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metastasisatthetimeofpresentationis0%.75Treatmentdependsonthestageandlocalextent
ofthetumor.Localizedtumorsaretreatedwithchemotherapyandwidesurgicalexcisionwith
or without radiation.Radiationmay be avoided if adequate margins canbe achieved atthe
timeofsurgery.Radiationcarriestheriskofsecondarysarcomaintheradiatedfield.Forthis
reason,amputationmaybetheprocedureofchoiceforthistumorinthefoot.
Lymphoma
IntroductionandDefinition
Primarylymphomaofbone(PLB)isararetumorthatcomprisesapproximately5%to7%of
malignantbonetumors.Secondaryboneinvolvementisseeninabout16%to20%ofpatients
with widespread lymphoma. Most cases of PLB are non-Hodgkin, diffuse large B-cell
lymphomas.Thereisalackofconsensusontheoptimaltreatment,whichgenerallyincludes
combined radiation and multiagent chemotherapy. Overall prognosis for PLB is generally
good.SurgeryisnotusedtocurePLB,butonlytostabilizeweakenedbonesortreatpathologic
fractures. Approximatelytwo-thirds of patients with PLB require surgery, most because of
pathologicfractures.
76
Lymphomainvolvingbonecanbeseparatedintofourgroups:
Asingleskeletalsite,withorwithoutregionallymphnodeinvolvement;
Multiplebonesareinvolved,butthereisnovisceralorlymphnodeinvolvement;
Patientspresentwithabonetumor,butworkupshowsinvolvementofothervisceralsites
ormultiplelymphnodesatmultiplesites;
Thepatienthasaknownlymphomaandabonebiopsyisdonetoruleoutinvolvementof
bone.
Groups1and2areconsideredbymanytobePLB,butthereisalackofconsensus.
77
IncidenceandDemographics
Themedianageatpresentationforsingle-bonePLBis44,andforpolyostoticPLBthemedian
ageis64.Themostcommonlocationistheappendicularskeleton,especiallythefemur and
tibia.PLBinthefootisrare,buthasbeenreportedinthecalcaneus,metatarsal,andtalus.
X-rayAppearanceandAdvancedImagingFindings
Radiographicfindingsinlymphomaofbonearevariableandnonspecific.Earlyon,themost
common appearance is a vague, mottled lucency in the metadiaphysis of a long bone. The
intraosseouslesionusuallyhaspermeativepatternoflysis,butmayappearblasticorsclerotic.
Periosteal reaction andcortical destruction are generallynot seen ontheinitial X-rays,but
willdevelopwithtime.Plainradiographsoftensubstantiallyunderestimatetheanatomicextent
ofthelesion.
FindingsonMRIarenonspecific,withlowsignalintensityonT1-weightedsequencesand
highsignalintensityonT2-weightedsequences.Thetumorenhancesinahomogeneousfashion
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afteradministrationofgadolinium.Approximatelythreeoutoffourtumorshaveanassociated
soft tissue mass. MRI findings that are indicative of PLB include an extensive marrowreplacing lesion and associated soft tissue mass with little or no cortical destruction.
Lymphomahasanincreaseduptakeonbonescan.
DifferentialDiagnosis
TheradiographicdifferentialdiagnosisofPLBincludesosteosarcoma,smallroundcelltumors
likeleukemia,myeloma,Langerhanscellhistiocytosis,Ewingsarcoma,andosteomyelitis.
PreferredBiopsyTechniqueforThisTumor
Open.Indisseminatedlymphoma,tru-cut needlebiopsyis morethanadequatefordiagnosis.
Because PLB is an unusual variant of lymphoma, small biopsy specimens leave room for
uncertainty. Lymphoma may be difficult to differentiate from Ewing sarcoma, chronic
osteomyelitis, Langerhans cell histiocytosis, and small cell undifferentiated carcinoma with
insufficientbiopsyspecimens.Alargerbiopsyspecimenhelps“prove”toallconcernedthat
thelesionistrulyalymphoma.
HistopathologyFindings
Ongrossexamination,primarynon-Hodgkinlymphomaoftheboneisagray-whitetumorthat
diffuselyinfiltrates bone. Pathologic diagnosis requires clinical suspicion of lymphoma for
good tissue handling. Itis essential togettissuewithout crush artifactor decalcification to
preserve cell morphology. Needle biopsy is notadequate. Non-Hodgkin lymphoma appears
most commonly with large cells with irregular cleaved nuclei and prominent nucleoli
surrounded by reticulin fibers. The most common subtype is diffuse histiocytic lymphoma.
Hodgkinlymphomahas amixedcellpopulationwithplasmacells,lymphocytes,histiocytes,
andeosinophils.Reed–Sternbergarelargecellswithabilobednucleusormultiplenucleiand
prominent eosinophilic nuceoli. Their presence is essential to the diagnosis of Hodgkin
lymphoma. The pathologic differential includes Ewing sarcoma, chronic osteomyelitis, and
eosinophilicgranuloma.
TreatmentOptionsforThisTumor
No definitive treatment guidelines have been established for the treatment of PLB.
Traditionally, treatment has beenbased onradiation therapyalone.Improved survival rates
werereportedwiththeadventofcombinedtreatmentwithchemotherapyandradiation.Other
than diagnostic biopsy, surgery is used for treatment or prevention of pathologic fractures.
Avascularnecrosisandextensivedestructionofbonemayalsorequiresurgery.Althoughwide
resectionis rarelyindicated in lymphoma of bone,extensive bone destructionespeciallyin
weight-bearing bones of the lower extremities may necessitate resectionand reconstruction
withstructuralallograftsand/orendoprosthesis.Inthefootandankle,PLBisgenerallytreated
withradiation,systemicchemotherapy,restrictionofweight-bearing,andprotectionofthefoot
with a fracture boot. The tumor generally responds well to treatment, and this results in
significantreversalofthedamagetotheaffectedbones.Fullweight-bearingcanberesumedin
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manycasesoncemedicalandradiationtreatmentshavebeenunderwayforafewmonths.
PreferredMarginforThisTumor
Anymarginisappropriate,surgeryisdoneforbonefragility,nottumorresection.
OutcomesofTreatmentandPrognosis
Prognosis of PLB is controversial. There are a wide range of prognoses reported in the
literature.VariationsinthedefinitionandthetreatmentofthePLBmaybesomeofthereasons
forthis.InonelargereportofPLB,76the overall5-yearsurvival was 91%. Overall 5-year
survivalforsystemiclymphomapatientswithboneinvolvementwas79%.Combinedmodality
treatmentisreportedtoyieldbettersurvival.Youngerageandlocalizeddiseasearepositive
prognosticfactors.
SpecialandUnusualFeatures
Pathologicfracturesfromlymphomamayoccuratthetimeofdiagnosis,duringtreatment,and
months or years after treatment is concluded. Fractures after treatment were attributed to
radiation doses of 50 Gy or higher, chemotherapy containing prednisolone, and other risk
factorssuchasoldage,femalesex,surgicalinfection,andPagetdisease.
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METASTATICTUMORSANDTUMORMIMICS
MetastaticBoneTumorsintheFoot
IntroductionandDemographics
Metastases distal to the knee are unusual, and metastases to the bones of the foot
(acrometastases)areevenmorerare.56Metastasestothebonedevelopin30%ofallpatients
withcancer,withonly0.007%to0.3%havingacrometastases.78Libsonetal.79foundthatthe
mostcommonsourceswere thecolorectal, renal,andlungcarcinomas, with bladder,uterus,
andbreast cancers also contributingmetastases to the foot.Zindricketal.56 found the main
sourcesofmetastases tothe bonesofthefeettobe thegenitourinarytract andcolon.80 The
talusismostcommonlyaffected,thenthecalcaneus.49Thetarsalbonesareinvolvedin50%of
cases, themetatarsalsin23%,whereas thephalanges accountfor only17% ofthe reported
cases80(Fig.11-7).
SymptomsandPresentation
Common complaints include foot pain, localized or diffuse swelling and progressive
enlargement, subunguallesion,oranulcerateddischarginglesion.Manycasespresenttothe
surgeonmimickingotherbenignprocesses,andadelayindiagnosismayresult.Patientsmay
have pain for weeks or months before radiographic changes can be detected.81 Clinical
features of metastasis to the phalanges include redness, warmth, swelling, ulceration, or
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tenderness.
80
FIGURE11-7.Ametatarsallesionina90-year-oldwomanwithhistoryofnephrectomyforrenalcancer6years
previous.
X-rayAppearanceandAdvancedImagingFindings
Radiologicsigns onplainX-rayswill varydependingon thetypeandaggressiveness ofthe
tumorandthetimeelapseduntilpresentation.Theseincludesofttissueswelling,moderateto
markedbone destruction, andinsome cases completebone lysis.80 Bone destructionis not
accompaniedbyadistinctperiostealreaction.
56,82
Whenthedistalphalanxofthebigtoeisthe
siteofthelesion,thejointisrarelyinvolvedorcrossedbythelesion,56andathinmarginof
subchondralboneusuallyremains,evenwithextremedestruction.
56,80
The subchondralbone
plateaswellasthejointspaceisusuallypreserved.80Theneoplasmmaycauseballooningof
thethinnedcorticalshellasitenlarges.
56
DifferentialDiagnosis
Theradiographicdifferentialdiagnosisofmetastasisincludesosteosarcoma,smallroundcell
tumorslikelymphoma,myeloma,Ewingsarcoma,andalsoosteomyelitis.Tuberculosissepsis
locatedinajointofthefootoranklecanpresentwithanaggressivedestructiveappearance
andmimicamalignancy.
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RecommendedBiopsyTechniqueforMetastaticBoneTumorsinthe
Foot
Patientswithmetastaticlesionsinthefootrequiresurgeryforbiopsyonly.Biopsymayalsobe
easilyaccomplishedviaCT-guidedtru-cutneedle.Ifthepatientalreadyhasaknowncancer
diagnosis,onlyasmallsampleofthetumorinthefootisrequiredtoconfirmthediagnosisof
metastasis.Insomecaseswheretherearewidespreadbonemetastasisfromaknownprimary
tumor, a biopsy of a newly discovered bone lesion in the foot is completely unnecessary.
Unusuallesionsandlesionslocatedinjointsshouldbecomprehensivelyculturedatthetimeof
biopsy.
SurgicalTreatmentforMetastaticBoneTumorsintheFoot
Reconstructive surgical intervention for these tumors is almost never necessary.
Immobilization with a removable fracture boot, offloading andrestricted weight-bearing to
preventfracture,andpaincontrolarewarranted.Radiationtreatmentisalmostalwaysneeded,
and prompt administration of antiresorptive bisphosphonate medications is strongly
recommendedtopreventfurtherbonedamage.
TumorMimics
IntroductionandDefinition
Tumor mimics are non-neoplastic lesionsthat mimicthe appearanceand behavior of a true
bone neoplasm. Becauseoftheoverlap inpresentationbetweenthese lesionsand true bone
tumors, a similar systematic approachtodiagnosis is necessary. The imaging,workup, and
biopsytechniquesareidenticaltothoseappropriateorpotentiallymalignantbonelesionsina
similar location.Because bonetumors are relatively rare, tumor mimics may be equally as
likelyinpatientgroups.Treatmentoftumormimicsdependsonthediagnosis.
StressFracture
IntroductionandDefinition
Most stress fractures can be readily identified based on the clinical history, plain X-ray
findings, andMRIfindings when necessary. However,insome cases, there is a lack of an
appropriatehistory,ortheimagingfindingsareequivocal.Stressfracturesmaybedifficultto
differentiatefrominfection,boneinfarction,ormalignantneoplasm.Stressfracturesarecaused
byrepetitiveepisodesofmechanicalloading,whichresultinbonestrain.Strainisaunitless
valuethatrepresentsachangeintheunitlengthperunitlengthofbone.Theamountofinjury
caused by a given amount of strain increases with both magnitude and rate of application.
During normal activities, a healthyboneiscapableoftargeted remodeling ofinjuredareas.
Both large loads applied over short periods and small loads applied repetitively without
sufficienttimeforboneremodelingcancausestressfractures.
Stressfracturescanbecaused byextrinsic andintrinsic factors.Intrinsicfactors include
thetypeofactivityorsport,trainingprogram,environmentalfactors,shoecharacteristics,and
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surface characteristics. Intrinsic factors include bone structure, muscle dysfunction, joint
flexibility,andfootshape.Acavusfootisassociatedwithanincreaseoftibialstressfractures,
whereasaplanusfootisassociatedwithmetatarsalstressfractures.
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IncidenceandDemographics
Stressfracturesmayoccuratanyageandinanypatient.Females,athletes,andmilitaryrecruits
haveincreasedrisk.Amongmaleandfemaleathletes,theincidenceisapproximately3%and
approximately 9.2%, respectively. The typical presentation is activity-related bone pain of
insidious onset following a burst of physical activity. Some patients lack any history of
increasedactivityorwalking.Childrenmaynotbeabletogiveahistorythatissufficientfor
thecliniciantoconsiderstressfractureinthedifferential.
Common locations of stress fracture include thetibia, tarsal navicular,metatarsals, and
fibula. Stress fracture location is based partly on the type of sport or activity. Endurance
athletes are atincreased riskof stress fracturesintheproximal skeletonsuch as the femur,
whereas power athletes suchas sprinters and weight lifters are more likely to have distal
stressfracturesinthetarsalbonesormetatarsals.
Riskfactorsincluderepetitive,high-intensitytraining,recreationalrunnersaveragingmore
than25milesperweek,andparticipationintrack,dance,basketball,andsoccer.Womenareat
higherriskthanmen.Patientswithlow25-hydroxyvitaminDlevelsaremorepronetostress
factors. Other riskfactors include eating disorders, amenorrhea, osteoporosis, smoking, and
consumingmorethan10alcoholicdrinksperweek.
X-rayAppearanceandAdvancedImagingFindings
In theearlystages,thetypicalradiographicfeatures ofstressfracture arenotpresent.There
maybeavague,partiallymineralizedmassthatcanbemistakenforatumor.AnMRIatthis
early stage further confoundsthe diagnosis, because the edema and early callusaround the
lesionhavesignalcharacteristicsidenticaltotumor.Abonescanattheearlystagemayormay
notdemonstratethe characteristicnarrow, transversebandofintensetracer uptake.AnMRI
maylackthecharacteristictransverselineoflowsignalintensityatthesiteofthefracture.
Low-qualityMRIscans,scansdegraded bymotionartifact,andlow field strengthMRIs
should be repeated. For maximum diagnostic value, MRIs should combine T1- and T2weighted images as well as short tau inversion recovery and fat-suppressed T2-weighted
images.High-resolutionmultisliceCTscanimagingfocusedontheareaofinterestcanbevery
helpful in revealing fracture lines. Limiting the CT scan to a narrow area of interest is
recommendedtoreduceradiationexposure.Bonescandoesnotappeartoincreasediagnostic
accuracyoverthecombinationofplainX-ray,MRI,andCTscan.Thecorrectdiagnosiscan
sometimesbemadebyreanalysisoftheavailableimagingfindings.Low-qualityX-raysshould
berepeated.ComparingnewX-rayswitholdoneswilloftenrevealusefulfindings.Overtime,
stress fractures evolve toward healing, maturation, and consolidation, whereas true bone
tumorsevolvetowardgrowth,bonedestruction,andalargersofttissuemass.
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TreatmentforThisTumorMimic
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Biopsy is only rarely necessary. Careful attention to the interpretation of the biopsy is
necessaryduetotheoverlapbetweenstressfracturehistologyandthehistologyofmalignant
bonetumorssuchasosteosarcoma.Treatmentofstressfractureisbyconventionalmeans.
Gout
Introduction
GouthasbeenknowntomimicmanydiseasessincethetimeofHippocrates.Theassociation
ofgoutwithpain,redness,andwarmthinthebigtoeissopervasivethatapatientwithgoutin
anyotherlocationisatriskofadelayindiagnosisevenwhentheclinicalpictureistypical.
Destructive bone lesions from gout may present in a teenaged patient or in a patient with
normaluricacid;withminimalredness,warmth,orpain;orinanatypicallocationinthefoot.
Gout can mimic botha softtissue tumor andan aggressive bone lesion. Clinical examples
includeapainful,expansilebonelesioninthemedialhallux sesamoidinateenagerwithno
prior history, a cystic tumor in thetalus of an obese teenager withconcurrent diagnosis of
juvenilerheumatoidarthritis,andadestructivelesionatabone–prosthesisinterfaceofatotal
ankle.
SymptomsandPresentation
Theintensityofthepainisthehallmarkofgout,and acarefulhistoryisusuallysufficientto
makethediagnosis.Atsomepointduringtheevolutionofthelesion,thepatientwillhavehad
severepainintheareaofthelesion,afewdaysoraweekwhenthepainwassoseverethat
normalfunctionwasimpossible.Acomparabledegreeofpainandsensitivitytopressureisnot
seeninbenignormalignantbonetumors.Patientsmaypresentaftertheacutephasehaspassed,
butthebonelesionremains.
X-rayAppearanceandAdvancedImagingFindings
Theradiologic appearanceofbonelesionsfromgoutmaymimicanaggressive ormalignant
tumor, withpoorlymarginatedbone lysis, cortical destruction, and anassociatedsoft tissue
mass. The MRI scan may overstate the worrisome features of the lesion and add to the
confusion.Itisoftensufficienttorealizethatthe“tumor”maysimplybegoutpresentinginan
unusuallocation.
The classic X-ray appearanceof goutinbone is a well-defined eccentric, periarticular
lytic erosive “punched-out” lesion with an overhanging edge. The cortical destruction and
associated soft tissue inflammation contribute to the appearance ofan aggressive tumor. In
contrasttorheumatoidarthritis,thejointspaceistypicallypreserveduntillate,andthereisno
associatedperiarticularosteopenia.Tophaceousdepositsdonotcontaincalcificationsbutare
plainlyvisibleontheX-rays.
MRIofgoutbonelesionsisnonspecific.MRImaybehelpfulindifferentiatingsofttissue
masses(tophi)fromatrueneoplasm.TheMRIappearanceoftophiischaracteristicallylowintermediatesignal intensityon T1-weighted images, with heterogeneoussignal intensityon
T2-weightedimages.Theedematoustissuesurroundingthetophusenhanceswithgadolinium.
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There have been recent developments in the ultrasound imaging of gout. The so-called
double contour sign is a specific ultrasonographic feature of gout, best seen with highresolutionequipment.Thisfindinghasalsobeendescribedas“urateicing.”Thisfindingarises
fromahyperechoiclayerofuratecrystals depositedonthecartilagesurface.Thisfindingis
presentinbothsymptomaticand“silent”jointsofpatientswithelevatedserumuricacidwith
or without full-blown gout. This new modality may potentially contribute to noninvasive
methodsforearlydiagnosisandbettermanagementoftheskeletaldamagethatgoutcancause.
Theroleofultrasoundinthediagnosisandmanagementofgoutisbeingreassessedinlightof
thesenewfindings.
Osteoarthritis-RelatedCystsofBone
The origin of osteoarthritis-related cysts (ganglioncysts) is unclear,buttheyare commonly
associatedwith degenerative conditionsofsoft tissue or bone.They mayoccur adjacent to
joints, tendons, fascial planes, and within bone. Most ganglion cysts can be definitively
diagnosed based onacareful history andphysical examination.By history, the lesionhas a
tendencytobothincreaseanddecreaseinsizeovertime.Onexam,ganglioncysttypicallyhas
superficial location, andmaybe adjacenttoajoint.When these lesions occurnear ajoint,
there is often osteoarthritis seen on the X-ray. The mass is soft when the nearby joint is
relaxed,andbecomes firmwhen thenearbyjointormuscle is tensed. Apenlight or a small
laserpointerwilltransilluminatethecyst.Whentheselesionsoccurnearajoint,thereisoften
osteoarthritis seenon theX-ray. An MRIshould be obtained to further delineate the mass.
Aspirationofthecharacteristicclearviscousmaterialfromthemassconfirmsthediagnosis.If
thecharacteristicfluidisnotobtained,nofurtherattemptsshouldbemade.Treatmentincludes
furtheraspirationandinjectionwithcortisone,whichshouldleadtoresolutioninabouthalfthe
cases.Surgicalremovalisindicatedforpersistent,large,ortroublesomecysts.
PVNSofBone
PVNSisanuncommonproliferativeconditionofthesynoviumthatcaninvadethebone.PVNS
wasfirstdescribedbyChassaignacin1852.Hedescribedanodularlesionthatarosewithin
the flexor tendon sheaths of the hand. Simon in 1864 recorded the localized form of the
disease. Moserinin 1909 noted thediffuse form. It wasJaffe in1941whocoined the term
“pigmentedvillonodularsynovitis”inacaseseries.78Itisgenerallybelievedtobeabenign
neoplastic process; however, some believe that it can occur secondary to inflammation or
trauma. A few cases of metastasis have been reported in the literature. It most commonly
occurs in the synovial lining of joints, and itcanalso occur intendon sheaths, where it is
calledgiantcelltumorofthetendonsheath.Thesetumorshavealsobeenfoundwithinsynovial
bursae.Theankleisthethirdmostcommonlocationfordiffuseintra-articularPVNS.Lesions
intheanklemayinvadethedistaltibiaortalus,orboth.Theankleandhindfootareaffected
approximately twice as often as the forefoot. PVNS has distinctive characteristics, which
allowittobeidentifiedonimagingstudies.
Onplainradiographs,PVNSmaypresentasasharplydefinedcysticlesionadjacenttothe
involvedjoint.Becausethelesionissynovialinorigin,bonecystsmayformonbothsidesof
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thejoint,whichdifferentiatesthislesionfromallotherbenignbonetumors.OtherthanPVNS,
lytic lesionson bothsidesofthe jointmaybecaused bysevere untreatedosteoarthritis and
septictuberculosisoftheankle.OnMRI,PVNShasauniqueanddiagnosticappearancedueto
thehemosiderincontainedwithinthetumor.
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