10/12/2015

CholangiocarcinomaClinicalPresentation:History,Physical,Causes

CholangiocarcinomaClinicalPresentation
Author:PeterEDarwin,MDChiefEditor:JulesEHarris,MD,FACP,FRCPCmore...
Updated:Mar26,2015

History
Signsandsymptomsofcholangiocarcinomaincludethefollowing:
Jaundice
Claycoloredstools
Bilirubinuria(darkurine)
Pruritus
Weightloss
Abdominalpain
Jaundiceisthemostcommonmanifestationofbileductcancerand,ingeneral,is
bestdetectedindirectsunlight.Theobstructionandsubsequentcholestasistendto
occurearlyifthetumorislocatedinthecommonbileductorcommonhepaticduct.
Jaundiceoftenoccurslaterinperihilarorintrahepatictumorsandisoftenamarker
ofadvanceddisease.Theexcessofconjugatedbilirubinisassociatedwith
bilirubinuriaandacholicstools.
Pruritususuallyisprecededbyjaundice,butitchingmaybetheinitialsymptomof
cholangiocarcinoma.Pruritusmayberelatedtocirculatingbileacids.
Weightlossisavariablefinding.Itmaybepresentinonethirdofpatientsatthe
timeofdiagnosis.
Abdominalpainisrelativelycommoninadvanceddisease.Itoftenisdescribedasa
dullacheintherightupperquadrant.

Physical
Ifthecholangiocarcinomaislocateddistaltothecysticducttakeoff,thepatientmay
haveapalpablegallbladder,whichiscommonlyknownasCourvoisiersign.
Anabdominalmassorpalpablelymphadenopathyisuncommon,buthepatomegaly
maybenotedinasmanyas25%ofpatients.

Causes
Theetiologyofmostbileductcancersremainsundetermined.Currently,gallstones
arenotbelievedtoincreasetheriskofcholangiocarcinoma.Chronicviralhepatitis
andcirrhosisalsodonotappeartoberiskfactors.

Infections
InSoutheastAsia,chronicinfectionswithliverflukes(Clonorchissinensisand
Opisthorchisviverrini)havebeencausallyrelatedtocholangiocarcinoma.
Otherparasites,suchasAscarislumbricoides,havebeenimplicatedinthe
pathogenesisofcholangiocarcinoma.
ObservationshaveraisedthepossibilitythatbacterialinfectionswithHelicobacter
speciesmayplayanetiologicroleinbiliarycancer. [9]

Inflammatoryboweldisease
Astrongrelationshipexistsbetweencholangiocarcinomaandprimarysclerosing
cholangitis.Cholangiocarcinomagenerallydevelopsinpatientswithlongstanding
ulcerativecolitisandprimarysclerosingcholangitis. [10]Thelifetimeriskof
developingthiscancerinthesettingofprimarysclerosingcholangitisis1020%.At
increasedriskarepatientswithulcerativecolitiswithoutsymptomaticprimary
sclerosingcholangitisandasmallsubsetofpatientswithCrohndisease.

Chemicalexposures
Certainchemicalexposureshavebeenimplicatedinthedevelopmentofbileduct
cancers,primarilyinworkersintheaircraft,rubber,andwoodfinishingindustries.
Cholangiocarcinomahasdevelopeddecadesafteradministrationoftheradiologic
contrastmediumthoriumdioxide(ie,Thorotrast).Thisproduct,whichresultsin
lifelongalphaparticleirradiationbythoriumdecayproducts,wasinusefromthe
1930suntilthe1950s. [11]

Miscellaneousconditions
Congenitaldiseasesofthebiliarytree,includingcholedochalcystsandCaroli
disease,havebeenassociatedwithcholangiocarcinoma.
Otherconditionsrarelyassociatedwithcholangiocarcinomaincludebileduct
adenomas,biliarypapillomatosis,andalpha1antitrypsindeficiency.Obesitymay
alsobeariskfactor. [12]
DifferentialDiagnoses

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CholangiocarcinomaClinicalPresentation:History,Physical,Causes

ContributorInformationandDisclosures
Author
PeterEDarwin,MDProfessorofMedicine,DirectorofGIEndoscopy,DepartmentofMedicine,Divisionof
Gastroenterology,UniversityofMarylandSchoolofMedicine
PeterEDarwin,MDisamemberofthefollowingmedicalsocieties:AmericanSocietyforGastrointestinal
Endoscopy
Disclosure:Nothingtodisclose.
Coauthor(s)
AndrewScottKennedy,MDPhysicianinChief,RadiationOncology
AndrewScottKennedy,MDisamemberofthefollowingmedicalsocieties:AlphaOmegaAlpha,American
AssociationforCancerResearch,AmericanSocietyforRadiationOncology,RadiologicalSocietyofNorth
America,AmericasHepatoPancreatoBiliaryAssociation,AmericanSocietyofClinicalOncology
Disclosure:Nothingtodisclose.
JenniferLynnBonheur,MDAttendingPhysician,DivisionofGastroenterology,LenoxHillHospital
JenniferLynnBonheur,MDisamemberofthefollowingmedicalsocieties:AmericanGastroenterological
Association,AmericanSocietyforGastrointestinalEndoscopy,NewYorkSocietyforGastrointestinalEndoscopy,
NewYorkAcademyofSciences,SigmaXi
Disclosure:Nothingtodisclose.
SpecialtyEditorBoard
FranciscoTalavera,PharmD,PhDAdjunctAssistantProfessor,UniversityofNebraskaMedicalCenterCollege
ofPharmacyEditorinChief,MedscapeDrugReference
Disclosure:ReceivedsalaryfromMedscapeforemployment.for:Medscape.
BenjaminMovsas,MD
BenjaminMovsas,MDisamemberofthefollowingmedicalsocieties:AmericanCollegeofRadiology,American
RadiumSociety,AmericanSocietyforRadiationOncology
Disclosure:Nothingtodisclose.
ChiefEditor
JulesEHarris,MD,FACP,FRCPCClinicalProfessorofMedicine,SectionofHematology/Oncology,University
ofArizonaCollegeofMedicine,ArizonaCancerCenter
JulesEHarris,MD,FACP,FRCPCisamemberofthefollowingmedicalsocieties:AmericanAssociationforthe
AdvancementofScience,AmericanSocietyofHematology,CentralSocietyforClinicalandTranslational
Research,AmericanSocietyofClinicalOncology
Disclosure:Nothingtodisclose.

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