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RheumatologyMain101-110Q1A10-year-oldwhiteboyisbroughttotheofficebyhisfosterparentsfortheevaluationofseverepaininhisleftkneeforthelastfewdays.Hisleftkneehasbeenhurtingsobadlythatheisnowunabletoplaywithhispeers.Thepainiscontinuousandnon-radiating.Hiskneeappearsswollen,buthedeniesanyhistoryoftrauma.Hisfamilyhistoryisunknown.Hisvitalsignsarestable.Physicalexaminationrevealsapaleyoungboy.Theleftkneeistender,erythematousandswollen.Bloodtestsreveal:X-rayofthekneerevealsthatthedistalfemurhasacentrallyticlesion,onionskinning,andamoth-eatenappearancewithsomeextensionintothesofttissue.Whatisthemostlikelydiagnosis?A.OsteomyelitisB.EwingssarcomaC.ChondrosarcomaD.GlomustumorE.FibrosarcomaA1Correctanswer:BEwing'ssarcomaisahighlymalignanttumorthatisfoundinthelowerextremitymorethantheupperextremity.Themostcommonsitesarethemetaphysisanddiaphysisofthefemurfollowedbythetibiaandhumerus.Thetumorisveryaggressiveandmetastasizesearlytothelungsandlymphnodes.Patientsaretypicallywhitemalesintheirfirstorseconddecadeoflife.Theclinicalpresentationincludespainandswellingforweeksormonths.Erythemaandwarmthofthelocalareaaresometimesseen.Patientsmayinitiallybediagnosedwithosteomyelitis,basedonthemisleadingpresentationofintermittentfevers,leukocytosis,anemia,andanincreasedESR.Nevertheless,Ewing'ssarcomacanbedistinctlycharacterizedradioiogicallywithitslameiiatedappearanceoronionskinperiostealreaction.Thislesionisusuallylytic,central,andaccompaniedbyendostealscalloping.Theonion-skinappearanceisoftenfollowedwithamoth-eatenormottledappearanceandextensionintosofttissue.Thetreatmentincludessurgery,radiation,andmulti-drugchemotherapy.RadiationorchemotherapyIsusedpreoperatively.(ChoiceA)Osteomyelitisisusuallymistakeninsuchascenariosinceitalsopresentswithfever,malaise,localjointpain,andswelling.X-rayfindingsinchronicosteomyelitisshowacentrallyticbonedefectwithsurroundingsclerosistermedasBrodie'sabscess.(ChoiceC)Chondrosarcomaasaprimarytumorisnotverycommon.Itoccursinthefifthorsixthdecadeoflife.Peoplewithdisorderslikemultipleenchondromasandhemangiomasareatahigherriskthanthegeneralpopulation.X-raytypicallyrevealsafusiformdefectwithscalloping.(ChoiceD)Glomustumorisacommonvascularbenigntumorcharacterizedbythetriadofsevereintermittentpain,tenderness,andsensitivitytotouch.Themostcommonsiteissubungualin70%ofthecases;theothersitesarethepalmandwrist.Theseareusuallyfoundinthefourthdecade,andaffectfemalesmorethanmales.(ChoiceE)Fibrosarcomaisamalignantspindlecellneoplasmfoundmostlyinpatients30-60yearsofage.Theclinicalpresentationisusuallyapainfulmass.Theradiologicalpictureisthatofanosteolyticlesionwhosemarginscanbewell-definedorraggedandmoth-eaten.EducationalObjective:Ewing'ssarcomaisoftenconfusedwithosteomyelitis;however,characteristicx-rayfindingscanpointtothediagnosis.Q2A16~year-oldpreviouslyhealthyboyIsbroughttotheofficeforevaluationofa2-monthhistoryofleftlegpain.Hehashaddull,aching,painovertheleftthighthatoccursmostlyatnightbutisimprovedwithibuprofen.Thepatienthashadnotrauma,fever,orpaininotherjointsandextremities.Heplaysbasketballinhighschoolandreportsthatthepainisnotrelatedtophysicalactivity.Examinationshowsnoredness,deformity,orfocaltenderness.Hislegx-rayisshownbelow.Whichofthefollowingisthemostlikelydiagnosis?A.EwingsarcomaB.OsteoidosteomaC.OsteomyelitisD.OsteosarcomaE.StressfractureA2Correctanswer:BThispatient'sclinicalpresentationandhypodenselesiononx-rayareconsistentwithanosteoidosteoma,abenignbone-formingtumorthatoccursmostofteninadolescenceandearlyadulthoodandismorecommoninmales.Osteoidosteomamostcommonlyaffectstheproximalfemurbutcanalsoinvolveotherlongbonesandthespine.Patientsdevelopincreasingpainthatworsensatnightwithoutrelationtophysicalactivity.Thepainistypicallyrelievedwithnonsteroidalanti-inflammatorydrugs(NSAIDs),andthisisstronglysuggestiveofthediagnosis.Childrenmaydevelopfocaltenderness,deformities,orswelling.However,adolescentsoftenhavenofocaifindingsonexamination.Plainx-raysofthelegshowthetypicalsmall,roundSucencywithscleroticmarginsandsometimescentralossification.NSAIDscanbeprescribedforsymptomaticrelief,andpatientsshouldbefollowedwithserialexaminationandx-raysevery4-5monthstomonitorthelesion.Surgicalresectionistypicallyreservedforpatientswithrefractorysymptoms.Mostlesionsspontaneouslyresolveoverseveralyears.(ChoiceA)Ewingsarcomapresentsinthelongbonesoftheextremitieswithpainformanyweeks/months,swelling,anddeformity.Theserarelesionsoftenhaveextensiveinvolvementoftheaffectedbone,andmaydemonstratemultiplelayersofnewsubperiostealboneformation.(ChoiceC)Osteomyelitisoftenpresentswithsystemicsymptoms(eg,fever),focalinflammation(eg,warmth,swelling,pain),anddecreasedfunction.Theabsenceofwarmthandswellingonexaminationinthispatientmakesthislesslikely.(ChoiceD)Osteosarcomatypicallypresentswithchroniclocalizedpain.Examinationusuallyshowsapalpablemass,andx-raysshowdestructionofthenormalbonepatternwithindistinctmarginsandamixtureofradiodenseandradiolucentareas.(ChoiceE)Stressfractureusuallypresentswithfocaltendernessthatworsenswithactivityanddecreaseswit