in children: A case report of challenging diagnosis Osteoﬁbrous adamantinoma dysplasia-like versus osteoﬁbrousdysplasia International Journal of Surgery Case Reports CASE REPORT – OPEN ACCESS

(1)

ContentslistsavailableatScienceDirect

International Journal of Surgery Case Reports

j o ur na l h o m e p a g e :w w w . c a s e r e p o r t s . c o m

Osteoﬁbrous dysplasia-like adamantinoma versus osteoﬁbrous dysplasia in children: A case report of challenging diagnosis

Achmad Fauzi Kamal

^a

, Fahmi Anshori

^a,∗

, Evelina Kodrat

^b

aDepartmentofOrthopaedic&Traumatology,CiptoMangunkusumoNationalCentralHospitalandFacultyofMedicine,UniversitasIndonesia,Jalan DiponegoroNo.71,JakartaPusat,Jakarta10430,Indonesia

bMusculoskletalPathologyDivision,DepartementofAnatomicPathology,FacultyofMedicineUniversitasIndonesia-CiptoMangunkusumoHospital, Jakarta,Indonesia

a rt i c l e i nf o

Articlehistory:

Received7January2021

Receivedinrevisedform22January2021 Accepted23January2021

Availableonline28January2021

Keywords:

Osteoﬁbrousdysplasia

Osteoﬁbrousdysplasia-likeadamantinoma Immunohistochemicalstaining

a b s t ra c t

INTRODUCTION:Osteoﬁbrousdysplasia(OFD)andOsteoﬁbrousdysplasia-likeAdamantinomahaveasim- ilarappearancebothinclinicalandradiography,butdifferentinitshistopathology.Despitethissimilarity, thetreatmentandprognosisaredifferent,thereforethediagnosisshouldbeestablishedprecisely.

CASEILLUSTRATION:Athree-year-oldboywasadmittedtohospitalafterfallingonhislowerleg.Abead sizelumpappearedonhistibiawithpainandswelling,whichlaterbecameenlarged.Diagnosisofosteofi- brousdysplasiaandadamantinomawasconsidered.Weperformedlimb-salvageprocedurebycurretage, bonegrafting,andinternalfixationapplication.Thehistologysectionshowedwovenbonerimmedby polygonalosteoblastcellwithinterveningfibrousstromaandsmallnestsoftumourcellsraisedthepossi- bilityofepithelialdifferentiation.Thepositivityforcytokeratinimmunostainingconfirmedthediagnosis asosteofibrousdysplasia-likeadamantinoma.Inthiscaseitisaveryrarespectrumofmalignancyin children.

DISCUSSION: These two tumor entities have identical radiographic characteristics, histopathology featuresthedistinction betweenclassicadamantinoma andOFD-like adamantinomabased onthe predominantepithelialcomponent.Therelationshipofosteofibrousdysplasiawithadamantinomais unclear.Severalauthorsconsideredpossiblecallingrelationshiposteofibrousdysplasiaas“juvenile adamantinoma”.However,doesnotruleoutthepossibleexistenceofdenovoosteofibrousdysplasia notrelatedtoadamantinoma.

CONCLUSIONS:OFD-likeadamantinomaandOsteofibrousDysplasiahadsimilarhistopathologypattern, apathologistmustbeawareofthisfeatureandperformimmunohistochemicalstainingforkeratinpar- ticularlywhenthehistopathologicalfeatureofosteofibrousdysplasiashowedsmallnestsoftumorcells withinthefibrousstroma.diagnosticchallengingandrequiremultidisciplinaryapproach.

1. Introduction

Osteoﬁbrousdysplasia(OFD)andOFD-likeAdamantinomahave asimilarappearancebothinclinicalandradiography,butdifferentinitshistopathology.Despitethissimilarity,thetreatmentand prognosisare verydifferent,thereforeestablishmentof diagno- sisshouldbeperformedprecisely.TheagespectrumofOFDisin theﬁrstandseconddecadeoflifewhereastheagespectrumof adamantinomaisolderthan20.ThepredilectionsiteofOFDmost oftenintheanteriorshaftofthetibiaofchildern[1,2].TheOFD makesuplessthan1%ofallprimarybonetumour,andusually developinpatientsyoungerthan20yearsoldorinskeletallyimma-

∗ Correspondingauthor.

E-mailaddresses:fauzikamal@yahoo.com(A.F.Kamal),fhmanshori@gmail.com (F.Anshori),evelina.kodrat@yahoo.com(E.Kodrat).

turepatients.MostlyallOFDoccurintheshaftoftibia,withfew reportsariseinﬁbulaandotherlongbonesinarmsuchashumerus, radius,andulna.Adamantinomacandevelopatanyagewithado- lescentandyoungadultaremostoftenaffected.Thesetumours frequentlyfoundinthemiddlepartofthetibia,andinmanycases theﬁbulaisalsoaffected.Inrareinstances,alsocanbefoundinthe bonesofthearm,rib,pelvic,foot,andspine.About20%ofthese tumourmetastasizetootherpartsofthebodies,mostoftentothe lung,lymphnode,andotherbones[3,4].

AlthoughOFDhastypicalhistopathologyfeature,OFD-likeareas arealsoobservedatperipheryofclassicadamantinoma,andsome haveindicatedthatOFDcouldbeeitheraprecursortooraregres- siveadamantinomaprocess[5].

Theso-called OFD-likeadamantinomasharescertainaspects ofboth OFDand adamantinoma.In anefforttobetterdescribe theirmorphology,clinicalcourse,andrelationship,expertshave analysedthephysiology,biochemical,histopathology,immunohis-

https://doi.org/10.1016/j.ijscr.2021.01.093

creativecommons.org/licenses/by-nc-nd/4.0/).

(2)

CASE REPORT – OPEN ACCESS

A.F.Kamaletal. InternationalJournalofSurgeryCaseReports80(2021)105599

tochemistry,ultrastructural,andmolecularcharacteristicsofOFD andadamantinomas.PatientswithOFDwereusuallyyoungerthan thoseofwithadamantinoma.Osteoblasticandosteoclastwasmore commoninOFDthaninOFD-likeadamantinoma.Inadditiontothe unnoticeablesmallclustersofepithelialcellsinOFD-likeadamantinoma,isolatedkeratin-positivecellswithaspecialultrastructural hybridfibroblastic-epithelial phenotypehavebeenfoundinthe stromaofallOFDandOFD-likeadamantinomas[6,7].Analysiswith insitufluorescencehybridizationrevealedtrisomies7,8,and/or 12intheOFD,OFD-like,andclassicadamantinomawithspindle cell stroma,supportingOFDneoplasticoriginanda typicalhis- togenesisforall3lesions.Trisomiesinosteoblastsorosteoclasts werenotfound,indicatingthattheosseousportionisreactiveand non-neoplastic.Herewepresentour3-year-oldboywithOsteofi- brousDysplasia-likeadamantinomathatcametoourcenter.Our manuscripthasbeenreportedinlinewiththeSCAREcriteria[15].

2. Casedescription

Athree-year-oldboycametoourpolyclinicwithchiefcomplain therewasalumpandpainonhislowerleg.Hecamewithahis- toryoftraumaintibia,andpainalongwithswelling.Abeadsize lumpappearedonhisproximaltibiawithpain,whichlaterbecame enlarged(Fig.1).

Fromphysicalexaminationthemasswasnotclearlyseen,no venectationnorwound,inpalpablewefoundhardmassatmid shafttibiawithpainvasscore3–4and,illdeﬁneborder,immobile.

Thecircumferentialdiameter24cm(contralateral23cm),range ofmotionhipandkneewasnormal.Distalneurovascularwithin normallimit(Figs.2and3).

Intraoperatively,the incisionwas madeat theanteromedial side of the leg layer by layer until the periosteum. Then the periosteumwasincisedatonesideandperformedcurettageuntil

Fig.1. Clinicalpictureofthepatient;hardpalpablemassmeasuring24cmcircumferential(contralateral23cm),immobilewithilldeﬁnemargin.

Fig.2.Preoperativeradiographyrightcruris;showinggeographiclyticlesionseptationatmidshafttibiawithoutperiostealreaction,narrowtransitionalzone,welldeﬁne margin,thereisnocortexbreakage,nosignsofttissueinvolvment.

(3)

Fig.3. MRIoftheleg;tumorlesionappearsonanteriorsideoftibia,isointensityonT1andhyperintensityonT2.Aftercontrastadministrationlesionuptakecontrastand enhancementoflesionwithhomogenouspattern,nosigninﬁltrationintosurroundingsofttissueandintramedullary.ConclusionfromMRIareprimarybonetumorsuggestif benignsuspectosteoﬁbrousdysplasia,nosignofmalignancy.

Fig.4.(A)identifyingandcurettageofthetumor,(B)bonedefectaftercurettageinthecruris,(C)bonegraftapplication,(D)smallDCPplateapplication.

(4)

CASE REPORT – OPEN ACCESS

Fig.5.(A)Thehistologyshowingirregulartrabeculaeofwovenbonerimmedbyosteoblastswithinterveningﬁbrousstromaandsmallnestsofepithelialcells(arrow),H&E, 40×;(B)Smallnestsoftumorcellpositiveforcytokeratin(100×).

Fig.6. Post-operativex-ray.

(5)

Fig.7.Clinicalpictureafterone-yearpost-operative.

thedistalandproximaledge.Afterthecurettage,thedefectwas ﬁlledwithbonegraftandtheperiosteumwassuturedback.For the reinforcementof theleg,a small 9-holesDCP wasapplied.

After that thewound was cleansedand sutured layerby layer (Figs.4–6).

DiagnosisofOFD-likeadamantinomawasconsideredafterthis casewasdiscussedinclinicopathologyforumandourpathologist suspiciousofmultipelsmallnestislandofepithelialcellinthestro- mal.Thenweproceedtoexcludethepossibilityofmalignancywith perfomedimmunohistochemicalstainingforkeratin.Thepositiv- ityforcytokeratininimmunostainingconﬁrmedthediagnosisas osteoﬁbrousdysplasia-likeadamantinoma.Inthiscase,itisvery rarespectrumofmalignancyinchildren.

Osteofibrousdysplasia-likeadamantinomahadtypicalfeature of osteofibrous dysplasia with spectrum age in childern, slow growingmass, fromimagingthere is nosignof soft tissueand intramedullary involvement ofthe tumor.Thehistology ofthis tumorisoverall similartoOFD,thebonytrabeculaearemostly rimmedbyosteoblastwithinterveningfasciclesoffibroblasticcells.

Thesmallnestsofepithelialcellsandindividualkeratinpositive cellsinthestromaarecharacteristicfeatureofOFD-likeadamantinoma.Therefore,itisessentialtoconﬁrmthediagnosisfromthe morphologyandimmunostaining.

We evaluatedthepatientoneyearaftersurgery,there isno complain,patientcanwalknormallywithoutaid.Clinicalandradi- ologicalevaluationthereisnosignofrecurrencyFigs.7and8.

3. Discussion

OsteoﬁbrousDysplasiaispredominantlyachildhood’sdisease, while adamantinoma usually occurs in adolescents and young adults,and indeedhalfofthepatientsinourOFDserieswere9 yearsoldorolder,whereasnonewithadamantinomawaslessthan 9yearsold.ThepredominantsymptomsforOFDispainless,how- everifthepainpresentitis mandatorytoincludetheOFD-like adamantinomaasadifferentialdiagnosis[1,5].

Thesetwo tumorentitieshaveidenticalradiographiccharac- teristicsincludingthepredilectionforthetibialdiaphysisanterior cortexanda multiloculated,mixedosteolyticandsclerotic pre- sentationwithscleroticmargins.Bothlesionsinthetibiamaybe multifocalorhavesynchronouslesionsintheﬁbula.Likeprevious studies,ourstudyshowedthattibialbowingismorecommonin OFD,howevermedullaryextensionismorefrequentinadamanti- noma, but there is no pathognomonic picture characteristic of eitherlesion.

The radiologic features of the OFD and OFD-like Adamanti- nomaarequitesimilar.Itisnotpossibletodistinguishbetween osteoﬁbrous dysplasia and OFD-like adamantinoma based on imagingalone[9].Classicadamantinomacouldbedistinguished fromosteoﬁbrousdysplasiaaccordingtotheinvolvementofthe medullarycavity and soft tissue extensionseen on MRI [9,12].

Completeinvolvementofthemedullarycavityisalmostalways seen in an adamantinoma. In contrast to OFD and OFD-like

(6)

CASE REPORT – OPEN ACCESS

Fig.8.Plainradiographyafterone-yearpost-operativeandimplantremoval.

adamantinoma,intramedullaryinvolvementisminimalorabsent [9,12].

Someauthorsuggestedosteofibrousdysplasiaareradiologically diagnostic, howeverradiological featureof osteofibrousdyspla- siaandOFD-likeadamantinomaareindistinguishable.Bothhave samesitepredilectiononmidshafttibia,anteriorbowing,multiple lucencies,scleroticfoci,orbothfeaturesinvolvingtheanterolat- eral cortex of the tibia withassociated cortical expansionand sclerosisoftheinterveningcortex.Thereisalittledifferentiation betweenOFD-likeadamantinomaandosteofibrousdysplasia.OFD- likeadamantinomausuallypresentwithlonghistoryofdullpain onanteriortibia,ontheotherhandosteofibrousdysplasiausu- allypainlesswithanteriorbowingoftibia,butclinicalsymptom isnotpathognomonic.However,whenwecomparedifferentiation betweenclassicadamantinomaandOFD-likeadamantinomamore clearly,in classicadamantinomamostlythereisinvolvement of intramedullarycavity,surroundingsofttissueandcompletecorti- caldisruption.Thisimagingfeatureareabletodistinguishbetween classicadamantinomawithOFD-likeadamantinoma[3].

From histopathology feature the distinction between classic adamantinomaandOFD-likeadamantinomabasedonthepredom- inantepithelialcomponent.Inclassicadamantinomatheepithelial component isdominantandeasilyseen,but theOFD-like areas areinconspicuous.ConverselyOFD-likeadamantinomahavesmall nestsofepithelialcomponentwithpredominantOFD-likeareas.

Thesmallnestsofepithelialtumorcellsarecharacteristicfeature ofOFD-likeadamantinoma.Thesmallnestofepithelialcellsalso distinguishesOFD-likeadamantinomafromosteoﬁbrousdyspla- sia,asintheosteoﬁbrousdysplasiathesmallnestofepithelialcells isnotseen.Onlysinglescatteredkeratin-positivestromalcellsare presentinOFD[3,14].

OFD-likeadamantinomaandosteofibrousdysplasiahavesimi- larhistopathologypattern,pathologistmustbeawaretoperform immunohistochemicalstainingforkeratinparticularlywhenthe histopathologicalfeature of osteofibrous dysplasia shows small nestsofepithelialtumourcellswithinthefibrousstroma[1,5].

Therelationshipofosteofibrousdysplasiawithadamantinoma isunclear.Czerniaketal.[3]reportedthatthereisacontinuum oflesionswithclassicadamantinomaatoneendandosteofibrous dysplasiaattheother.Thishypothesisisalsosupportedbyanother authorconsideredthispossiblerelationshipbycallingosteofibrous dysplasiaas“juvenileadamantinoma”.Thishypothesis,however doesnotsetasidethepossibilityofdenovoosteofibrousdysplasia thathasnorelationtoadamantinoma.However,thestudydeter- minedthattherewasnodefiniteevidenceofaprecursorrolefor osteofibrousdysplasia[3,9,11].

AstheOFDisnotmalignantlesionandwillnotgrowafterthe patientreachesskeletalmaturity,thetreatmenttypicallyinvolves observationandconservativetreatment.However,ifthelesionhas causedconsiderable damagetothebone it is advisabletoper-

(7)

formsurgicalremovalofthelesioninordertostabilizethebone.

On theotherhand, treatmentof adamantinomaalwaysrequire surgery. They do not respond to chemotherapy and radiation.

Thetreatmentforadamantinomaissomewhatlikethetreatment forGCTandosteosarcomawithregardstosurgicalmanagement, adamantinomaisafarmoreaggressivelesionthanOFDandassuch requireswidelocalresectionwithreconstructivesurgeryforopti- malmanagement.Adamantinomahasariskoflocalrecurrenceand pulmonarymetastaticdiseasedespiteislowerthananotherbone malignancy.Variousstudysuggeststhatacurettagecombinedwith bonegraftcouldbeconsideredforthetreatmentofthetumorlike adamantinoma[8–10].Conservativemanagement withobserva- tionsometimescanbedone,howeverresectionwithclearmargin isrequiredforpatientswithadamantinoma.[8,13].Oneyearafter surgeryweevaluatepatientandwefoundnosignofrecurrency norprogressionintoworsecondition.However,becausethiscase of low-grademalignancy weplantoevaluateeveryyearinthis patient.

4. Conclusion

We reported a rare case of OFD-like adamantinoma which was treatedby limbsalvage surgery withcurettage,bone graft application, ORIFplateandscrew.OFD-like Adamantinomaand osteofibrousdysplasiahadsimilarhistopathologypattern,pathol- ogistmustbeawaretoperformimmunohistochemicalstainingfor keratin particularlywhen thehistopathological featureof osteofibrous dysplasia shows small nestsof tumorcells withinthe fibrousstroma.AlthoughtheincidenceofOFD-likeadamantinoma islow,itisimportanttorecognizethisrarebonetumour.Further- more,todifferentiatebetweenosteofibrousdysplasiaandOFD-like Adamantinomastilldiagnosticchallengingandrequiremultidisci- plinaryapproach.

Conﬂictsofinterest

Theauthorsreportnodeclarationsofinterest.

Funding

The authorsreportnoexternal sourceoffundingduringthe writingofthisarticle.

Ethicalapproval

Ethicalapprovalwasnotrequiredinthiscasereport.

Consent

Writteninformedconsentwasobtainedfromthepatientfor publicationofthiscasereportandaccompanyingimages.Acopy ofthewrittenconsentisavailableforreviewbytheEditor-in-Chief ofthisjournalonrequest.

Authorcontribution

Achmad Fauzi Kamal contributes in the study concept or design,datacollection,analysisandinterpretation,oversightand

leadershipresponsibilityfortheresearchactivityplanningandexe- cution,includingmentorshipexternaltothecoreteam.

FahmiAnshoricontributestothestudyconceptordesign,super- visingandcriticallyreviewthemanuscript.

EvelinaKodratcontributestothestudyconceptordesign,data collectionandwritingthepaper.

Registrationofresearchstudies Notapplicable.

Guarantor

AchmadFauziKamal isthesoleguarantorof thissubmitted article.

Provenanceandpeerreview

Notcommissioned,externallypeer-reviewed Acknowledgement

Noconﬂictofinterestregardingforthepublicationofthispaper.

References

[1]B.Sarisozen,K.Durak,C.Ozturk,Adamantinomaofthetibiaina nine-year-oldchild,ActaOrthop.Belg.68(4)(2002)412–416.

[2]H.Nouri,H.Jaafoura,M.Bouaziz,etal.,Dedifferentiatedadamantinoma associatewithﬁbrousdysplasia,Orthop.Traumatol.Surg.Res.97(2011) 770–775.

[3]B.Czerniak,DorfmanandCzerniak’sbonetumors,in:DorfmanandCzerniak’s BoneTumors,2nded.,Elsevier,2015,pp.1142–1170p.

[4]K.K.Unni,C.Y.Inwards,Dahlin’sBoneTumors,6thed.,LippincottWilliams&

Wilkins,2009,halamanpage.

[5]B.C.Gleason,B.Liegl-Atzwanger,H.P.Kozakewich,etal.,Osteoﬁbrous Dysplasiaandadamantinomainchildrenandadolescents:aclinicopathologic reappraisal,Am.J.Surg.Pathol.32(2008)363–376.

[6]J.Wang,R.Boyle,S.McCarthy,etal.,OsteoﬁbrousDysplasia-like adamantinoma:acasereport,Pathology.50(Supplement1)(2018)S129.

[7]R.N.Kim,G.Ahn,G.-W.Kim,H.Y.Cho,Y.H.Oh,D.-H.Chung,Osteoﬁbrous Dysplasia-likeadamantinoma-acasereportswithitsimmunohistochemical andultrastructuralstudies,KoreanJ.Pathol.38(2004)50–55.

[8]D.W.Scholﬁeld,Z.Sadozai,C.Ghali,etal.,DoesOsteoﬁbrousDysplasia progresstoadamantinomaandhowshouldtheybetreated?BoneJtJ.99-B (2017)409–416.

[9]A.Ratra,A.Wooldridge,G.Brindley,Osteoﬁbrousdysplasia-like

adamantinomaofthetibiaina15-Year-Oldgirl,Am.J.Orthop.44(10)(2015) E411–3.

[10]B.R.Southam,A.H.Crawford,D.A.Billmire,J.Geller,D.VonAllmen,A.P.

Schumaier,etal.,Long-termfollow-upofadamantinomaofthetibia complicatedbymetastasesandasecondunrelatedprimarycancer:acase reportandliteraturereview,CaseRep.Orthop.2018(2018)1–9.

[11]D.Jain,V.K.Jain,R.K.Vasishta,P.Ranjan,Y.Kumar,Adamantinoma:a clinicopathologicalreviewandupdate,Diagn.Pathol.3(1)(2008).

[12]S.Bethapudi,D.A.Ritchie,E.Macduff,J.Straiton,Imaginginosteoﬁbrous dysplasia,osteoﬁbrousdysplasia-likeadamantinoma,andclassic adamantinoma,Clin.Radiol.69(2)(2014)200–208.

[13]H.Nouri,H.Jaafoura,M.Bouaziz,M.Ouertatani,L.Abid,M.H.Meherzi,etal., Dedifferentiatedadamantinomaassociatedwithﬁbrousdysplasia,Orthop.

Traumatol.Surg.Res.97(7)(2011)770–775,http://dx.doi.org/10.1016/j.otsr.

2011.05.005[Internet]Availablefrom:.

[14]G.P.nielsen,P.C.W.Hogendoorn,AdamantinomaoflongboneinWHO classiﬁcationoftumor,in:SoftTissueandBoneTumor,5thed.,IARC,2020, pp.463–466.

[15]R.A.Agha,T.Franchi,C.Sohrabi,G.Mathew,fortheSCAREGroup,TheSCARE 2020guideline:updatingconsensusSurgicalCaseReport(SCARE)guidelines, Int.J.Surg.84(2020)226–230.

OpenAccess

ThisarticleispublishedOpenAccessatsciencedirect.com.ItisdistributedundertheIJSCRSupplementaltermsandconditions,which permitsunrestrictednoncommercialuse,distribution,andreproductioninanymedium,providedtheoriginalauthorsandsourceare credited.