Documenti di Didattica
Documenti di Professioni
Documenti di Cultura
Juvenilenasopharyngealangiofibromastaging:Anoverview
COPYRIGHTEDMATERIAL,DONOTREPRODUCE
June4,2015
Juvenilenasopharyngealangiofibromastaging:An
overview
ByNadaAliAlshaikh,MDAnnaEleftheriadou,MD,PhD
Introduction
Juvenilenasopharyngealangiofibroma(JNA)isararebenignnasopharyngealtumorthataccountsfor0.05to0.5%ofall
headandnecktumors.1Itexclusivelyaffectsadolescentboys.1Itwasfirstdescribedin1906byChauveau,whogaveit
itsname.2However,theoldestrecordedsurgicalprocedureforJNAisattributedtoHippocrates,whoperformeda
longitudinalsplittingofthenasalridgetoremoveaJNA.3
Histologically,JNAsareunencapsulatedtumorsconsistingofabundantvascularchannelsthatlackthenormalmuscular
layerinthechannelwall,whichexplainstheirtendencytowardspontaneousbleeding.Thevascularchannelsare
surroundedbyanetworkoffibrocollagenoustissue.4
Althoughtheyarehistologicallybenign,JNAsarehighlyvascular,locallyaggressive,anddestructiveinnature.They
tendtoexpandintothenose,paranasalsinuses,vidiancanal,andpterygopalatinefossa.5Fromthere,theycaninvade
theinfratemporalfossathroughthepterygomaxillaryfissure,ortheycanextendintotheorbitthroughtheinferiororbital
fissureorintothemiddlecranialfossa,eitherdirectlyorthroughtheforamenlacerum,foramenrotundum,foramenovale
(viaextensionbeyondthesphenoidsinusandvidiancanal),orsuperiororbitalfissure.Erosionthroughtheposteriorwall
ofthesphenoidsinuscanresultinintracranialextension.6
Etiology.TheetiologyofJNAisstillunclear.79Osbornin1959proposedthatthetumorcouldbeeitherahamartomaor
agrowthofresidualfetalerectiletissuesunderhormonalstimulation.9GirgisandFahmyconsideredthetumorasa
paragangliomabasedonthehistologicappearanceofundifferentiatedepithelioidcellsatthemarginsoftheJNA.10
SchicketalsuggestedthatJNArepresentsthegrowthofaresidualvascularplexusfromtheinvolutionofthefirst
branchialartery.11Thisarterycommunicateswiththeinternalcarotidarteryandthemaxillaryarterytemporarilyduring
fetallife,anditspersistencemayleadtothedevelopmentofJNAsecondarytogrowthstimulationatthetimeof
adolescence.Thiscouldexplaintheoccasionalvascularcontributionfromtheinternalcarotidartery.Anothertheoryis
thatJNAdevelopsfromasteroidstimulatedhamartomatousturbinatetissue,whichwouldexplainthenaturalinvolution
ofJNAafterpuberty.10
Todate,themostacceptedtheoryofJNAgenesisisthatitarisesasaresultofrepeatedepisodesofmicrohemorrhages
andrepairbyfibroustissueformationintheareaofthesphenopalatineforamen,whichisrichinvascularerectiletissues
thatdilateinresponsetotheincreaseinsexualhormoneproductionduringadolescence.12Thisprocessisbelievedto
leadtotheformationofJNA,especiallyifthecapillariesweremalformed.
TheexactsiteoforiginofJNAisalsostillunknown.However,thereisaconsensusthatitoriginatesinthesuperior
marginofthesphenopalatineforamenatthepointwherethepterygoidprocessofthesphenoidbonemeetsthe
sphenoidprocessofthepalatineboneandthehorizontalalaofthevomer.13
Diagnosis.PatientswithJNAusuallypresentwithunilateralnasalobstruction,recurrentepistaxis,anda
nasopharyngealmass.Asthediseaseadvances,patientsmaypresentwithfacialswelling,cranialneuropathy,and
proptosis.1Biopsyiscontraindicatedbecauseoftheriskofintractablebleeding.Thediagnosisismadebyconsidering
elementsoftheclinicalpresentationwithradiologicfindingsoncontrastenhancedcomputedtomography(CT),magnetic
resonanceimaging(MRI),and/orangiography.
Treatment.ThereisgeneralagreementthatsurgeryisthebestmodalityoftreatmentavailableforJNA.14Basedonthe
factthatJNAisahighlyvasculartumor,preoperativeangiographyandembolizationofthefeedingvesselsfromthe
externalcarotidarteryarehighlyrecommendedbymanysurgeonsasameansofreducingtheriskofextensive
intraoperativebleeding.Differentsurgicalapproacheshavebeendescribedintheliteraturetheyincludetranspalatal,
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
1/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
lateralrhinotomy,transantral,midfacedegloving,infratemporalfossa,craniotomy,transnasalmicroscopic,and
transnasalendoscopicapproaches.13OtherdescribedtechniquesforresectionofJNAsincludetheuseoftheKTPlaser
andultrasonicallyactivatedscalpel.15
Othertreatmentmodalitieshavebeenused,mostlyforextensivediseasewithintracranialinvasion,forresidualor
recurrenttumorsatsurgicallyinaccessibleanatomicsites,andwhenahighriskofmajorcomplicationsisanticipated.
Thesealternativesincluderadiotherapy,hormonaltherapy,andembolization.Morerecently,gammakniferadiosurgery
hasbeenusedforthetreatmentofresidualJNAafteraconservativesubtotalresection.16
EventhoughtransnasalendoscopicresectionofJNAshasbeenusedformorethanadecade,clearindicationsforits
useindifferentstagesofJNAhaveyettobeestablished.Mostsurgeonsconsidertheextentandgrowthpatternofthe
tumortodeterminethefeasibilityofendoscopicremoval.Theendoscopicapproachcarriestheadvantagesofminimal
softtissuedissection,avoidanceoffacialincisionsandfacialbonydisruption,theavailabilityofamagnifiedmultiangled
view,andminimalmorbiditywithashorthospitalizationtime.
Nowadays,thereisaconsensusamongmostsurgeonsthattumorsconfinedtothenasopharynx,pterygopalatinefossa,
andparanasalsinusescanbesuccessfullyremovedviaatransnasalendoscopicapproach.17However,thefeasibility
andefficiencyoftheendoscopicmanagementofJNAsthatinvadetheinfratemporalfossaand/ortheskullbaseisstill
problematic.18,19
Stagingsystems.Stagingsystemsforanytumorareimportantbecausetheyusuallystandardizetheguidelinesfor
classificationandmanagementbasedoncumulativefactorsthatinfluencethesurgicaldecision,aswellastheprognosis
aftersurgery,includingtheriskofresidualandrecurrentdisease.Stagingsystemsalsocanservetoeliminateany
confusionthatmightbeengenderedbydifferentreportsintheliterature,andtheyallowforbetterinterinstitutionaldata
comparisons.
ManysystemshavebeenproposedtoclassifythestagesofJNA.FactorsthatinfluencesurgicaldecisionmakinginJNA
includetheextentandsizeofatumor,thetechnicaldifficultiesencounteredinsurgery,andthecommonsitesofresidual
andrecurrentdisease.20Inthisarticle,wereviewtheclassificationsystemsforJNA,andwediscusstheirimpacton
evaluation,management,andprognosis,aswellassomeoftheadvantagesanddisadvantagesofthedifferentsystems.
Literaturereview
WeconductedastructuredreviewofthePubMed,Embase,andCochraneCollaborationdatabases(theCochrane
CentralRegisterofControlledTrialsandtheCochraneDatabaseofSystematicReviews)usingthefollowingMeSH
terms:juvenilenasopharyngealangiofibroma,nasopharyngealtumor,nasopharyngealdisease,juvenileangiofibroma,
angiofibromastaging,tumorclassification,andtumorstaging.Wefoundnorandomizedcontrolledtrialsorsystematic
reviewsintheentireEnglishlanguageliteratureregardingtheuseofdifferentstagingsystemsforJNAandtheir
influenceinmanagementdecisionsandpredictionsofresidualandrecurrentdisease.Therefore,weconductedourown
reviewofpublishedstagingsystems.Thedetailsofsomeofthesesystemsareshownintable1.Asummaryofreportson
thetreatmentofJNAisshownintable2.
Table1.SelectedclassificationsystemsforJNA
StageI
StageII
StageIII
StageIV
StageV
Sessions,21
1981
A.Minimalextension
intothepterygo
A.Involvement palatinefossa
ofthenoseor
nasopharyngeal B.Fulloccupationof
thepterygopalatine
vault
fossa
B.Extension
intooneormore C.Infratemporal
extensionw/orw/o
sinuses
involvementofthe
cheek
Intracranialextension
Chandler,22
1984
Involvementof
Extensionintothe
the
nasalcavityor
nasopharyngeal
sphenoidsinus
vault
Extensionintothe
maxillarysinuses,
ethmoidsinuses,
Intracranial
pterygopalatinefossa,
extension
infratemporalfossa,orbit,
orcheek
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
2/11
7/24/2015
Antonelli,23
1987
Juvenilenasopharyngealangiofibromastaging:Anoverview
Extensionintooneor
Confinementto
Extensionintothe
moreofthefollowing:
thenasalfossa
Intracranial
sphenoidsinusand/or maxillarysinus,ethmoid
and/or
extension
pterygomaxillaryfossa sinus,orbit,infratemporal
nasopharynx
fossa,cheek,andpalate
Invasionofthe
Confinementto
pterygopalatinefossa
Andrews
thenoseor
ormaxillary,ethmoid,
Fisch,241989 nasopharyngeal
orsphenoidsinus
vault
w/bonedestruction
Bagatella
Mazzoni,26
1995
StageIfeaturesplus
extensiontooneor
moreofthefollowing
Confinementto
w/bonedestruction:
the
thecontralateralnaso
nasopharynx
pharynxandnasal
andipsilateral
fossa,sinuses,
nasalfossa
parapharyngeal
w/nobone
space,
destruction
pterygomaxillary
fossa,andinfraspheno
temporalfossa
A.Extensionintothe
infratemporalfossaor
orbit
B.Intracranialextension
intotheextradural
parasellararea
StageIIfeaturesplus
extensionintothe
temporalandzygomatic
fossaeorintraorbitalor
intracranialextradural
extention
A.Minimalextension
intothe
A.Minimalskullbase
A.Involvement pterygopalatinefossa involvement,including
ofthenoseor
themiddlecranialfossa
nasopharyngeal B.Fulloccupationof and/orbaseofthe
thepterygopalatine
pterygoidplates
Radkowski,27 vault
fossa
1996
B.Extension
B.Extensiveintracranial
intooneormore C.Infratemporalfossa involvementw/orw/o
extensionorextension invasionintothe
sinuses
posteriortothe
cavernoussinus
pterygoidplates
Onerci,28
2006
Deepextensionintothe
cancellousboneatthe
Extensionintothe
baseofthepterygoid
Extensioninto
maxillarysinusorthe musclesorthebodyand
thenose,
anteriorcranialfossa, greaterwingofthe
nasopharyngeal
fulloccupationofthe sphenoidbone,signifcant
vault,and
pterygomaxillary
extensiontothe
ethmoidand
fossa,andlimited
infratemporalfossaor
sphenoid
extensiontothe
pterygoidplates
sinuses
infratemporalfossa
posteriorlyortheorbital
region,andobliterationof
thecavernoussinus
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
A.Intracranial
extension
intradural
extensionw/o
infiltrationofthe
cavernoussinus,
pituitaryfossa,or
opticchiasma
B.Intracranial
intradural
extension
w/infiltrationofthe
cavernoussinus,
pituitaryfossa,or
opticchiasma
Intracranial
intradural
Intracranial
extensionbetween
thepituitarygland
andinternal
carotidartery,
tumorextension
posterolateralto
theinternalcarotid
artery,middle
fossaextension
andextensive
intracranial
extension
3/11
7/24/2015
INCan,31
2008
Juvenilenasopharyngealangiofibromastaging:Anoverview
A.Involvementofthe
nasopharynx,nasal
fossa,maxillary
antrum,anterior
ethmoidcells,and
sphenoidsinus
Involvementof
invasionintothe
the
pterygomaxillary
nasopharynx,
fossaornfratemporal
nasalfossae,
fossaanteriortothe
maxillary
pterygoidplates,with
antrum,anterior
itsgreatestdiameter
ethmoidcells,
andsphenoid <6cm
sinus
B.Invasionintothe
Invasionintothe
infratemporalfossa
posteriortothepterygoid
platesorintothe
posteriorethmoidcells
Extensiveskull
baseinvasion>2
cmorintracranial
invasion
pterygomaxillaryfossa
orinfratemporalfossa
anteriortothe
pterygoidplates,with
itsgreatestdiameter
6cm
UPMC,32
2010
V(M):Medial
intracranial
extension
Skullbaseerosion w/residual
w/involvementof vascularityfrom
Involvementof Involvementofthe
theinternal
Skullbaseerosionw/
theorbitand
thenasalcavity paranasalsinusesand
involvementoftheorbit infratemporalfossa carotidartery
and
lateralpterygopalatine
V(L):Lateral
andinfratemporalfossa w/residual
pterygopalatine fossaw/noresidual
intracranial
w/noresidualvascularity vascularityfrom
fossa
vascularity
theinternalcarotid extension
w/residual
artery
vascularityfrom
theinternal
carotidartery
Table2.SummaryofreportsonthetreatmentofJNA
Author
Staging
N
system
Residual
or
recurrent
tumor,%
Snyderman
etal,32
35 UPMC
2010
All/NA
30
27endoscopic,6combinedendoscopicopenapproach,2
23
openapproach
Andrews
Nicolaiet
46 Fisch,
al,292010
Onerci
All/all
40
Allendoscopic
Midilliet
al,42009
42 Radkowski All/none 25
Khalifaand
Andrews
Ragab,19 32
Fisch
2008
Guptaet
All/all
16
28 Radkowski All/none 21
8.7
12endoscopic,10lateralrhinotomy,7midfacedegloving,
17
6transpalatalapproach,6craniotomy,1midfacesplitting
16endoscopIcallyassistedantralwindowapproach,16
endoscopIcallyassistedmidfacedegloving
NA
Allendoscopic
3.6
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
4/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
al,332008
19lateralrhinotomy,4transpalatalapproach,4midface
degloving,4anterolateralosteoplasticmaxillotomy,3
medialosteoplasticmaxillotomy,2endoscopic,2
craniofacialresection,2notmentioned,14radiotherapy
Yiotakiset
20 Radkowski All/some 12
al,172008
9endoscopic,6transpalatalapproach,5midface
degloving
90
Danesiet
Andrews
85
al,342008
Fisch
All/33
44midfacedegloving,41lateralrhinotomy
Andradeet
Andrews
12
al,52007
Fisch
All/none None
Allendoscopic
Allendoscopic
17
Borgheiet
23 Radkowski All/some None
al,352006
Allendoscopic
61
El
Banhawyet 20 Fisch
al,362006
All/some None
Midfacedegloving
Tyagiet
Andrews
95
al,302006
Fisch
All/some 25
65combinedtranspalatalandtransantralapproach,15
transpalatalapproach,10infratemporalfossaapproach,5 2
craniotomy
Onerciet
36 Onerci
al,282006
NA
NA
24openapproach,12endoscopic
33
Tosunet
24 Radkowski All/5
al,372006
All
10transpalatalapproach,9endoscopic,4lateral
rhinotomy,1midfacedegloving
17
Cansizet
22 Fisch
al,382006
All/9
13
16midfacedegloving,4endoscopic,2combinedmidface
59
deglovingandinfratemporalfossaapproach
Hofmannet
21 Fisch
al,392005
NA
19
Allendoscopic
9.5
Kaniaet
20 Radkowski All/some All
al,402005
8endoscopic,5midfacedegloving,3endoscopically
assistedmidfacedegloving,2lateralrhinotomy,2
endoscopicallyassistedcraniotomy
15
Andrews
Pasquiniet
6 Fisch,
All/all
al,412004
Radkowski
Allendoscopic
16.7
AllendoscopIcallyassistedmidfacedegloving
Carrilloet
54 INCan
al,312008
All/none 8
Eloyet
6 Radkowski All/all
al,202007
El
Banhawyet 15 Fisch
al,422004
35
All
All
All/some 2
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
5/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
Mannet
30 Fisch
al,132004
Onerciet
al,72003
All/NA
All
15transnasalapproach,8midfacedegloving,6lateral
rhinotomy,1transpalatalapproach
NA
All
8endoscopic,4endoscopicallyassistedmidface
degloving
NA
Wormald
andVan
7 Radkowski All/NA
Hasselt,43
2003
All
Allendoscopic
29
Senneset
33 Sessions
al,142003
All/NA
NA
NA
NA
Nicolaiet
Andrews
15
al,442003
Fisch
NA/all
All
Allendoscopic
13
Mairet
5 Fisch
al,452003
NA/all
All
AllendoscopicwiththeNd:YAGlaser
NA
Rogeret
20 Radkowski All/some 19
al,462002
Allendoscopic
40
Petrusonet
32 Chandler
al,472002
16lateralrhinotomy,13transantralapproach,2
radiotherapy,1embolization
NA
Allendoscopic
12 Radkowski NA
22/some 17
Jorissenet
13 Radkowski NA
al,482000
All
NA=
information
not
available.
Sessionssystem.Introducedin1981,theSessionsclassificationsystemisregardedasthefirststandardizedJNA
stagingsystem(table1).21Sessionsandcolleaguesproposedthisradiologicstagingsystemwiththegoalofeliminating
confusionamongdifferentinstitutionswithregardtosurgicalapproaches,morbidity,andcurerates.TheyusedCTscans
todefinetheanatomiclocationofthedisease,andtheyusednasopharyngealcarcinomaasastagingmodel.They
believedthatitistumorextensionratherthantumorsizethatdeterminesthestageandthesurgicalapproachtotumor
clearance.However,Sessionsetalfailedtoincorporatesurgicalchallenges,treatmentoutcomes,andsitesofresidualor
recurrentdiseaseintotheirclassificationsystem.Furthermore,atthattime,intracranialextensionwasageneralterm
thatdidnotspecifywhetherthedurahadbeenpenetrated.Inouropinion,thisisofgreatimportanceinsurgicalplanning
andprognosis,butthemajorpitfalloftheSessionssystemisthatitisbasedonthestagingofnasopharyngeal
carcinoma.Basingthestagingsystemofabenignprocessonthatofamalignantprocessislikelytoresultingenerally
inaccuratefindings,sincethepathophysiology,nature,andbehaviorofthesetwoprocessesiscompletelydifferent.
Chandlersystem.ThesystemdevisedbyChandleretalin1984wasbasedontheclinicalevaluationoftumor
extensionandsizecombinedwithradiologicfindingsonCTand/orMRI(table1).22Theirsystemintegratedknown
tumorextensionandrationalplanningwiththetherapeuticapproachesavailableatthattime.
Thissystemhasbeenadoptedbysomesurgeonsandfoundtobeusefulinmakingdecisionsaboutthesurgical
approachandmanagementofdifferentJNApatients.However,Chandleretalstagednasalcavityinvolvementhigher
thannasopharyngealinvolvement,suggestingthattheformerrequiresdifferentandmoreextensivesurgery,whichisnot
thecase.Inaddition,theyfailedtoconsiderthecomplexityofintracranialextension,andthustheirsystemachievedless
popularitythandidsomeoftheothersystems.Ultimately,theirsystemisunreliablebecauseitwasbasedonthe
AmericanJointCommitteeonCancersystemasnoted,angiofibromaisabenignlesionthatfollowsadifferentcourse
frommalignantlesions.
Antonellisystem.AninterestingclassificationsystemwasintroducedbyAntonellietalin1987(table1).23Theybased
theirsystemontheirexperienceinmanaging19casesofJNA.Theirsystemwasbasedentirelyonclinicoradiologicdata
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
6/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
ontumorsizeandextension.However,thissystemdidnotgainpopularity,probablybecauseitdidnotcorrelatewellwith
surgicalapproachesandoutcomes.Forexample,stageIIIinthissystemincludedmaxillaryandethmoidsinusextension
alongwithinfratemporalfossainvolvement,eventhoughinfratemporalinvolvementrequiresmoreextensivesurgery
thandoesextensionintothemaxillaryorethmoidsinuses.
AndrewsFischsystem.Basedonhiswideexperienceinskullbasesurgery,FischintroducedastagingsystemforJNA,
whichhe,Andrews,andothersmodifiedin1989themodificationiscalledtheAndrewsFischsystem(table1).24This
systemhasbecomeoneofthemostpopularinusetoday.Infact,mostsurgeonsworldwideconsiderittobethe
standard.
Intheirreport,Andrews,Fisch,andcolleaguesdescribedindetailthegrowthpatternofJNAasitoriginatesinthe
superiormarginofthesphenopalatineforamenatthejunctionofthesphenoidprocessofthepalatineboneandthe
pterygoidprocessofthesphenoidbone.24Fromthere,thetumorgrowsintothenasopharyngealandnasalspaces
anteriorlyandposteriorlybeforeiterodesthebonesofthemaxillary,ethmoid,andsphenoidsinuses.Itcanalsospread
tothepterygopalatinefossa,andfromthereitcanerodethepterygoidplatesandspreadintotheinfratemporalfossaor
throughtheinferiororbitalfissureintotheorbit.Fromthebaseofthepterygoidplates,thetumorcanexpandthrough
threeforamina(lacerum,rotundum,andovale)intothemiddlecranialfossa,whereitcanfurtherinvadetheparasellar
areawithoutintraduralinvasion.Inveryrareandveryadvancedstages,thetumorcanerodetheposteriorwallofthe
sphenoidsinusandinfiltratethepituitaryfossa,opticchiasm,and/orthecavernoussinus.
Thisanalysisofgrowthcharacteristicsandthecomplexityoftumorextensions,especiallyattheskullbase,madethis
classificationsystemwellrecognizedinternationallyasthefirstcomprehensive,practical,andapplicableguidetothe
surgicalapproachandpredictionofoutcomeinthemanagementofJNA.
Forexample,thissystemclassifieserosionintotheskullbasewithoutintraduralextensionasstageIII,whileintradural
growthisclassifiedasstageIV.Thisdistinctionisverywellexplainedbythefactthatextraduralinvasionoftheskullbase
isoperableandcanbeextirpatedcompletelywithoutmajorcomplications,whileintraduralinvasionisassociatedwitha
highriskofmajorcomplicationsand,ifitisextensive,shouldbetreatedwithtumordebulkingandpostoperative
radiotherapy.
TheAndrewsFischsystemwasdesignedduringatimewhentheonlysurgicalapproachesavailablefortumorsatthis
regionwereopenprocedures.Therefore,itsmajordrawbackintermsofcurrentapplicationinouropinionisthatitdoes
nottakeintoaccountrecentadvancesinbothradiologicimagingandsurgicaltechniques.Asaresult,itisdifficultto
predictcurerates,risksofcomplications,andsitesofresidualandrecurrentdiseasewiththeAndrewsFischsystem.
Mishrasystem.Thatsameyear,Mishraetalproposedtheirsystem,butitdidnotbecomewidelyaccepted.25Their
systemwasbasedontheirexperiencewith100casesofJNA.TheynoticedthatthegrowthpatternofJNAdiffers
accordingtothepatient'sageatpresentationinthatitismoreextensiveinadolescentsthaninpatientsyoungerthan10
years.Theyalsoclassifiedtumorextensionchronologicallyasprimary,secondary,andtertiary.Theyproposedthat(1)
primaryextensionsgrowinananterior,posterior,ormedialdirection,(2)secondaryextensionsstartgrowinglaterallyor
tothecontralateralnostril,and(3)tertiaryextensionsinvadethecheek,orbit,infratemporalfossa,andskullbase.
Mishraetalbasedtheirsystemontheirearlieranalysesofpatternsofgrowthandextensionandtheavailablesurgical
approachesandmodalities.However,despitethefactthattheygavegreatconsiderationtothedifferentcategoriesof
extensionandsubclassifiedskullbaseextensionaccordingtothepresenceorabsenceofduralinvolvement,theydid
notgivedueconsiderationtoallofthesurgicaloptionsthatwereavailableatthetime.
AnothershortcomingoftheMishrasystemisthatonly25%ofthecasestheauthorsreviewedhadbeenwellevaluated
preoperativelybyCT.Finally,theyreportedanoticeablyhighsurgicalfailurerate(27%).Alloftheseissuesputthe
validityandapplicabilityoftheMishrasystemintoquestion.
BagatellaMazzonisystem.In1995,BagatellaandMazzonisuggestedamodificationoftheoriginalFischstaging
system(table1).26Thenewsystemtookintoaccounttheprogressivedegreesoftumorextensionandthesurgical
difficultiesencounteredinattackingthem.Theauthorsbasedtheirclassificationon34consecutivelypresentingcasesof
JNAthatwereallmanagedwithasimilarmicroscopictransmaxillarysurgicalapproach.Inouropinion,theBagatella
Mazzonisystemwashardtoadoptbecauseitfailedtoguidesurgicaldecisionmakingortocorrelatewiththeprognosis.
Radkowskisystem.In1996,RadkowskietalsuggestedsomemodificationstotheSessionssystembasedontheir
experiencewith23cases(table1).27Theycontendedthatpreoperativetumorstageistheprimaryfactoraffectingtumor
recurrence.TheRadkowskisystemcombinedtheadvantagesofsomeoftheothersystemsandcorrelatedeachstage
withthebestsurgicalapproachthatwouldminimizetheriskofresidualorrecurrenttumor.Theirsystemhasbecomeone
ofthemostwidelyusedtoday.
OnemodificationoftheSessionssysteminvolvedstageIIRadkowskietaladdedtumorextensionposteriortothe
pterygoidplatestostageIIC.TheyalsomodifiedstageIIIbysubdividingitintosubstagesIIIAandIIIB.StageIIIAwas
definedasminimalskullbaseinvolvement,includingthemiddlecranialfossaand/orbaseofthepterygoidplatesstage
IIIBwasdefinedasextensiveintracranialinvolvementwithorwithoutinvasionintothecavernoussinus.Basedontheir
newsystem,Radkowskietalgraded9oftheir23caseshigherthantheywouldhavebeengradedwiththeSessions
system4caseswereupgradedtostageIICand5tostageIIIB.23
ThepopularityoftheRadkowskisystemnotwithstanding,theanalysisbyRadkowskietalshowedthattherewassome
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
7/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
confusionabouttheSessionssystemRadkowskietalthoughtthatSessionshadclassifiedminimalintracranial
extensionasstageIICwheninfactSessionslistedintracranialextensioningeneralasstageIII.Moreover,theupgrading
ofsomecasesdidnotchangethewaytheyweremanaged,nordidithaveaneffectonrecurrencerecurrencesafter
primarysurgerywereseenin5ofthe23cases(22%),3ofwhichhadbeenupgraded.
Onercisystem.By2006,advancesinendoscopicandmicroscopicsurgeryandtheevolutionofnewtechniquesin
preoperativeevaluationandembolizationpromptedadesireforanewclassificationsystembasedonthesitesof
residualandrecurrentdisease.Tothatend,Onercietalsuggestedamodificationofexistingstagingsystemsbasedon
theirretrospectiveanalysisof36patientstheytreated(table1).28Basedondiseaseextent,theirsystemtookinto
accountthenewsurgicalapproachesthathadevolvedandthesurgicaldifficultiesthathadbeenencountered,aswellas
thesitesofresidualdisease.Theiraimwastoachieveabetterevaluationofthetumorandareductionintheriskof
residualandrecurrentdisease.
IntheOnercisystem,involvementoftheethmoidandsphenoidsinuseswasconsideredstageIdiseasebecausein
thesecases,thetumorcanbecompletelyremovedviaanendoscopicendonasalapproachwithoutadditionalsurgical
challenges.
MaxillarysinusextensionandtotalinvolvementofthepterygopalatinefossawerebothclassifiedasstageII,sinceboth
situationsindicatethepresenceofalargetumorthatwillrequiresomemodificationsofthesurgicalapproachand
technique.Minimalinvolvementoftheinfratemporalfossa,definedasnoextensionbeyondthelateralborderofthe
posteriormaxillarysinuswall,wasalsoclassifiedasstageIIdisease,sincetheauthorsbelievedthatsuchanextension
couldberemovedendoscopically.
InfratemporalfossainvolvementbeyondthelateralborderoftheposteriormaxillarysinuswallwasclassifiedasstageIII
disease,sinceitnecessitatesanexternalsurgicalapproachforcompletetumorclearance.
Despitethefactthatsomereportsintheliteraturehadrevealedthatthemostcommonsiteofdiseaserecurrencewasthe
baseofthepterygoidplates(75to93%ofcases),28Onercietalwerethefirsttoaddressthisissue.Theybelievedthat
suchinvolvementwasnotonlyassociatedwithahighrateofrecurrence,butitalsomightindicatethepresenceofmiddle
cranialfossaextensionbecauseitisconsideredtobethemostlikelyrouteofintracranialinvolvement.Therefore,
extensionintothecancellousboneofthepterygoidplatesandinvolvementofthepterygoidmuscleswereclassifiedas
stageIIIdisease.
Intracranialextensionwasclassifiedaccordingtothesiteofinvolvement.Anteriorcranialfossaextensionwasclassified
asstageIIdiseasesinceitisapproachableendoscopically,whilemiddlecranialfossaextensionwasclassifiedasstage
IVdiseasebecauseitrequiresanexternalapproachforitsclearance.AccordingtoOnercietal,allstageIVtumorsare
extensiveandshouldbemanagedviaacombinedendoscopicandexternalapproach,althoughcompleteclearance
occasionallymaynotbepossible.
TheOnercistagingsystemisrelativelynew,andithasbeentestedinonlyonestudyof46casesofJNAreportedby
Nicolaietalin2010(table2).29However,thesetumorshadbeenresectedentirelythroughanendoscopicapproach,
despitethefactthat26ofthesecaseswereOnercistageIIItumors.AlthoughwebelievethattheOnercistagingsystemis
quitecomprehensiveandbasedonlongexperiencewiththemanagementofJNAviabothopenandendoscopic
approaches,thepossibilitythatitwouldfailtoguidethesurgeontothebestresectionmodalityisasignificantdrawback.
FurtherreportsareneededtoevaluatethevalidityoftheOnercistagingsystem.
Tyagisystem.Laterin2006,TyagietalproposedsomemodificationstostagesII,III,andIVoftheAndrewsFisch
system.30Theybasedtheirclassificationofintracranialextraduralextensiononthesizeofthetumorandthesurgical
approachnecessaryforitsexcision.SmallextraduralextensionswereclassifiedasstageIIIB,andtherecommended
surgicalapproachwasacombinedtranspalatalandtransmaxillaryprocedure.Largeextraduralextensionwasclassified
stageIVA,andfrontotemporalcraniotomywastherecommendedapproach.
Intheirreportof95casesofJNArangingbetweenstageIIandIV,theyindicatedthatmostcaseswereresectedviaan
openapproach(table2).30However,theirmodificationhasnotbeenadvocatedbyotherauthorswhosubsequently
reportedtheirownseries.Webelievethattheirmodificationdidnotaddressadvancementsinendoscopicsinusand
skullbasesurgery.
INCansystem.In2008,CarrilloetalattheInstitutoNacionaldeCancerologia(INCan)inMexicoCityintroduceda
promisingnewstagingsystemthatwasbasedoncorrelatingtumorsize,tumorlocation,thebestsurgicalapproach,and
therecurrencerate(table1).31Theyhadretrospectivelyreviewed54casesofJNAandcomparedrecurrenceratesand
diseasefreesurvival(DFS)betweentheAndrewsFischandRadkowskisystems(table2).WiththeRadkowskisystem,
theyfoundthatDFSforstageIIIBpatientswasbetterthanthatforstageIIIApatients.AccordingtotheAndrewsFisch
system,patientswithstageIIIBdiseasehadashorterDFSthandidthosewithstageIVAandIVBdisease.
CarrilloetalalsofoundthattumorsizewasasignificantfactorinrecurrenceandDFSintheearlystages(stageIandII).
OtherfactorsthatcontributedtohighrecurrenceratesandshorterDFSwereextensionofthetumorintothe
pterygomaxillaryfissure,intracranialinvasion,skullbaseinvasion,andinfratemporalfossainvasion.Basedonthese
findings,theauthorssubclassifiedinfratemporalfossainvasionasanteriorandposteriorinrelationtothepterygoid
plates.Theyalsoclassifiedskullbaseinvasionaccordingtothedegreeofduralpenetration.
AccordingtotheINCansystem,stageIandstageIIAdiseasecanbemanagedexclusivelyviaanendoscopicapproach,
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
8/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
stageIIBandstageIIIviaacombinedendoscopicandopenapproach(preferablyfacialdegloving),andstageIVviaa
combinedanterolateralorlateralskullbaseapproach.CarrilloetalconcludedthattheINCansystemhasabetterimpact
onthepredictionofrecurrenceandDFSforpatientswithadvanceddisease.Assuch,itservesasagoodguidetothe
bestsurgicalapproachineachstageofdisease.However,sincethisclassificationisrelativelynew,itsapplicabilityand
successhaveyettobedetermined.
UPMCsystem.Finally,in2010,SnydermanandcolleaguesattheUniversityofPittsburghMedicalCenter(UPMC)
introducedthemostrecentendoscopicstagingsystemforJNA(table1).32Theytookintoconsiderationthecurrent
advancementsinendonasalsurgicalapproaches,theroutesforintracranialextensionofthetumor,andtheextentof
vascularsupplyfromtheinternalcarotidartery(ICA)theybelievedthatthesizeofthetumoranditsextensionintothe
sinusesarelesspredictiveofoutcomeafterendonasalresection.Intheirseries,74%ofcasesinvolvedskullbase
erosion,and51%involvedresidualvascularityfromtheICAafterembolizationoftheexternalcarotidarterytributaries
(table2).Theyconsideredthesetobethemostimportantpredictivefactorsforprognosisintermsofbloodloss,the
numberofoperationsrequiredtoresectthetumorcompletely,andtumorrecurrence.Assuch,thestagingsystemthey
proposedclassifiedthepresenceofresidualvascularityfromtheICAasstageIV,whileintracranialextensionwas
classifiedasstageV.
TheUPMCsystemisverycomprehensive,andittakesintoconsiderationthenatureofthetumorandcurrentsurgical
approaches.Inaddition,itcorrelateswellwithprognosis.Finally,itgivessurgeonsthechoicetotakethesurgical
approachoftheirpreference.However,surgeonscanstageJNAwiththeUPMCsystemonlyafterembolizationofthe
tumor.Inotherwords,ifnoembolizationisperformedbeforesurgery,theUMPCstagingsystemisnotapplicable.In
addition,thisstagingsystemisnotaguidetodeterminingwhichapproachshouldbeusedforeachJNAstage.Finally,
becauseitisanewsystem,itneedstobeadoptedbymoresurgeonsbeforeitsvaliditycanbeascertained.
Conclusion
WhenlookingatsomeoftheseriesofJNAcasesthathavebeenpublishedintheliteratureoverthepast15years,itis
clearthattheRadkowskisystemandeithertheoriginalFischsystemortheAndrewsFischsystemwerethemost
frequentlyusedbysurgeons(table2).4,5,7,13,14,17,19,20,2848Itappearsthatsurgeonshavefoundthatthesesystems
correlateaccuratelyandsignificantlywiththeirdecisionsonsurgicalapproachestoJNAwithdifferentdegreesof
extensionandthattheyaregoodindicatorsofdiseaserecurrenceandprognosis.Inaddition,itisimportanttoconsider
theOnerci,INCan,andUPMCsystems,whichseemtocorrelatebetterwithcurrentadvancesindiagnosticimagingand
surgicaltechniques.However,sincethesethreesystemsarerelativelynew,moreexperiencewiththemisencouraged
beforeweadvocateanyoneofthemasthenewstandarduponwhichtostageJNA.
Inthisreview,wehavehighlightedtheexistingcontroversiesregardingthestagingofJNA.Nowadays,withthe
significantadvancesinourunderstandingoftransnasalendoscopicapproaches,instrumentation,andimaging
technologysuchasintraoperativeMRIandimageguidednavigationsystemstherehasbeenanoticeablechangeinthe
expectedratesofresidualandrecurrentdisease,aswellastheoverallprognosisforpatientswithJNA.
TherehaslongbeenasubstantialneedforauniversalstandardizedJNAstagingsystemthattakesintoconsiderationall
thefactorsthatplayaroleinJNA.Inouropinion,onlyonesystemtheUPMCsystemmeetsthisneed.However,since
thissystemisstillnewandhasnotbeenclinicallyappliedbysurgeonsotherthanSnydermanetal,32thereisaneedfor
anintegratedmultidisciplinaryeffortbyexperiencedsurgeonstousetheUPMCsystemandtosharetheirexperiences
withitintermsofitscredibilityandfeasibilityasabalanced,informative,andguidingstagingsystem.Oncethathappens,
perhapsitcanbeuniversallyadoptedastheidealstandardizedstagingsystemforJNA.
References
1. PryorSG,MooreEJ,KasperbauerJL.Endoscopicversustraditionalapproachesforexcisionofjuvenile
nasopharyngealangiofibroma.Laryngoscope2005115(7):12017.
2. ChauveauC.Juvenilenasopharyngealangiofibroma.ArchIntLaryngol1906.Citedby:BorgheiP,Baradaranfar
MH,BorgheiSH,SokhandonF.Transnasalendoscopicresectionofjuvenilenasopharyngealangiofibroma
withoutpreoperativeembolization.EarNoseThroatJ200685(11):7403,746.
3. MairEA,BattiataA,CaslerJD.Endoscopiclaserassistedexcisionofjuvenilenasopharyngealangiofibromas.
ArchOtolaryngolHeadNeckSurg2003129(4):4549.
4. MidilliR,KarciB,AkyildizS.Juvenilenasopharyngealangiofibroma:Analysisof42casesandimportantaspects
ofendoscopicapproach.IntJPediatrOtorhinolaryngol200973(3):4018.
5. AndradeNA,PintoJA,NbregaMdeO,etal.Exclusivelyendoscopicsurgeryforjuvenilenasopharyngeal
angiofibroma.OtolaryngolHeadNeckSurg2007137(3):4926.
6. EnepekidesDJ.Recentadvancesinthetreatmentofjuvenileangiofibroma.CurrOpinOtolaryngolHeadNeck
Surg200412(6):4959.
7. OnerciTM,YcelOT,OretmenoluO.Endoscopicsurgeryintreatmentofjuvenilenasopharyngeal
angiofibroma.IntJPediatrOtorhinolaryngol200367(11):121925.
8. NewlandsSD,WeymullerEAJr.Endoscopictreatmentofjuvenilenasopharyngealangiofibroma.AmJRhinol
199913(3):21319.
9. OsbornDA.Thesocalledjuvenileangiofibromaofthenasopharynx.JLaryngolOtol195973(5):295316.
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
9/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
10. GirgisIH,FahmySANasopharyngealfibroma:Itshistopathologicalnature.JLaryngolOtol197387(11):1107
23.
11. SchickB,PlinkertPK,PrescherA.Aetiologyofangiofibromas:Reflectionontheirspecificvascularcomponent[in
German].Laryngorhinootologie200281(4):2804.
12. MarshallAH,BradleyPJ.Managementdilemmasinthetreatmentandfollowupofadvancedjuvenile
nasopharyngealangiofibroma.ORLJOtorhinolaryngolRelatSpec200668(5):2738.
13. MannWJ,JeckerP,AmedeeRG.Juvenileangiofibromas:Changingsurgicalconceptoverthelast20years.
Laryngoscope2004114(2):2913.
14. SennesLU,ButuganO,SanchezTG,etal.Juvenilenasopharyngealangiofibroma:Theroutesofinvasion.
Rhinology200341(4):23540.
15. NakamuraH,KawasakiM,HiguchiY,etal.Transnasalendoscopicresectionofjuvenilenasopharyngeal
angiofibromawithKTPlaser.EurArchOtorhinolaryngol1999256(4):21214.
16. CarrauRL,SnydermanCH,KassamAB,JungreisCA.Endoscopicandendoscopicassistedsurgeryforjuvenile
angiofibroma.Laryngoscope2001111(3):4837.
17. YiotakisI,EleftheriadouA,DavilisD,etal.JuvenilenasopharyngealangiofibromastagesIandII:Acomparative
studyofsurgicalapproaches.IntJPediatrOtorhinolaryngol200872(6):793800.
18. DouglasR,WormaldPJ.Endoscopicsurgeryforjuvenilenasopharyngealangiofibroma:Wherearethelimits?
CurrOpinOtolaryngolHeadNeckSurg200614(1):15.
19. KhalifaMA,RagabSM.EndoscopicassistedantralwindowapproachfortypeIIInasopharyngealangiofibroma
withinfratemporalfossaextension.IntJPediatrOtorhinolaryngol200872(12):185560.
20. EloyP,WateletJB,HatertAS,etal.Endonasalendoscopicresectionofjuvenilenasopharyngealangiofibroma.
Rhinology200745(1):2430.
21. SessionsRB,BryanRN,NaclerioRM,AlfordBR.Radiographicstagingofjuvenileangiofibroma.HeadNeck
Surg19813(4):27983.
22. ChandlerJR,GouldingR,MoskowitzL,QuencerRM.Nasopharyngealangiofibromas:Stagingandmanagement.
AnnOtolRhinolLaryngol198493(4Pt1):3229.
23. AntonelliAR,CappielloJ,DiLorenzoD,etal.Diagnosis,staging,andtreatmentofjuvenilenasopharyngeal
angiofibroma(JNA).Laryngoscope198797(11):131925.
24. AndrewsJC,FischU,ValavanisA,etal.Thesurgicalmanagementofextensivenasopharyngealangiofibromas
withtheinfratemporalfossaapproach.Laryngoscope198999(4):42937.
25. MishraSC,ShuklaGK,BhatiaN,PantMC.Arationalclassificationofangiofibromasofthepostnasalspace.J
LaryngolOtol1989103(10):91216.
26. BagatellaF,MazzoniA.Microsurgeryinjuvenilenasopharyngealangiofibroma:Alateronasalapproachwith
nasomaxillarypedicledflap.SkullBaseSurg19955(4):21926.
27. RadkowskiD,McGillT,HealyGB,etal.Angiofibroma.Changesinstagingandtreatment.ArchOtolaryngolHead
NeckSurg1996122(2):1229.
28. OnerciM,OretmenoluO,YcelT.Juvenilenasopharyngealangiofibroma:Arevisedstagingsystem.
Rhinology200644(1):3945.
29. NicolaiP,VillaretAB,FarinaD,etal.Endoscopicsurgeryforjuvenileangiofibroma:Acriticalreviewofindications
after46cases.AmJRhinolAllergy201024(2):e6772.
30. TyagiI,SyalR,GoyalA.Stagingandsurgicalapproachesinlargejuvenileangiofibromastudyof95cases.IntJ
PediatrOtorhinolaryngol200670(9):161927.
31. CarrilloJF,MaldonadoF,AlboresO,etal.Juvenilenasopharyngealangiofibroma:Clinicalfactorsassociated
withrecurrence,andproposalofastagingsystem.JSurgOncol200898(2):7580.
32. SnydermanCH,PantH,CarrauRL,GardnerP.Anewendoscopicstagingsystemforangiofibromas.Arch
OtolaryngolHeadNeckSurg2010136(6):58894.
33. GuptaAK,RajiniganthMG,GuptaAK.Endoscopicapproachtojuvenilenasopharyngealangiofibroma:Our
experienceatatertiarycarecentre.JLaryngolOtol2008122(11):11859.
34. DanesiG,PancieraDT,HarveyRJ,AgostinisC.Juvenilenasopharyngealangiofibroma:Evaluationandsurgical
managementofadvanceddisease.OtolaryngolHeadNeckSurg2008138(5):5816.
35. BorgheiP,BaradaranfarMH,BorgheiSH,SokhandonF.Transnasalendoscopicresectionofjuvenile
nasopharyngealangiofibromawithoutpreoperativeembolization.EarNoseThroatJ200685(11):7403,746.
36. ElBanhawyOA,RagabA,ElSharnobyMM.SurgicalresectionoftypeIIIjuvenileangiofibromawithout
preoperativeembolization.IntJPediatrOtorhinolaryngol200670(10):171523.
37. TosunF,OzerC,GerekM,YetiserS.Surgicalapproachesfornasopharyngealangiofibroma:Comparative
analysisandcurrenttrends.JCraniofacSurg200617(1):1520.
38. CansizH,GvenMG,SekercioluN.Surgicalapproachestojuvenilenasopharyngealangiofibroma.J
CraniomaxillofacSurg200634(1):38.
39. HofmannT,BernalSprekelsenM,KoeleW,etal.Endoscopicresectionofjuvenileangiofibromaslongterm
results.Rhinology200543(4):2829.
40. KaniaRE,SauvagetE,GuichardJP,etal.EarlypostoperativeCTscanningforjuvenilenasopharyngeal
angiofibroma:Detectionofresidualdisease.AJNRAmJNeuroradiol200526(1):828.
41. PasquiniE,SciarrettaV,FrankG,etal.Endoscopictreatmentofbenigntumorsofthenoseandparanasal
sinuses.OtolaryngolHeadNeckSurg2004131(3):1806.
42. ElBanhawyOA,ShehabElDienAelH,AmerT.EndoscopicassistedmidfacialdeglovingapproachfortypeIII
juvenileangiofibroma.IntJPediatrOtorhinolaryngol200468(1):218.
43. WormaldPJ,VanHasseltA.Endoscopicremovalofjuvenileangiofibromas.OtolaryngolHeadNeckSurg2003
129(6):68491.
44. NicolaiP,BerlucchiM,TomenzoliD,etal.Endoscopicsurgeryforjuvenileangiofibroma:Whenandhow.
Laryngoscope2003113(5):77582.
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
10/11
7/24/2015
Juvenilenasopharyngealangiofibromastaging:Anoverview
45. MairEA,BattiataA,CaslerJD.Endoscopiclaserassistedexcisionofjuvenilenasopharyngealangiofibromas.
ArchOtolaryngolHeadNeckSurg2003129(4):4549.
46. RogerG,TranBaHuyP,FroehlichP,etal.Exclusivelyendoscopicremovalofjuvenilenasopharyngeal
angiofibroma:Trendsandlimits.ArchOtolaryngolHeadNeckSurg2002128(8):92835.
47. PetrusonK,RodriguezCatarinoM,PetrusonB,FiniziaC.Juvenilenasopharyngealangiofibroma:Longterm
resultsinpreoperativeembolizedandnonembolizedpatients.ActaOtolaryngol2002122(1):96100.
48. JorissenM,EloyP,RombauxP,etal.Endoscopicsinussurgeryforjuvenilenasopharyngealangiofibroma.Acta
OtorhinolaryngolBelg200054(2):20119.
FromtheDepartmentofOtolaryngology,DammamMedicalComplex,Dammam,KingdomofSaudiArabia(Dr.Alshaikh)
andtheENTDepartment,GeneralHospitalofRethymnon,Rethymnon,Greece(Dr.Eleftheriadou).
Correspondingauthor:NadaAliAlshaikh,MD,DepartmentofOtolaryngology,DammamMedicalComplex,POBox
2471,Dammam31451,SaudiArabia.Email:nadaats@yahoo.com
EarNoseThroatJ.2015June94(6):E12
COPYRIGHT2015BYVENDOMEGROUP.UNAUTHORIZEDREPRODUCTIONOR
DISTRIBUTIONSTRICTLYPROHIBITED
SourceURL:http://www.entjournal.com/article/juvenilenasopharyngealangiofibromastagingoverview
http://www.entjournal.com/print/article/juvenilenasopharyngealangiofibromastagingoverview
11/11