BrazJOtorhinolaryngol.2016;82(5):610---613
www.bjorl.org
Brazilian
Journal
of
OTORHINOLARYNGOLOGY
CASE
REPORT
Ameloblastic
fibrodentinosarcoma:
a
rare
malignant
odontogenic
tumor
夽
Fibrodentinossarcoma
ameloblástico:
um
raro
tumor
odontogênico
maligno
Leorik
Pereira
da
Silva
a,∗,
Jefferson
da
Rocha
Tenório
a,
Bartolomeu
Cavalcanti
de
Melo
Júnior
b,
José
Paulo
da
Silva
Filho
c,
George
João
Ferreira
do
Nascimento
d,
Ana
Paula
Veras
Sobral
eaUniversidadeFederaldoRioGrandedoNorte(UFRN),DepartamentodeOdontologia,PatologiaOral,Natal,RN,Brazil
bHospitalUniversitárioOswaldoCruz,DepartamentodeCirurgiadeCabec¸aePescoc¸o,Recife,PE,Brazil
cInstitutoSãoLeopoldoMandic,CentrodePesquisas,SLMandic,SãoPaulo,SP,Brazil
dUniversidadeFederaldeCampinaGrande(UFCG),FaculdadedeOdontologia,CentroAcadêmicodeCiênciasBiológicas,
PatologiaOral,Patos,PB,Brazil
eUniversidadedePernambuco(UPE),FaculdadedeOdontologia,PatologiaOral,Camaragibe,PE,Brazil
Received7April2015;accepted28April2015 Availableonline8September2015
Introduction
Ameloblasticfibrodentinosarcoma (AFDS)isa rare odonto-genictumorhistologically characterizedby asarcomatous ectomesenchymal component associated with variable amountsofbenign ameloblastomatousepitheliumandthe presenceof dysplastic dentin.1 About 14cases have been
reportedinthemedicalliterature.TheetiologyoftheAFDS
ispoorlyunderstood;however,approximatelyone-thirdof
AFDSappeartorepresentthemalignanttransformationof
pre-existingameloblasticfibrodentinoma.1,2
夽 Pleasecitethisarticleas:daSilvaLP,daRochaTenórioJ,deMelo
JúniorBC,daSilvaFilhoJP,doNascimentoGJ,SobralAP. Ameloblas-ticfibrodentinosarcoma:araremalignantodontogenictumor.Braz JOtorhinolaryngol.2016;82:610---3.
∗Correspondingauthor.
E-mails:[email protected],
[email protected](L.P.daSilva).
AFDShasapredilectionforthemandible,anditismost
commonly seen in male patients in the third decade of
life. Patients often present with a painful swelling, and
AFDS is radiographically characterized by a multilocular
radiolucentlesionwithindistinct margins,withorwithout
radiopaquefoci.Metastasesarerare,butrecurrenceshave
been reported. The treatment of choice is wide surgical
resection.3
Giventheabove,theaimofthisreportistodescribea
caseofaggressivemandibularAFDSwithemphasison
clini-cal,radiological,andhistopathologicalaspects.
Case
report
A 19-year-oldman wasreferred topublicservice for oral
pathology consultation in 2014. The extraoral inspection
showedalargeswellingwithstrongfacialasymmetryonleft
sideoftheface(Fig.1).Intraorally,anextensiveulcerated
andnecroticmasswasobservedfromthetooth35
extend-ingtothemandiblebodyandramus,withclinicalabsence
ofteeth36,36,and38.Despitethepresenceofulceration,
http://dx.doi.org/10.1016/j.bjorl.2015.04.009
Ameloblasticfibrodentinosarcoma 611
Figure1 Clinicalfindings.Frontviewofthepatientshowing markedfacialasymmetry.
thepatientonlyreportedpainonpalpation,andlimitation
inmouthopeningwasnoted.Therewasnoclinicalevidence
ofregionallymphadenopathy.
The radiographic exam revealed an ill-defined
radi-olucency measuring about 7cm×4.5cm associated with
impacted molar (36), lacking sclerotic borders and
asso-ciated with the presence of multiple radiopaque flecks.
Expansionandthinningofcorticalbonewerealsoobserved
(Fig.2).Calcifyingepithelialodontogenictumor,calcifying
odontogeniccyst,andameloblasticfibrodontomawerethe
mainclinicaldifferentialdiagnosesconsidered.
An incisional biopsy was performed and submitted to
histopathologicalexam.Microscopicexaminationofthe
sec-tionsroutinelystainedinhematoxylinandeosinrevealeda
biphasic neoplasticproliferation of odontogenicepithelial
andmesenchymal tissue.The odontogenicepithelial
com-ponentconsistedofmultiplecordsandislandsboundedby
Figure2 Panoramicradiographshowingmultilocular exten-sive radiolucent lesion with radiopaque foci in the left mandible,associatedwithimpactedmolar(36).
Figure 3 Histopathological findings. Biphasic pattern with benign odontogenic epithelium surrounded by sarcomatous componentwithintensecellularandnuclearpleomorphismand mitoticfigures(H&Estain,200×).
columnarto cuboidal ameloblast-like cells, withreversed
nuclear polarity. In the center of these structures, the
neoplastic cells had a loose aspect, resembling the
stel-late reticulum of the enamel organ. The mesenchymal
componentconsistedofa primitiveconnectivetissuethat
displayedmarkedpleomorphismcharacterizedbyvariation
in cell size and shape, as well as nuclear
hyperchro-matism, alteration in nuclear to cytoplasmic ratio, and
scattered mitotic figures (Fig. 3). Areas of necrosis focus
wereidentified.Juxta-epithelial hyalinizationwasevident
in few areas. Focal areas of dentinoid-like material with
tubuleswerealsoobserved(Fig.4).Enamelformationcould
notbeidentified,evenonmultiplesections.Furthermore,
ameloblasticfibromaareaswerepresent.Therefore,
clini-cal,radiographic,andhistopathologicalfindingssupported
thediagnosisofameloblasticfibrodentinosarcoma.
Theoncologistandsurgeonchosetoperform
chemother-apyprevioustothesurgicaltreatment.Initially,threecycles
ofadrugcombination wereadministered,comprising
vin-cristine,cyclophosphamide,and cloxorubicin. Thepatient
didnotrespondwelltoinitialtreatmentanddidnotappear
forthesurgeryonthedayscheduled,returningonlyafter
fourweeks.Thus,anewcombinationofdrugsinthreecycles
612 daSilvaLPetal.
Figure 5 Craniocaudal view of the patient showing a sig-nificant increase in tumor mass after eight sessions of chemotherapy.
wasinstituted, consisting of ifosfamide, carboplatin, and
etoposideinordertoreducetumorsizebeforesurgery.
However, after two cycles of chemotherapy with
dif-ferentdrugs, thepatient showeda significant increase in
tumormasswithskininvasion,limitedmouthopening,and
extreme facial deformity (Fig. 5). After the unexpected
aggressiveevolutionofthetumor,surgeonsperformed
rad-ical left hemimandibulectomy with peripheral myotomy
of the muscle insertions associatedwith resection of the
affectedskin.Thebonemarginof3cmbeforethe
mandibu-lar symphysis andthe peripheral muscles removed witha
margin of 2cm were tumor-free. The left inferior
alveo-lar nervewasinvolved by the tumor; however,there was
nopresenceofinvasionofbloodorlymphaticvessels.
Ipsi-lateral supraomohyoidneck dissection of 18 lymphnodes
showednotumorcells. Thepatientunderwent
postopera-tiveradiotherapywith6000Gy.
The patienthasbeen re-evaluated everythreemonths
withchestX-rayandCTscanoftheheadandneck.There
hasbeennoevidenceofregionalordistantmetastasis,and
thepatienthasbeenunderclinicalfollow-upforoneanda
halfyears.
Discussion
Odontogenic tumors (OT) consist of a group of rare and
heterogeneous lesions, representing less than 4% of all
specimensof the oral andmaxillofacial region.Malignant
odontogenicneoplasmsareevenrarerandconstituteasmall
percentage of OT. In various series published worldwide,
thefrequencyvariesbetween0%and6.1%.4The
pathogen-esisofmalignantOTisunclear,althoughsomeauthorshave
suggestedalterationsincellularcycle,expressionof
proto-oncogenes,andmutationsintumorsuppressorgenesinthe
pathogenesisoftheselesions.4
Microscopically,theblandepithelialcomponentofAFDS
issimilartothatseeninameloblasticfibrosarcoma,although
it is less frequent.5,6 The definitive diagnosis of AFDS is
establishedbasedonhistopathologicevaluationofthe
mes-enchymalcomponent,whichusuallydemonstratesfeatures
ofmalignancy,includingcellularatypia,pleomorphism,and
mitoticfigures.Inaddition,whenamaterialsimilartothe
dentinoidisobserved,thefinaldiagnosisshouldbeAFDS.7,8
Despitethemorphologicaldifferences,theWorldHealth
Organization (WHO) distinguishes odontogenic sarcoma
devoid of dental hard tissue (ameloblastic
fibrosar-coma) from those displaying focal evidence of dentinoid
(ameloblasticfibrodentinosarcoma)ordentinoidplus
enam-eloid (ameloblastic fibro-odontosarcoma), but the WHO
panelacknowledgesthatpresenceorabsenceofdentalhard
tissueinanodontogenicsarcomaisofnoprognostic
signif-icance.The literaturereportsthatthe biologicalbehavior
oftheAFDSis,ingeneral,similartootherodontogenic
sar-comas,withhighlocalaggressivenessandlowpotentialfor
regionallymphnodeinvolvementordistantmetastasis.6---8
Radiographically,AFDS canshow a uni- or multilocular
appearance,withpoorlycircumscribedoutlinesassociated
withtooth and oneor more denseopacities. The present
caseshowedamultilocularappearanceassociatedwiththe
leftlowerfirstmolar(36)andslightdenseopacities.These
radiographicfindingsandthelocationofthemasshighly
sug-gestthepossibilityofodontogeniccystsandtumor.3,8The
clinico-radiographic differential diagnosis should include
calcifyingepithelial odontogenictumor,calcifying
odonto-geniccyst,andameloblasticfibrodontoma.However,acase
withanirregular borderandexpansionandperforation of
the cortexes should beinterpreted with caution,and the
possibilityof malignant odontogenictumor shouldbe
sus-pected.
ThemeanageatthetimeofdiagnosisofAFDSin62cases
reviewed by Bregniet al.7 was27.3 ina wideage range,
from3to83 years.Accordingtothese62 publishedcases,
thetumorismorecommoninmalesthanfemales(59.7%vs.
37.1%).Furthermore, odontogenicsarcomasaremore
fre-quentinthemandible(79%ofcases)thanthemaxilla(21%),
withthemajorityofthecaseslocatedinposteriorregionof
themandible.9
Odontogenicsarcomasarereportedasahighlyrecurrent
lesion. Till date, 25 (35%) of the 71 reported cases have
had at least one recurrence during follow-up period and
14patients(19.7%)havediedoftheirdiseasewithinthree
monthsto19 years.Clinicalfindingsvaryamongreported
cases, but usually signs and symptoms include pain and
swelling.Despiteregionallymphnode involvementor
dis-tantmetastasesreportedin fewcases,someauthorshave
consideredAFDSasalow-gradesarcoma.2---8
Inarecentmulticenterepidemiologicalstudyconducted
inLatinAmerica,25casesofmalignantodontogenictumors
werereported,and sixcaseswereodontogenic sarcomas.
Allcaseshadthediagnosisofameloblasticfibrosarcomaand
nocaseofAFDSwasidentified.10Inaddition,ina
retrospec-tivestudyconductedinBrazil,240odontogenictumorswere
described;however,nocasesofodontogenicsarcomawere
found.11
ThetreatmentofchoiceforAFDSisradicalsurgical
exci-sion without primary neck dissection. Some investigators
Ameloblasticfibrodentinosarcoma 613
butitsbenefitsareuncertain.3Owingtotherarityofcases,
it is difficult toaccurately estimate long-term prognosis.
Particularly inthis case, thepatient didnot satisfactorily
respondtochemotherapy beforesurgery, leadingto
clini-calworseningandincreasedtumoraggressiveness;thisfact
emphasizes the dilemma of whether or not the adjuvant
chemotherapyshouldbeindicatedinthetreatmentofthese
sarcomas.
Conclusion
Insummary,thiscasereportdescribesthefirstcaseofAFDS
inBrazilandemphasizestheimportanceofconsideringthe
odontogenicsarcomas asa differentialdiagnosisof
maxil-laryosteolyticlesions,despitebeingextremelyrarelesions.
Thus,theselesionsareachallengingdiagnosisforclinicians
andpathologists.
Conflicts
of
interest
Theauthorsdeclarenoconflictsofinterest.
Acknowledgements
CAPESandCNPqforpostgraduatescholarships.Dr.Rachel
and staff of the Oncology Center in University Hospital
OsvaldoCruz.
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