Rev. bras. ortop. vol.52 número2

rev bras ortop.2017;52(2):238–240

SOCIEDADE BRASILEIRA DE ORTOPEDIA E TRAUMATOLOGIA

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Case

report

Pindborg

tumor

in

the

distal

femur

夽

Alex

Oliboni

Sussela

_,

_Paulo

_Ricardo

_Picon

_Alves

_,

_Vinicius

_Duval

_da

_Silva

_,

Carlos

Daniel

de

Garcia

Bolze

_,

_Osvaldo

_André

_Serafini

a_{PontifíciaUniversidadeCatólicadoRioGrandedoSul,FaculdadedeMedicina,PortoAlegre,RS,Brazil}

b_{UniversidadeCatólicadoRioGrandedoSul,HospitalSãoLucas,Servic¸odeOrtopediaeTraumatologia,PortoAlegre,RS,Brazil}

c_{UniversidadeCatólicadoRioGrandedoSul,HospitalSãoLucas,LaboratóriodeAnatomiaPatológicaeCitopatologia,PortoAlegre,RS,}

Brazil

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Articlehistory: Received20May2016 Accepted30May2016

Availableonline20February2017

Keywords: Femur

Boneneoplasms Biopsy

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b

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TodescribeacaseofpossiblediagnosisofPindborgtumoronthedistalfemur.

A32-years-oldfemalepatient,anativeofBolivia,residentinBrazil,arrivedtothisservice fortumorresearchintherightfemur.Afterbiopsiesandresectionofthelesion,thecasewas referredtoanalysisandconsultancyintheUnitedStates.Inareportofreviewbythe Pathol-ogyLaboratory,itwascharacterizedthehistologicalappearanceandimmunohistochemical profilewerecharacteristicofPindborgtumor.Currently,thepatientisbeingfollowed-upat theOrthopedicsandTraumatologyDepartmentofthisinstitution,andpresentsagood evo-lution.Thisstudypresentsthecaseofapatientwithararetumorthatwasinvestigatedfor anextensiveperiodandthroughmanytests.Pindborgtumorwassuggestedasadiagnostic hypothesisduetothecharacteristicsandbehavioroftheneoplasticlesion.Althoughthis lesionismorecommonlyobservedinodontology,theneoplasiawascompatiblewiththe diagnosis.Therefore,despitethefactthatthistumorhasbenigncharacteristics,long-term monitoringisnecessary,giventhehighrateoftumorrecurrence.

©2017PublishedbyElsevierEditoraLtda.onbehalfofSociedadeBrasileiradeOrtopedia eTraumatologia.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http:// creativecommons.org/licenses/by-nc-nd/4.0/).

Tumor

de

Pindborg

em

fêmur

distal

Palavras-chave: Fêmur

Neoplasiasósseas Biópsia

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e

s

u

m

o

DescreverumcasodepossíveldiagnósticotumordePindborgemfêmurdistal.

Apacientede32anos,naturaldaBolívia,residentenoBrasil,veioaesseservic¸opara investigac¸ãodemassatumoralemfêmurdireito.Apósfeituradebiópsiaseressecc¸ãoda lesão,ocasofoiencaminhadoparaanáliseeconsultorianosEstadosUnidos.Emlaudode revisãodoLaboratóriodePatologia,foicaracterizadoqueoaspectohistológicoeoperfil

夽

StudyconductedatUniversidadeCatólicadoRioGrandedoSul,HospitalSãoLucas,PortoAlegre,RS,Brazil. ∗ _{Correspondingauthor.}

E-mail:alexoliboni@gmail.com(A.O.Sussela). http://dx.doi.org/10.1016/j.rboe.2017.02.002

rev bras ortop.2017;52(2):238–240

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imuno-histoquímicoeramcaracterísticosdetumordePindborg.Atualmente,apacienteé acompanhadanoServic¸odeOrtopediaeTraumatologiadanossainstituic¸ãoeapresenta boaevoluc¸ão.Orelatodescreveocasodeumapacientecomumaneoplasiararaquefoi investigadapormuitotempoecomauxíliodemuitosexames.OtumordePindborgfoi sugeridocomhipótesediagnósticadevidoàscaracterísticaseaocomportamentoneoplásico dalesão.Adespeitodeserumalesãomaiscomumenteobservadanoâmbitoodontológico,a neoplasiadapacientemostrou-secompatívelcomodiagnóstico.Assim,mesmosetratando deumtumorcomcaracterísticasbenignas,háanecessidadedeacompanhamentoporlongo tempo,hajavistaoaltograuderecorrênciadotumor.

©2017PublicadoporElsevierEditoraLtda.emnomedeSociedadeBrasileirade OrtopediaeTraumatologia.Este ´eumartigoOpenAccesssobumalicenc¸aCCBY-NC-ND (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction

Pindborgtumor,alsoknownascalcifyingodontogenic epithe-lialtumor,isahighlyrareneoplasm,characterizedbylocal invasivenessandpresentingamyloidmaterial.Thisneoplasm emergesasapainlessmassofslowgrowth,withno predilec-tionforgender. Itaffects patientsbetweenthesecond and sixth decadeoflife,mainly inthe fourthdecade.Thevast majorityofthesetumorsare intraosseousmasses;only6% of them are extraosseous. Upon radiological examination, thetumorisusuallymultilocularandseptatedor,less com-monly,unilocularandradiolucent,andcalcificationswithin thelesionaresometimesobserved.

Case

report

A 32-year-old female patient, originally from Bolivia, was admittedtothishospitalinAugust2009withacomplaintof painintherightkneeforoverayear.Shedeniedprevious sur-geriesonthearea,aswellasusinganymedications.Patient presentedwithrestrictedmobilityoftherightlowerlimband painuponpalpation inthe regionofthe rightfemoral epi-condyle.Onthe13th ofthe samemonth,shewasreferred to a surgical procedure – femoral bone biopsy. The speci-men,whichmeasured1.6cm,waspositiveforamyloidusing Congoredstaining.Inascintigraphy,performedonAugust 19,anirregularradiomarkerconcentrationwasobservedin thedistalthirdoftherightfemur.Anewbiopsy,performed onNovember9th,showedbonefragmentwithdense atypi-calcellinfiltrateandpositivityforamyloidusingCongored staining.Intheradiologicalexamination,aninsufflating oste-olyticlesionwasidentifiedintheinnermarginofthedistal endofthefemur,withseptationsinitsinterior.Withthe sus-picionoflargecelltumor,patientunderwenttumorresection surgeryonDecember21,2009.Microscopicexaminationofthe surgicalspecimenfoundatumoralmasspartiallycoatedby adiposeandmuscletissue,measuring6.5×4.1×3.4cm.When dissected,thelesionwascharacterizedbyits whitish,firm, andmattecolor.Partofthedistalportionofthefemurand tumorwereresected.Patienthadagoodrecoveryaftersurgery. TissuematerialwassenttotheHSLPathologyLaboratory. Ini-tialdiagnosis was unclassifiedneoplasm; subsequently, an immunohistochemicalanalysiswasperformed,whichledto a diagnosis compatible with mesenchymal neoplasm with

Fig.1–Post-surgeryradiographofthelesionindicatingthe presenceofsurgically-insertedorthopediccementmaterial.

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Fig.2–Post-surgicalrecoverylimbflexion.

Fig.3– Post-surgicalrecoverylimbextension.

Discussion

Described in 1958by Jens JorgenPindborg, the tumor that bears hisname isavery rare neoplasm.There are around 200casesdescribedinthecurrentliterature.1_{Descriptionof} this neoplasm shares many features with ameloblastoma, butPindborgtumorislessaggressiveandpresentsaslower growth.2_{Itisknownthatthistypeoftumorrequiresalong} follow-upperiod,becausethereisahighriskoftumor recur-renceifithasbeenincompletelyresected.Accordingtothe literature,tumorrecurrencerateisaround15%;inthese,the frequencyoflesionsthatweretreatedwithcurettageishigh. Inspiteofpresentinghighmitoticactivity,acharacteristicof malignancy,Pindborgtumorisabenignneoplasiawithgood prognosis.3,4_{Thehistologicalaspectsofthisneoplasiainclude} fibrousstroma,islandsofpolyhedralepithelialcells, homoge-neousamyloidcontent,eosinophilia,andpositivereactionto Congoredstaining.Insomecases,itispossibletofindfocal areasofclearcells,calledrarevariantsofclearcells,which presentalessfavorableprognosisforthepatient.3,5_Treatment forthis typeofneoplasia consistsofsurgicalremovalwith sufficientsafetymargintopreventrecurrences.1

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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1.PindborgJJ.Acalcifyingepithelialodontogenictumor.Cancer. 1958;11(4):838–43.

2.DahlinDC.Bonetumors–generalaspectsanddataon6221 cases.3rded.Springfield,IL,USA:CharlesC.Thomas;1978. 3.MüllerD,Manojlovi´cS,Luksi´cI,Grgurevi´cJ.Calcifying

epithelialodontogenictumorofthemaxilla(Pindborgtumor). CollAntropol.2012;36Suppl.2:205–8.

4.TakataT,SlootwegPJ.Calcifyingepithelialodontogenic tumour.In:BarnesL,EvesonJW,ReichartP,SidranskyD, editors.Pathologyandgenetics–headandnecktumours. Lyon:IARCPress;2005.p.302–3.