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moreappropriate,effective,andimmediatemanagementtobeinstituted.
ACKNOWLEDGMENTS
IwouldliketothankLorettaCacaceDPM,MPHforherexcellentassistanceinthepreparation
of this manuscript for publication while a 4th-year student at the New York College of
PodiatricMedicine.
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S
SECTIONA:
SoftTissueTumorsoftheFootandAnkle
oft tissue tumors of the foot and ankle are different from those of the rest of the
musculoskeletalsystemintermsoftype,riskofmalignancy,age,treatment,andprognosis.
Tumors of the foot andankle maybedegenerative,reactive, posttraumatic, orneoplastic in
origin,ortheymayarisefromanunknowncause.Thetreatmentvarieswidelydependingonthe
type of tumor.To select the appropriate treatment,the surgeon must begin with anaccurate
diagnosis.Theultimategoalsoftreatmentaretoeliminatethetumorandrestorethepatient’s
long-termmobilityandfunction.
According to the most recent World Health Organization definitions, soft tissue tumors
should be described as benign, intermediate (locally aggressive), intermediate (rarely
metastasizing),andmalignant.Thesetermshavebeenintroducedtoeliminateconfusionarising
fromoutdateddescriptionssuchas“intermediatemalignancy.”Accordingtothisterminology,
benigntumorsgrowlocally,donotinfiltrate,donotrecur,orrecurinanondestructivefashion.
Schwannomaisanexampleofa benigntumor.Intermediate(locallyaggressive)tumorsmay
grow inan infiltrative fashionand may recur locallyunless excised with a wide marginof
normal tissue. Plantar fibromais anexample.Intermediate (rarelymetastasizing)tumors are
locallyaggressive,growinaninfiltrativefashion,andarelikelytorecurlocally,andalsohave
a demonstrated ability to metastasize to distant sites. An example of this type of tumor is
angiomatoidfibroushistiocytoma.Malignanttumorssuchassynovialsarcoma(SS)andclear
cell sarcoma (CCS)grow inan aggressive, infiltrative, andlocallydestructive manner,and
carryahighriskofregionalanddistantmetastasis.
The mostcommonbenign soft tissuetumors inthe footand ankle includeganglion cyst,
plantar fibroma, hemangioma, schwannoma, neurofibroma, pigmented villonodular synovitis
(PVNS),andgiantcelltumor oftendon sheath. Lipomaisrare inthe footandankle. Tumor
mimicsarebenignnon-neoplasticsofttissuemasseswhosebehaviorissimilartothatoftrue
tumors. Examples include gouty tophi, synovial cysts, synovial masses from degenerative
tendinopathy,rheumatoidnodules,andepidermalcysts.1Morethan100othertypesofbenign
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andmalignantsofttissuetumorsmayoccurinthefoot,butacomprehensivedescriptionofthem
is outside the scope of this chapter. Instead, a strategy for evaluation and management is
presentedthatappliestoanytumor.
Alargerproportionofsofttissuetumorsinthefootandanklearemalignantthanelsewhere
inthe body. Each individual surgeon’s chance of encountering a malignanttumor will vary
dependingonpracticeprofileandreferralsources.Insomepublishedreports,asmanyas20%
to62%ofsofttissuetumorsinthefootandanklearemalignant,althoughthetrueincidenceis
notknown.
1–3
Allfootandanklesurgeonsshouldbevigilant.Theriskofmalignancyincreases
with age. Malignant melanoma is the most common soft tissue malignancy in the foot.
Pleomorphicsarcoma(PS;previouslynamedmalignantfibroushistiocytoma[MFH]),SS,and
CCSarethemostcommonsofttissuesarcomasinthefootandankle.
Surgeryistheprimaryformoflocalcontrolforfootandanklesarcomas.Thesurgeonmust
achieve an appropriate margin around the tumor to minimize the risk of local recurrence.
Surgicalmarginsaredefinedbythetermsintralesional,marginal,wide,andradical.Theterm
enbloc,meaningthetumorisremovedinonepiece,hasnooncologicsignificanceandisnot
by itself an adequate description. Opening, entering, or spilling the tumor by design or by
accidentsuchasduring acurettageofabonecystalwayscreatesanintralesionalmargin.A
marginalmarginiscreatedwhenthesurgeondissectsoutsidethetumoradjacenttothecapsule
or pseudocapsule, even ifthe tumoris never entered or exposed.A wide marginis created
whenthesurgeondissectscompletelyoutsidethepseudocapsuleandallreactiveorabnormal
appearingtissues.Bydefinition,awidemarginincludesacontinuousunbrokencuffofnormal
tissuesurroundingtheentiresurgicalspecimen.Aradicalmarginisachievedwhentheentire
compartmentorcompartmentscontainingthetumorareresected,alongwithanyandallbone,
nerve,vessel,ortendoninthecompartment.Toachievearadicalmargininthefootandankle
atleastapartialamputationisrequired.
Forbenigntumors,simpleremovalwithanintralesionalorwidemarginisadequate.There
isnoadverseeffectifthesurgeonspillstumorcellsintothenearbytissues,orleavesaportion
of the tumor behind. For intermediate tumors, the risk of local recurrence and the debility
associatedwithmultiplesurgeriesdemandsamorethoroughsurgicalapproach.Awidemargin
is the goal, buta marginal marginis the most likely outcome. The surgical resection must
encompasstheentiretumor,anypseudocapsule,andthethickestpossiblecuffofnormaltissue
aroundthemass.Thesurgeoncanadjustthesizeofthemarginsaccordingtothepropensityof
thetumortorecur.Forexample,plantarfibromaisabenigntumor,butitcarriesaveryhigh
riskoflocalrecurrence,andamarginof1to2cmisoptimal.Aschwannomacanberesected
withamarginalmargin.
Local control of malignant tumors requires an uncompromised wide margin. Wide
resectionsaremorelikelytocompromisetheskeletalstability,neurovascularstatus,andsoft
tissueintegrityofthelimb.Surgeonsseektobalanceandoptimizetheoncologicandfunctional
outcomes, andmayacceptmarginal marginsinordertofacilitatelimb salvage.4Thegoal of
surgeryistoresecttheentiretumorenbloc,withanuncontaminatedmarginof5to10mmof
normaltissuesurroundingallaspectsofthemass.Atruewidemarginrequiresthatnopartof
thetumor,itscapsule,orthereactivezonearoundthetumoriseverseeninthesurgicalfield.
Allpartsofthedissectiontakeplacethroughuninvolved,nonreactivenormaltissues,andthese
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Table11-1.
normaltissuescompletelyenvelopallpartsoftheexcisedspecimen.Spillingorexposureof
anypartoftheactualtumorisunacceptable.Surgeonswhoareunprepared forthedegreeof
collateraldamagetothefootthatisrequiredtoachieveatruewidemarginaroundasarcoma
shouldnotattempttheseresections.
Adjuvant chemotherapy and radiation therapy are given routinely for osteosarcoma and
Ewingsarcoma,andareusedforotherhigh-gradesarcomasonacase-by-casebasis.Thereis
strongevidencethatconservativesurgerycombinedwithradiationtherapyresultineffective
local control. Postoperative radiationcan be administered by external beaminfractionated
dosesorbybrachytherapyusingsurgicallyimplantedradiationsourceswithashorterduration
oftreatment.Nostrongevidencefavorsonemethodovertheother.Thereisincreasinginterest
inpreoperative radiationtherapy, whichmore precisely targets the tumor anddamagesless
normal tissue. However, a definite survival benefit has not yet been demonstrated.
5
Preoperativeradiationresultsinhigherratesofmajorwoundcomplications,whichmayhave
devastatingeffectsinthefootandankle.
EVALUATIONOFASOFTTISSUEMASS
HistoryandPhysical
The initial evaluation should include a complete history, a comprehensive regional
examination,andplainradiographsoftheaffectedarea.Thisinformationisthenusedbythe
clinicianto determinethe likelihoodofmalignancy, todecide onthe besttypeof advanced
imagingtoemploy, andtoevaluatetheneedforadiagnostic biopsy. For softtissuetumors,
history and physical examination findings can be used to determine the potential for
malignancy, based on the presence or absence of five specific “good” or “bad” clinical
findings. This assessment is made based solely on the clinical features of the tumor; no
advancedimagingisnecessary.Factorsincludedintheassessmentincludepain,growth,size,
location,andexaminationfindings(Table11-1).Benigntumorsaremorelikelytobepainful,
notprogressivelygrowing,small,superficialtothefascia,anddifficulttopalpateasadistinct
mass.Malignanttumorstendtobepainless,progressivelygrowing,large,deeptothefascia,
andwelldefinedonexam.Theriskofmalignancyincreaseswiththenumberof“bad”features
thetumorhas.
ClinicalFeaturesThatHelpDistinguishBenignSoftTissue
MassesfromMalignantSoftTissueTumors
Good Bad
Painhistory Painwaspresentfromthebeginning Nopainorpaindevelopedlater
Growthhistory Growsandshrinks Growsprogressively
Size Small(<2cm) Large(>3cm)
Location Superficial Deep
Findingsonexam Anindistinctmass Adefinitemass
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Thelikelihoodofmalignancyincreasesasthenumberof“badfeatures”increases.
6
Certain physical examination findings might point toward a specific diagnosis. For
example, hemangiomas have a characteristic blue-purple color that may be visible in the
overlying skin. These lesions are typically painful to palpation. Ganglion cysts can be
transilluminatedwithapenlightoralaserpointerand,whentransilluminationisdemonstrated,
aspiratedtoconfirmthediagnosis.
Laboratorytestsarenotgenerallyusefulintheinitialmanagementoftumorsofthefootand
ankle, unless there is clinical evidence of infection, an inflammatory condition such as
rheumatoidarthritis,orgout.Nonspecificlaboratoryscreeningtestsarenotrecommended.
ImagingEvaluationofSoftTissueTumors
The history and physical examination may be adequate to confirm the diagnosis of many
commonsofttissuemassesinthefootandankle,suchasganglioncystandplantarfibroma.In
thesecases,imagingofthelesionmaynotbeclinicallyindicated.Forsofttissuetumorsthat
cannotbereadilydiagnosed,twoorthogonalhigh-qualityplainradiographsarerecommended
astheinitialimagingstudy.Theradiographicfindingsmaypointtoapossiblediagnosis.For
example, intramuscular hemangiomas (IMHs) may contain small rounded pebble-like
calcificationscalledphleboliths,whereas30%ofSSscontainamorphouscalcifications.Soft
tissuesarcomasandmetastaticcancersinthefootmayinvadeanearbybone,aworrisomesign
associatedwith malignancy. Advanced imaging otherthanMRI,suchas CTscans,positron
emissiontomography(PET)scans,andbonescans,isnotcommonlyusefulintheevaluationof
softtissuelesions.
7
The malignant potential of some soft tissue tumors may be difficult to determine with
confidence. The history and physical examination findings can be unhelpful or even
misleading. A contrast-enhanced MRI examination with a dedicated extremity coil is
recommendedforlesionslargerthan2or3cmandforallsofttissuetumorswithworrisome
clinicalfeatures.TheMRIsignalcharacteristicsmayinsomecasesbeadequatetoidentifythe
exactnatureofthe lesion,or forthe differentialtobe narrowed, butinothercases theMRI
findingsarenonspecific.Nevertheless,MRIisveryhelpfulindeterminingthesizeandextent
ofthetumoraswellasitsrelationshiptotheneurovascularelements.MRIisanessentialtool
forclinicalstaging,biopsyplacement,surgicalplanning,andevaluationofresponsetotherapy.
DeterminateversusIndeterminateSoftTissueTumors
Asystematicapproachtoassessmentofthepotentialformalignancyofsofttissuemasseshas
beenproposed wherebysoft tissuetumors are divided into two groups: “determinate” and
“indeterminate”basedontheclinicalandradiographicfindings.Thesubsequentmanagement
planfollowsdirectlyfromthisassessment.
8,9
Softtissuetumorsforwhichaspecificdiagnosiscanbemadebasedonacombinationof
history, the physical examination, and analysis of the MRI findings are called determinate
lesions.Determinatelesionsthatmayoccurinthefootandankleincludelipoma,hemangioma,
ganglion cyst, some plantar fibromas, and PVNS. Determinate lesions are treated with
observationorbyexcisionalbiopsy,dependingontheclinicalsituation.
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Soft tissuetumors forwhichnospecific diagnosis isconfirmedbythe exam,X-ray, and
MRI findings are called indeterminate lesions. These lesions are typically isointense with
muscle(dark)onT1-weightedimagingandhyperintense(bright)onT2-weightedimaging.In
thefootandankle,thisgroupoftumorsincludesallsofttissuesarcomassuchasSS,CCS,and
liposarcoma,aswellasanumberofbenigntumors,suchasgiantcelltumoroftendonsheath,
peripheral nervesheath tumors,andsomeplantarfibromas.Indeterminatelesionsaretreated
withincisionalorneedlebiopsy,alongwithappropriatereferraltoanorthopediconcologist.
Ifthereisanydoubt,itisbesttodefer thebiopsytothespecialistteam.Definitivesurgical
removalofindeterminatelesionsisalwaysperformedasaseparateprocedureafterthefinal
pathologicdiagnosisandstagingarecompleted.
BIOPSYPLANNINGANDTECHNIQUES
Somefootandanklesurgeonsreferextremitytumorstoaspecialistforbiopsyanddefinitive
treatment,whereasothersfeelcomfortablemanagingsofttissuelesionswithintheirscopeof
practice. A systematic approachtothe performance ofthe biopsyis recommended because
improper timing, technique, or management of the biopsy process increases the risk of
complications.Thebiopsytechniqueshouldbechosenwithcaresothatanaccuratediagnosis
ismadewithminimalrisk.Complicationsofimproperlyplannedorexecutedbiopsiesinclude
errors in diagnosis, inadequate or nondiagnostic biopsy material, surgical site infection,
hematoma, and contamination of nearby tissues. Biopsy complications have been shown to
increase the riskofanotherwiseavoidableamputation.Theseproblems maybe avoided by
earlyreferraltoanorthopediconcologistortumorspecialistpriortothebiopsy.
Thesurgeonisresponsibleforconsultingwiththepathologistpriortoperformingabiopsy
incaseswheremalignancyispossible(forall indeterminatelesions). Directcommunication
bytelephoneishighlyrecommended as a meansofinsuring thatthesurgeon andpathologist
collaborateinanoptimalfashiontomaximizethediagnosticyieldofthebiopsyandminimize
potentially devastating errors. Because the biopsywill alter theimaging appearance of the
tumor,alldiagnosticscansshouldbecompletedpriortobiopsy.
Themostappropriatebiopsytechniqueshouldbeenselectedaccordingtotheclinicaland
anatomic situation. Options include needle aspiration, tru-cut or large-bore needle biopsy,
incisionalbiopsy,andexcisionalbiopsy.
Fineneedleaspiration(FNA)andcoreneedletechniqueshavetheadvantageofbeingwell
tolerated by the patient and causing few complications. FNA is an appropriate choice for
patientswithaknowncancerdiagnosiswhoaresuspectedtohavearecurrenceorametastasis
inthefootorankle.FNAisalsousefulforotherlesionswherethereislittledoubtaboutthe
diagnosisandthebiopsyisonlyneededtoconfirmwhatisalreadyknown.However,FNAis
inadequatetoevaluatethetissuecytoarchitecture,andtheamountofmaterialobtainedmaybe
inadequatefortissuebankingandancillarystudies.FNAandcoreneedlebiopsyisthefirstline biopsy technique for soft tissue tumors in some cancer centers. In those centers, a
dedicatedandexperiencedteammanagesallsofttissuetumors.Thisapproachcanminimizeor
eliminatetheshortcomingsofneedletechniques.
In settings where an experienced biopsy team and an experienced solid tumor
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Table11-2.
■
■
■
■
■
cytopathologist are not available, needle biopsy may be inadequate or inconclusive. Open
(incisional)biopsytechniquesarepreferredinthesecases.Openbiopsyisasafeandeffective
techniquewhenproperlyplannedandexecuted.Moretissueallowsforbetterunderstandingof
the cytoarchitecture of the lesion and reduces the risk of a nondiagnostic result. Essential
materialforancillarystudiesandtissuebankingcanbeobtained.
Excision of the tumor immediately after open biopsy is possible for a small group of
tumors where the frozen section features of the tumor are highly diagnostic. This group
includes giant cell tumor of tendon sheath, plantar fibroma, schwannoma, andother tumors
dependingon theexperienceand confidenceofthe pathologist.Thesurgeonandpathologist
should both be highly experienced in managing tumors. If sufficient doubt about the final
diagnosis exists or the pathologist is not comfortable making a decision based on frozen
sectionalone,thedefinitivesurgeryshouldbedelayeduntilthefinalpathologicanalysishas
beencompleted.Mostindeterminatetumorswillrequireimmunohistochemicalstainsandother
specializedexaminationsfordiagnosis.Definitivesurgerycantakeplace7to14daysafterthe
biopsy.
Thesuccessofanincisionalbiopsydependsoncarefulprebiopsyplanning,properbiopsy
execution,andadequatepostbiopsycareofthepatient(Table11-2).Allimagingstudiesmust
becompletedpriortobiopsy.Thepathologistandthesurgeonshoulddiscussthecasepriorto
thebiopsyortheymaydosointheoperatingroom.Atourniquetisappliedafterelevationbut
without exsanguination. Tumor sampling is accomplished through adequate longitudinal
incisionsthatapproachthelesionmostdirectly.Theincisionshouldbelongenoughtoallow
visualizationoftheprocedure, confirmationof appropriate sampling,and hemostasis. Small
“keyhole”biopsyincisionsarenotrecommended.Contaminationofuninvolvedstructuresand
the neurovascularbundlesespeciallynearthemedialmalleolusshouldbecarefullyavoided.
Thetourniquetshouldbereleasedafterthetumorissampled,andmeasuresshouldbetakento
insurethatthewoundiscompletelydry.Unintentionalspreadofthetumormayoccurbecause
of postbiopsy bleeding. A hematoma may track under subcutaneous tissues or through
intramuscular spaces, causing a wide zone of contamination. During open biopsy, the
pathologistshouldexamineafrozensectionofthebiopsymaterial toverifythatan adequate
amountofviabletumorhasbeenobtained.Culturesshouldbeperformedunlessthepotential
forinfectionhas been definitivelyruled out.A moderately compressive dressingshouldbe
appliedandthepatientismadenonweight-bearingwiththeextremityelevatedfor3or4days.
Thesurgeonwhoperformsthebiopsyshouldscheduleaface-to-faceencounterwiththepatient
in7to10daystodiscusstheresultsoftheprocedureandthefuturetreatmentplan.Itisbestto
avoiddeliveringbiopsyresultsoverthetelephoneorviaanintermediary.
ChecklistofTasksRequiredforaSuccessfulBiopsy
Prebiopsy
Allimagingstudiescompleted
Appropriatebiopsytechniqueselected
Imagingreviewedtoplanapproachandtumorsamplingstrategy
Pathologistconsultedbytelephoneordirectlyinoperatingroom
Operatingroomawareofafrozensectionplanned
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