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Cholangiocarcinoma
A.ProfFrankGaillardetal.

Cholangiocarcinomaisamalignanttumourarisingfromcholangiocytesinthebiliarytree.Ittendstohavea
poorprognosisandhighmorbidity.Itisthesecondmostcommonprimaryhepatictumour,withintrahepatic
cholangiocarcinomas(ICCs)accountingfor1020%ofprimarylivertumours.

Onthispage:
Article:

Epidemiology
Clinicalpresentation
Pathology
Radiographicfeatures
Treatmentandprognosis
Differentialdiagnosis
Relatedarticles
References

Images:

Casesandfigures

Epidemiology

Althoughoverallcholangiocarcinomaisrare,therearesignificantregionalvariationsinincidencewithmuch
higherratesseeninsoutheastAsia2.

Incidenceisusuallyintheelderly(7thdecade)7.Theremaybeaslightmalepredilection.

Riskfactors

Anumberofriskfactorsforcholangiocarcinomahavebeenidentifiedincluding12,9:

primarysclerosingcholangitis(PSC)
majorriskfactorinwesterncountries
recurrentpyogeniccholangitis(hepatolithiasis)
majorriskfactorinendemicareas
choledocholithiasismorethancholelithiasis10,11
Asianliverflukes
Opisthorchisviverrini
Clonorchissinensis(clonorchiasis)
Carolidisease/choledochalcysts
lifetimeriskof1015%2
toxins
thorotrast

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dioxin
polyvinylchloride
heavyalcoholuse
viralinfection(s)
HIV
hepatitisB
hepatitisC
EBV

Clinicalpresentation

Typicallythepresentationiswithpainlessjaundice.

Pathology

Macroscopicallycholangiocarcinomashaveanumberofdifferentgrowthpatterns(seebelow),andtheir
macroscopicappearancewillreflectthis.Ingeneral,theyarescleroticmasseswithouthaemorrhageor
macroscopicnecrosis2.

Histologically,cholangiocarcinomasaredividedintowell,moderatelyandpoorlydifferentiated
adenocarcinomas2.Inspecimensofbileductsfrompatientswithhepatolithiasis,biliaryintraepithelial
neoplasia(BilIN)iscommonfindingandisconsideredtobeaprecursorlesionofcholangiocarcinoma.Itis
typicallyamicroscopiclesionwithaflatormicropapillarydysplasticepithelium.Itissynonymouswith
carcinomainsitu2.

Ingeneral,theactivetumourisattheperiphery,withthecentralportionshavingbeenreplacedbyfibrosis,
accountingforthecapsularretractionwhichmaybeseeninintrahepatictumours.

Growthpatterns/types

Cholangiocarcinomascanbeeitherintraorextrahepatic.Theyarealsoclassifiedaccordingtomacroscopic
growthpattern2:

intrahepatic
extrahepatic

Massforming

Intrahepaticexophyticnodular(peripheral)tumoursaremostcommonlyofthemassformingtype3.They
demonstratevariableamountsofcentralfibrosis,usuallymarked.

Periductalinfiltrating

Periductalinfiltratingintrahepatictumoursaremostcommonatthehilum,wheretheyareknownasKlatskin
tumours3,butcanbeseenincombinationwithmassformingtumourswithintheliver.Growthalongthewalls
oftheductmaynarrowordilatetheduct.

Intraductal

Intraductaltumoursmakeup818%ofresectedcholangiocarcinomas3andamuchsmallernumberofall
cholangiocarcinomas(asmostareinoperable).Theyarecharacterisedbyalterationsinductcalibre,usuallyduct
ectasiawithorwithoutavisiblemass.Ifamassisvisibleitmaybemuralorpolypoidinshape2.Theduct
dilatationisthoughttobeduetoabundantmucinproduction.Thisentityisthoughttobesimilartothe
pancreaticIPMN.

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Extrahepatic/largeduct

Thereismuchconfusionintheliteratureastothedefinitionofextrahepaticcholangiocarcinomas,andthereis
thussomeoverlap.

Regardingthedistributionoflargeduct(hilarandextrahepatic)tumours3:

intrahepaticlargeducts:15%
hilum/proximalthirdofCBD:50%
middlethirdCBD:17%
distalthirdCBD:18%

Thesetumoursaremostcommonlyinfiltrating,althoughbothexophytic(massforming)andpolypoid
(intraductal)typesareidentified.Theyhavesimilarappearancestotheirintrahepaticcounterparts3.

Staging

Stagingdependsonthegrowthpattern/typeofcholangiocarcinoma.

See:Cholangiocarcinomastaging

Radiographicfeatures

Ultrasound

Theappearancewillvaryaccordingtothegrowthpattern.

Massformingintrahepatic:tumourswillbeahomogeneousmassofintermediateechogenicitywitha
peripheralhypoechoichaloofcompressedliver.Theytendtobewelldelineatedbutirregularinoutlineandare
oftenassociatedwithcapsularretraction2,whichifpresentishelpfulindistinguishingcholangiocarcinomas
fromotherhepatictumours.

Periductalinfiltratingintrahepatic:tumourstypicallyareassociatedwithalteredcalibrebileduct(narrowed
ordilated)withoutawelldefinedmass.

Intraductal:tumoursarecharacterisedbyalterationsinductcalibre,usuallyductectasiawithorwithouta
visiblemass.Ifapolypoidmassisseen,itisusuallyhyperechoiccomparedtosurroundingliver2.

Contrastenhancedultrasoundmayaidwiththediagnosisofcholangiocarcinoma8:

arterialphase:
peripheralirregularrimlikeenhancement
heterogeneouscentralhypoenhancement
portalvenousphase/delayedphase:
decreasedechogenicityrelativetobackgroundliver("washout")

CT

Massformingcholangiocarcinomas:aretypicallyhomogeneouslylowinattenuationonnoncontrastscans,
anddemonstrateheterogeneousminorperipheralenhancementwithgradualenhancementcentrally23.Therate
andextentofenhancementdependsonthedegreeofcentralfibrosis2.Again,capsularretractionmaybe
evident.Thebileductsdistaltothemassaretypicallydilated.

Althoughnarrowingoftheportalveinsorlessfrequentlyhepaticveinsisseen,unlikeHCC,
cholangiocarcinomaonlyrarelyformsatumourthrombus2.

Lobarorsegmentalhepaticatrophyisusuallyassociatedwithvascularinvasion6.
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Periductalinfiltrating:intrahepatictumoursappearasregionsofthickeningoftheperiductalparenchyma
withalteredcalibreoftheinvolvedduct(narrowedordilated).Thesearemostcommonatthehepatichilum.
Thereisusuallysomedistaldilatationofthebiliarytree.

Intraductaltumours:arecharacterisedbyalterationsinductcalibre,usuallyductectasiawithorwithouta
visiblemass.Ifapolypoidmassisseenitishypoattenuatingonprecontrastimaginganddemonstrates
enhancement2.

MRI/MRCP

AppearancesonMRaresimilartothosedescribedaboveonCT,exceptthatMRismoresensitivetocontrast
enhancement3.

DWI/ADC:aperipherallyhyperintense"target"appearanceonDWIfavourscholangiocarcinomaover
hepatocellularcarcinoma

Directcholangiography

Directcholangiographyisablankettermforanyimagingobtainedwithintrabiliarytreecontrastandincludes:

PTC
ERCP
CTIVC
MRCP

Allthesemodalitiesnotonlyallowevaluationofthebiliarytreebutareinvaluableinplanningtreatmentas
assessingforresectability.

Treatmentandprognosis

Themostimportantfactorinprognosisiswhetherornotthetumourcanberesected.Evenwithresection,the
prognosisispoorwithfiveyearsurvivalofonly1044%4.

Thepatternofmetastaticspreadincludes1:

intrahepaticvascularinvolvementwithnumerouslocalmetastases.
regionallymphnodes(50%atautopsy)
haematogenous(50%atautopsy)
lungs
bones,especiallyvertebrae
adrenals
brain

Differentialdiagnosis

Differentialdiagnosisisdifferentaccordingtowhetherthetumourisintraorextrahepaticanddependingonthe
growthpattern.

Foranintrahepaticmassformingcholangiocarcinomaconsider:

livermetastases
centralnecrosis(highT2signal)ismorecommon
hepatocellularcarcinoma(HCC)
tumourthrombusmorecommon
capsularretractionuncommon
mayappearverysimilar
otherprimarylivertumours
hepaticabscess
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Foraperiductalinfiltratingcholangiocarcinomaconsider:

benignstricture
usuallyshortsegment
regularmargin
symmetricnarrowing
noductalenhancement
nolymphnodeenlargement
noperiductalsofttissuemass
periportallymphangiticmetastasis2

Foranintraductalcholangiocarcinomaconsider:

intraductalinvasionbyanHCC
extraductalmass
hepatolithiasis
noenhancement
higherattenuation
biliarycystadenomaorcystadenocarcinoma
intratumouralcystsdonotcommunicatewiththebiliarytree
benignstricture

Quizquestions
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