Rev. bras. ortop. vol.49 número4

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Case

Report

Calcaneal

chondrosarcoma:

a

case

report

夽,夽夽

Frederico

Barra

de

Moraes

_,

_Nicolle

_Diniz

_Linhares,

Pryscilla

Moreira

de

Souza

Domingues,

Vanessa

Nogueira

Machado

Warzocha,

Jefferson

Martins

Soares

FaculdadedeMedicinadaUniversidadeFederaldeGoiás,Goiânia,GO,Brazil

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Articlehistory:

Received23April2013 Accepted9October2013 Availableonline14May2014

Keywords:

Chondrosarcoma Calcaneus Boneneoplasms

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Theobjectivewastoreportonararecaseofchondrosarcomaofthebonesofthefoot,and specificallythecalcaneus.Thepatientwasa30-year-oldwomanwithacomplaintof pain-lessnodulationonthelateralfaceofthecalcaneus,whichshehadhadforaroundeight years,whichthenstartedtopresentsignificantpain.Radiographyshowedatumorwith impreciselimits,compromisingthecalcaneus,talusandlateralmalleolus.Thelesionhad adestructive,aggressiveandosteolyticappearance,withinvasionofthesurroundingsoft tissuesandthepresenceofpointsofcalcification.Amputationwasperformedinthe mid-dlethirdoftherightlowerleg,withahistologicaldiagnosisofchondrosarcoma.Thecase evolvedwithlocalrecurrenceofthetumorformationandsubsequentamputationinthe middlethirdoftherightthigh.Oneyearafterthesecondamputation,thepatientevolved withmetastasisfromthechondrosarcomainsofttissuesthroughoutthebodyandinthe lungs,andshediedoneyearandtenmonthsafterthediagnosiswasmade. Chondrosarco-masthatinvolvethecalcaneusarerareinyoungadults,withfewreportsintheliterature. Forthisreason,thepresentreportbecomesimportantinthatitshowsthateventhough thisisararecondition,itispresentinourenvironment.Earlyinvestigationisessential, especiallybymeansofimagingexaminations,withtheaimofdiminishingthechancesof malignanttransformationandconsequentcomplications,soastoavoiddeath.

©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublishedbyElsevierEditora Ltda.Allrightsreserved.

Condrossarcoma

de

calcâneo:

relato

de

caso

Palavras-chave:

Condrossarcoma Calcâneo Neoplasiasósseas

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Relatarumcasorarodecondrossarcomadosossosdopé,maisespecificamentenocalcâneo. Mulherde30anos,comqueixadenodulac¸ãoindolornafacelateraldocalcâneohaviacerca deoitoanos,queinicioucomdorimportante.Aradiografiamostrouumtumordelimites imprecisos,quecomprometeuocalcâneo,otáluseomaléololateral.Alesãoapresentou aspectodestrutivo,agressivoeosteolítico,cominvasãodepartesmolescircunvizinhase

夽_{Pleasecitethisarticleas:deMoraesFB,LinharesND,deSouzaDominguesPM,WarzochaVNM,SoaresJM.Condrossarcomadecalcâneo:}

relatodecaso.RevBrasOrtop.2014;49:409–413.

夽夽

WorkperformedintheDepartmentofOrthopedicsandTraumatology,HospitaldasClínicas,SchoolofMedicine,UniversidadeFederal deGoiás,Goiânia,GO,Brazil.

∗ _{Correspondingauthor.}

E-mail:[email protected](F.B.deMoraes).

2255-4971/$–seefrontmatter©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublishedbyElsevierEditoraLtda.Allrightsreserved.

teriormente,amputac¸ãonoterc¸omédiodacoxadireita.Apósumanodaúltimaamputac¸ão, apacienteevoluiucommetástasedecondrossarcomaempartesmolespelocorpoenos pul-mõesefaleceuumanoedezmesesapósodiagnóstico.Oscondrossarcomasqueenvolvem ocalcâneoemadultosjovenssãoraros,compoucosrelatosnaliteratura.Poressemotivo, opresenterelatotorna-seimportantenosentidodemostrarqueapesardeessaseruma afecc¸ãorara,aindaassimelaestápresentenonossomeio.Éimprescindívelsuainvestigac¸ão precoce,principalmenteporexamesdeimagem,comointuitodediminuiraschancesde malignizac¸ãoe,consequentemente,decomplicac¸õeseevitaroóbito.

©2014SociedadeBrasileiradeOrtopediaeTraumatologia.PublicadoporElsevier EditoraLtda.Todososdireitosreservados.

Introduction

Sarcomasareraremesenchymatousneoplasms(lessthan1% ofall types ofcancer) that arise inmuscles, endothelium, cartilageandsupportstructures.Theincidenceofmalignant sarcomasislow:1.5per100,000inhabitants.Thisincidence results in approximately 8000 new cases per year in the UnitedStates.1_{Sarcomasgenerallyhavemesodermalorigin,}

althoughsomearederivedfromtheneuroectoderm,andthey aredividedintotwogroups:boneandsoft-tissuesarcomas.It shouldbenotedthatapproximately3%ofbonetumorsoccur inthefootorankle.2,3

Chondrosarcomasare tumorsthatdevelopduring adult-hood and old age, with maximum incidence between the fourthand sixth decades oflife.They accountfor approx-imately 20–25% ofbone sarcomas, but involvement ofthe calcaneus israre. They preferentially affect the flat bones, especiallythescapularandpelvicbelts,butmayalsoaffect the diaphyseal parts of the long bones. They may arise primarily or secondarily, as malignant transformation of enchondromasor,morerarely,fromthecartilaginouscover ofosteochondromas.2,3

Chondrosarcomashavean indolent naturalhistory, and patientstypicallypresentwithpainandtumefaction. Radio-logically, the lesion may have a lobular appearance, with mottled,spottyorring-likecalcificationofthecartilaginous matrix.Itisdifficulttodistinguishlow-gradechondrosarcoma frombenignlesionsthroughradiographsorhistological exam-ination.Forthisreason,clinicalhistory-takingandphysical examinationarefundamentalstepsinthediagnostic reason-ing.

Theobjectivesofthiscasewere thereforetoreportona rarecaseofchondrosarcomaofthecalcaneusinayoungadult patientwithseveralcomplications,andtoreviewthe litera-tureonthissubject.

Case

report

Thepatientwasa30-year-oldwhitewomanwhowasassessed inthe outpatientclinic ofHospital das Clínicas, School of MedicineoftheFederalUniversityofGoiás,withahistoryofa sprainedrightanklethatshehadsufferedapproximatelyeight yearsearlier,withsubsequentappearanceofpainless nodula-tiononthelateralfaceofthecalcaneus.Thetumorformation

had gradually increasedinvolumeand the patient started topresentapainfulcondition.Forthisreason,atahospital elsewhereinthesamestate,thetumorwasresected.However, completehealingwasnotachievedandtheoperativewound continuedtobeopen,withactivesecretion.

On physicalexamination,the rightfoot and ankle were seen to bevery swollen, with tenseand shiny skin. There weretwofistulasinthelateralregionofthecalcaneus,with yellowish secretion. The foot was in the equinus position andwaspainfulonpalpation.Radiographyshowedatumor withimpreciselimitsthatcompromisedthecalcaneus,talus and lateral malleolus. The lesion presented a destructive, aggressiveandosteolyticappearance,withinvasionofthe sur-roundingsofttissuesandpresenceofpointsofcalcificationin thetumorformation(Fig.1).

The initial chest radiograph had a normal appearance. Macroscopically, thetumortissuepresentedastraw-yellow color,and anatomopathologicalexaminationindicatedthat thiswasachondrosarcoma.Onemonthaftertheinitial con-sultation,amputation wasperformedintheproximalthird oftherightfoot,buthealingoftheoperativewounddidnot takeplaceandtherewaseliminationofnecroticmaterialand yellowish secretion. Radiography ofthe amputation stump showedanosteolyticlesionintheamputatedtibia,andthis was considered tobe recurrencein theamputation stump (Fig.2).

Six months after the first amputation, the patient was admitted tothe hospital. Achondrosarcoma was observed in the amputation stump of the tibia and a new amputa-tion was performed inthe middle thirdof the right thigh (Fig.3A).Oneyearlater,thepatientreturnedtotheoutpatient clinicwithacomplaintofdyspnea,withacompromised gen-eralconditionandnodulationofrubberyconsistencyoverthe entirebody.Biopsiesontheselesionsrevealedthepresenceof soft-tissuemetastasisfromthechondrosarcoma.Chest radio-graphyrevealedmetastaticnodulationinbothlungs(Fig.3B). Thepatientdiedlaterintheweekofthereturnvisit, approxi-matelyoneyearandtenmonthsafterthediagnosis.

Discussion

Fig.1–Radiographsoftherightfootandankleinanteroposteriorview(A)andlateralview(B),showinglesionof

destructive,aggressiveandosteolyticappearance,withinvasionofthesurroundingsofttissuesandthepresenceofpoints ofcalcificationinthetumorformation.

Fig.2–Radiographsoftheamputationstumpinanteroposteriorview(A)andlateralview(B),showinganosteolyticlesion intheamputatedtibia.

intramedullaryandconventionaljuxtacorticalvariants,with clear,undifferentiatedandmesenchymalcells,and90%areof conventionalchondrosarcomatype.4

They generally develop as intramedullary lesions and affectthescapularandpelvicbeltsmorefrequentlythanthe feet.Amongthechondrosarcomasaffectingthebonesofthe foot,those that affect the calcaneus are more common.5,6

They may be primary or secondary and, in the latter case,theyrepresentmalignanttransformationof enchondro-masorosteochondromas.Furthermore,theyare commonly associatedwiththe hereditarysyndrome ofmultiple exos-tosis in 6% of the cases.7,8 _{Since osteochondromas are}

commonlyasymptomatic, the patients are unaware ofthe existenceofthelesion,such thatsudden pain andrapidly growing pain are the clinical manifestations of suspected malignancy,alongwithfocalcompressiveneuropathy.9 _The

processofmalignanttransformationoccursatafrequencyof 5%.7

Chondrosarcomas are the second commonest type of boneneoplasmamongthemalignantprimarybonetumors. The behavior of these tumors is variable, ranging from a slow-growth form with little possibility of metastasis, to an aggressive sarcomatous form with a high likelihood of metastatic transformation. They affect males and females equally,withoutanypreferentialage.Theyarepredominantly observedinindividualsbetweentheirfifthandsixthdecades oflifeandaremorefrequentlyseenintheappendicular skele-ton.Clinically,theyarecharacterizedbyinsidious,progressive andlong-durationpain.

Thediagnosisofcalcanealchondrosarcomaismade radio-logically.Thus,severalmethodsareusedfordetectingthese neoplasms, such as conventional radiography, computed tomography (CT) and magneticresonance imaging (MRI).10

Fig.3–Anteroposteriorradiographoftherightthighshowingamputationinthemiddlethirdofthefemur(A). Posteroanteriorradiographofthechestshowingmetastaticnodulationinbothlungs(B).

“popped”,spottyorring-likecalcificationofthecartilaginous matrix.11 _{One good option for following up patients who}

presentsometypeofbonetumoralterationistouseserial radiographsinordertostudy malignant transformation.In these,itmaybepossibletodifferentiatebetweenlesionswith well-definedandindistinctmargins,ortonotethatthelesion increasesinsize,ortonotethatitsmineralizationtakeson the appearance ofopaque glass.7 _{Radiography ofthe right}

footofourpatientshowedatumorwithimpreciselimitsthat compromisedthecalcaneus,talusandlateralmalleolus.The lesionhadadestructive,aggressiveandosteolyticappearance, withinvasionofsurroundingsofttissuesandthepresenceof pointsofcalcificationinthetumorformation.

Relative radiolucency inside a previously mineralized regionofthecartilaginouscoveralsoimpliesthepossibility ofsarcomatousdegeneration.Althoughthethicknessofthe demineralizedcartilaginouscoverisgenerallylessthan1cm inbenignosteochondromas,it isgenerallymorethan 2cm whenmalignant transformationoccurs.Evidence regarding thethicknessofthecartilaginouscoverisgenerally,butnot always, areliable sign ofmalignity orbenignity. Themore radiotransparentthetumoris,thehigherthelikelihoodthat itwillbeofhigh-grade.Slow-growinglow-gradetumorscause reactivethickeningofthecortex,whilehigh-gradeneoplasms thataremoreaggressivedestroythecortexandforma soft-tissuemass.7

CT is an excellent auxiliary method for diagnosing osteosarcomasandchondrosarcomas.Itprovidessignificant improvementindeterminationsofthemorphological mod-ificationsresultingfrombenignormalignantdisease,along withhigh-qualityimageswithexcellentanatomical resolu-tionandreducedlevelsofartifacts.10_{Inmanystudies,CThas}

presentedveryhigh sensitivityfordetectingmorphological alterationscausedbyneoplasia(between80%and100%),and

hasshowntheprimaryoriginofthelesions,withexcellent viewingofradiopaqueareasandthenumberofneoplasticfoci present.10_{Thishighsensitivityratehasbeendemonstratedby}

thecapacitythatCThasforshowingtheextentofthelesion, itsdepthandtheinvolvementoftheadjacentsoftandhard tissues,withreducedlevelsoftechnicalartifacts.Thus,the radiographpatternofchondrosarcomas,observedusingCT, mayhelpandfacilitatethefinalhistopathologicaldiagnosis, throughtheindividualcharacteristicsoftheselesions,which maybeidentifiedandanalyzedthroughqualitativestudies. Moreover,CTisasafemethodfordeterminingthe morpho-logicalalterationscausedbymalignantandbenignneoplasia andshowstherelationshipbetweenthelesionandthe adja-centtissues,andalsothedegreeofinfiltrationanddepthof the tumormass. Inthismanner,the prognosiscanbe bet-terestablishedandtherapythatismoreappropriatecanbe administered.10

Totreatchondrosarcomas,itisimportanttoseparatethem intotwomajorgroupsseeninimagingexaminations:localized and aggressive lesions.Localized lesions are oflow histo-logical grade,withlittlepossibilityofmetastasis.Thus,the treatment consists of wide resection of the lesion, which makesitpossibletoconservetheboneadjacenttothetumor.9

In aggressivecases,thehistology pointstoward intermedi-ate andhigh-gradetumors, whichare treatedbymeansof amputation.12

Inthepresentcase,itwasdecidedtoamputatethelimb, butwiththedevelopmentofeffectivechemotherapy,salvage surgeryonthelimbhasbecomethepreferredtreatmentfor primarybonesarcomas.Currently,90%ofthepatientswith sarcomasinlimbextremitiescansuccessfullyundergo sal-vagesurgeryonthelimb.13_{Nonetheless,itisalsoworthnoting}

thatthere arestill greatdoubtsin relationtolimbsalvage surgeryforthefoot,giventhatbecausethefoothasa compart-mentalizedstructure,it enablesearlyinvolvementofbones andtheadjacentsofttissues,whichleadsthegreatmajorityof physicianstochoosetoamputatethelimb.However, depend-ingonthesizeandlocationofthetumorinthefoot,surgical marginsmaybeattainedincalcanealtumorswhenthe neu-rovascularstructuresofthetarsaltunnelremainunimpaired. Suchsituationsprovidetheoptionoftumorresectionand sub-sequentreconstructionandsalvageofthelimb,usingbone, muscleandskingrafts,therebyensuringthatthepatient con-tinuestohaveabodypartthatisessentialforphysiological locomotion.

Survivalwilldependonachievingcompletecontrolover theprimaryneoplasmsothatspreadingintoneighboring tis-suesandoccurrencesofmetastaticfociare avoided.Inthis regard,thefirstsurgicalinterventionshouldhavethemain aimofperformingresectionthatiswideenoughtoprevent localrecurrence.Forthistobeachieved,surgicalmarginsof 4cm onall sidesarenecessary, thereby leadingtocurefor almostallpatientsandresultingin10-yearsurvivalof97%.7

Wideresection ofcasesofchondrosarcoma ofthe thoracic wallisadvocatedbysomeauthors becauseofthedifficulty inmakingapreoperativehistopathologicaldiagnosis,thereby representingaformofprophylaxisagainstfuture complica-tions suchasmetastases.14 _{Inthis sense, the firstsurgical}

intervention madein the patient of the casepresented in thisstudydoesnotseemtohavebeenthebesttherapeutic approach,giventhatthelesionrecurredandthatpulmonary metastasissubsequentlyoccurred.Thiscorroboratestheidea thatwideintervention,althoughconsideredtoberadicalby someauthors,isinmany casesthebesttherapeuticoption andhasaninfluenceonthepatient’ssurvival.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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