbileductcancersremainsundetermined.PossibleEtiologiesInfectionsInSEAsia,chronicinfxwithliverflukesClonorchissinensis,

OpisthorchisviverriniandFasciolaHepaticaInflammatoryboweldiseaseCCCgenerallydevelopsinpatientswithlong-standingulcerativecolitisandPSC.Chemicalexposuresprimarilyamongworkersintheaircraft,rubber,andwoodfinishingindustries.ThorotrastCongenitaldiseasesofthebiliarytree

choledochalcystsandCarolidiseasePathophysiologyofCCCLong-standinginflammationaswithPSC,chronicparasiticinfectionsuggestedtoplayarolebyinducinghyperplasia

cellularproliferation

malignanttransformation.Growslowlyandinfiltratewallsoftheducts,dissectingalongtissueplanesLocalextension:

liver,portahepatis,regionalLNoftheceliacandpancreaticoduodenalchains.SymptomsandSignsProgressivejaundice

MCmanifestationofbileductcancerTheobstructionandsubsequentcholestasistendstooccurearlyifthetumorislocatedinth