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DuringtheBiopsy
Adequatelongitudinalincisions
Directapproachwithminimaldissection,noflapsorundermining
Donotexposetheneurovascularbundle
Donotbiopsythepseudocapsule
Biopsythemostcellularareaofthetumor—usuallytheoutermostpart
Pathologistperformsfrozensectiontoverifybiopsyisadequate
Performculturesifindicatedorifnoevidenceofaneoplasmseenonfrozensection
Postbiopsy
Meticuloushemostasiswithtourniquetreleased
Avoiddrains
Mildlycompressivebandageorsplintapplied
Strictactivityrestrictionandelevationwritteninorders
Scheduleaface-to-facefollow-upvisittosharebiopsyresult
Excisionalbiopsyisappropriateforthedeterminationofsofttissuetumorsinthefootor
ankle as discussed earlier, and may be advisable for small and superficial indeterminate
tumors.Duringexcisionalbiopsy,careshouldbetakenthatthetumorisnotexposedorentered.
Theentiretumor,thesurroundingcapsule,andasmallamountofsurroundingnormalsofttissue
aretakenenbloc.Excisionalbiopsyisneverappropriateforlargeordeepsofttissuetumors.
MANAGEMENTOFAPATIENTWITHANUNPLANNED
EXCISIONOFASOFTTISSUESARCOMA
Soft tissuesarcomas are rareandmayhavean indolentpresentation.Asaresult,unplanned
excisionofsofttissuesarcomainthefootandankleiscommon.10Unplannedsurgeryisdefined
as excisional biopsy or unplanned resection of a malignant sarcoma without an adequate
margin.Outcomesafterunplannedexcisioninallbodysitesareinferiortothoseafterplanned
surgery.11Theimplicationsofunplannedtreatmentofsarcomasinthefootmaybemoreserious
thanelsewhereinthebody.Thepotentialforcontaminationofuninvolvedstructuresishigher,
andunplannedsurgeriesmakethesubsequentwideexcisionsurgerymoredifficult.
12
Management following unplanned resection of a sarcoma typically requires complete
excisionof the entire tumor bed and any surgicallycontaminated tissues toachieve a wide
margin. Unplanned surgery results in additional soft tissue reconstruction compared with
planned surgery.13 If the initial surgery resulted in extensive contamination of surrounding
structures,amputationmaybenecessarytoachievelocalcontrol.Followingrepeatexcisionin
cases without clinically palpable residual tumor, 50% of the cases were found to have
microscopic residual tumor.Followingrepeat excision,85%ofthepatientswerealive and
diseasefreeat5years.
BENIGNSOFTTISSUETUMORS
GanglionCyst
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ClinicalPresentation
Ganglioncyststendtopresentinyoungtomiddleageadults,morecommonlywomen,andare
very rare in children. The most common location is around the ankle joint or midfoot,
especially onthedorsolateral surface.The lesionsmayariseinassociationwith peripheral
nerves,joints,fascia,andbone.Intheauthor’sexperience,simple,superficialganglioncysts
rarelyaffecttheforefoot.Inthesecases,examinationrevealsapalpable,well-definedmass,
usually1to3cm,withnopainorverymildpain.Thelesionoccursnearthejointoralonga
tendonsheath.Themassmaygrowandshrinkaccordingtotheactivitylevel.Insomecases,
loading or flexing the tendon or joint will make the mass feel noticeably more firm. The
ganglion cysts are typically not associated with significant degenerative pathology of the
nearbytendonorjoint.
Ganglioncystmaypresentinapatientwithnonlocalizedpain,inwhomanMRIscanofthe
footreveals a smallperiarticular cystic mass, oftenadjacenttoajointinthehindfoot,sinus
tarsi,ormidtarsaljoint.Thesecystscanbeaspiratedandinjectedwithultrasoundguidance,or
excised. In addition, lesions that have MRI features consistent with ganglion cyst are
occasionallyfoundinthe plantaraspectoftheforefootadjacenttothemetatarsalphalangeal
joints. In the author’s experience, these lesions are caused by plantar plate degeneration
associatedwithagingandoveruse.Treatmentoftheselesionsisanalogoustothetreatmentof
metatarsalgiaandplantarplate–relatedpain.
ClinicalExam
Atypicalganglioncystissuperficial,firm,somewhatcompressible,andusuallypainless.The
cystwilltransilluminatewithapenlightor alaserpointer.Transilluminationistheextended
transmissionof light through the lesion dueto thesemitransparent material inthecyst. The
roomlightsmayneedtobedimmedandtheareashouldbeshieldedfromexternallightsources
tomaximizetheeffect.The surgeonshouldilluminateanearbyareatoobservethe expected
amountoflighttransmissionthroughnormaltissue.
DiagnosticProcedure
Thecombinationoftransilluminationandneedleaspirationconfirmsthediagnosisofganglion
cyst. Once transillumination is confirmed, aspiration should follow. Lesions that do not
transilluminateshouldnotbeaspirated.Aspirationofganglioncystsissimple,safe,andwell
toleratedinanofficesetting.Asinglepuncture intothe center ofthecysticmasswitha22gauge or larger needle should be performed. The diagnosis is confirmed if slightly
yellow/clear,nonturbid,viscousorjelly-likematerialisaspiratedfromthecyst.Thismaterial
canbeidentifiedbyitscharacteristicappearanceandmaterialproperties,andthuslaboratory
analysisisnotrequired.Ifunexpectedmaterial,crystals,pus,orbloodisencountered,cultures
andlaboratoryand/orpathologicanalysisisrecommended.Ifaspirationisnegative,theneedle
shouldbewithdrawn.Additionalpuncturesandmultiplepassesshouldnotbemade.AnMRI
isrecommendedtocharacterizethelesion.
Transilluminationandaspiration maylead toaconclusive diagnosis andfurtherworkup
canthusbeavoided.MRIisunnecessaryandtreatmentisatthediscretionofthesurgeon.The
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aspirationmaybecurative.Ifthelesionrecursafteraspiration,surgicalremovalcanproceed
withoutfurtherworkup.
PlantarFascialFibromaandFibromatosis
ClinicalPresentation
Plantarfibromatosis(alsoknownasLedderhosedisease)isanonencapsulatedthickeningand
proliferationofthecentralandmedialbandsoftheplantarfascia.Thelesionsmayalsooccur
in atypical locations, such as in the distal or proximal plantar fascia and in subcutaneous
tissues.The cause is unknown.One-third toone-halfof patients also present with bilateral
nodules. Approximately one-third to two-thirds of patients will also have fibromas in the
palmar fascia, the knuckle pads, or elsewhere.Patients with Dupuytrencontracture havean
increasedriskforplantarfibromas.Mostpatientsareasymptomatic,butsomehaveactivityrelated pain or shoe irritation. When the lesions are large enough to press on the plantar
nerves,theremayalsobenumbnessordysesthesiainthedistalportionsofthefoot.
DifferentialdiagnosisforthistumorisSS.
14
DiagnosticProcedure
Plainradiographsarerecommendedtoruleoutthepresenceofintralesionalcalcificationsthat
areseeninSSbutnotinplantarfibroma.Nolaboratoryexamsarerecommended.MRIisnot
necessaryforsmall,stable,andtypicallylocatedlesions.MRIisstronglyrecommendedifthe
lesionislargeorenlarging,oratypicalinanyway.
Initial managementshould consistofshoemodifications,customorreadymadeorthotics,
andpainmedication.Numerousnonsurgicaltreatmentshavebeenrecommendedorpublished
inlow-qualityclinicalstudies,includingexternalbeamradiation,15extracorporealshockwave
therapy,16injectionsofcorticosteroids,collagenaseclostridiumhistolyticuminjections,17and
transdermaltherapywith15%verapamilgel.Noneofthesetreatmentscanberecommended
duetothelowqualityortotalabsenceofanysupportingdata.
Surgical removalis reserved for largelesionsthat are causing significantdisabilitythat
have failed a well-documented course of nonoperative care. Surgeons treating this lesion
should be prepared to undertake an aggressive and comprehensive resection of the lesion,
because recurrences after surgical treatmentare inverselyproportional tothequality of the
margin.
14,18
Localfasciectomyhasa100%recurrencerate.Thepresenceofmultiplefascialnodules,
bilateral nodules, and family history is associated with increased risk of recurrence.
14,19
Aggressive resection with a wide margin(subtotal fasciectomy) is necessaryandcarries a
recurrencerate of25%. Radical fasciectomyis a misnomer because theresectiondoes not
achieve a radical margin.The surgeon should achieve a minimum of a 1 cm marginat the
fascial boundaries and thewidest possible margin at the skin surface that will still permit
primaryclosure.Amarginalmarginmustbeacceptedatthedeepsurfaceofthelesiontoallow
preservationofthemedialandlateralneurovascularbundles.Incasesofrecurrenceafterlocal
excision,asubtotalfasciectomycanbeexpectedtobesuccessfulin75%ofcases.Skingrafts
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andtissue transfers mayberequiredtofacilitatecoverageand closure inreoperated cases.
Fibromasthatrecuraftersubtotalfasciectomyshouldbeobserved.
PigmentedVillonodularSynovitis
ClinicalPresentation
PVNS is a locally aggressive synovial proliferation of unknown origin. The tumor cells
consistentlyoverexpresscolony-stimulatingfactor-1(CSF1)thathasbeenimplicatedintumor
growth.20Inaddition,DNAaneuploidy,chromosomaltranslocations,andtumorexpressionof
p63andnm23havebeenreportedinavariableproportionofcases.
Various clinical presentations of this benign tumor exist, including giant cell tumor of
tendon sheath/tenosynovial giant cell tumor, which are described later, and PVNS. Most
patientsareintheirthirtiesandforties.Thislesionisrareinchildren.Approximately2%to
10%ofcasesoccurinthefootandankle.21TherearetwoformsofPVNS:diffuseandnodular.
Nodular PVNS occurs most commonly in the forefoot.22 Diffuse PVNS is found more
commonlyinthehindfootandpresentswithosteoarthritis.PVNSmayalsooccurintheankle
andoftenpresentsasamildlypainfuljointwithswelling.
DiagnosticProcedure
X-rayimagingisoftenusedinidentifyingPVNS.TheradiologicappearanceofPVNSdepends
onthe location.A noduleintheforefootmayhavesofttissueswellingandboneerosionon
plainX-ray.Theankleusuallyonlyhasa softtissuemass,butboneerosionorcystsmaybe
present.Thejointspaceisusuallypreservedandtheremaybeaneffusion.MRIscanisableto
identifythehemosiderincontainedinthelesionandcandemonstratetheextentofthesynovial
involvementaswellasboneerosionandcysts.Hemosiderinappearsasloworabsentsignal
(signaldropout)onbothT1-andT2-weightedimages.
On gross examination, the diffuse form of PVNS is a tan mass of villi and folds of
synovium. The lesion may be sessile or have several pedunculatednodules. Bony invasion
throughthejointcapsuleispossible.ThelocalformofPVNSisapedunculatedfirmnodule.
Microscopically,PVNSischaracterizedbysynovialcellhyperplasiabothonthesurfaceand
belowthesynovium.Alsopresentarescatteredgiantcells,hemosiderin,andfoamcells.The
locationofthepolyhedralcellsbelowthesynovialmembranesuggeststhatperhapsthecellof
origin is a fibrohistiocyte. The pathologic differential includes hemosiderotic synovitis,
rheumatoidarthritis,andsynovialchondromatosis.
Treatment of mild cases of diffuse PVNS is by nonoperative means with clinical and
radiologic follow-up. Symptomatic relief can be obtained with a combination of moderate
activity restriction, offloading the joint with a removable fracture boot, and daily
administrationofibuprofenornaproxen.Completeresolutionofsymptomsmaytake12to24
months.Patientsareatriskforrecurrenceofsymptomsiftheyreturntohighactivitylevelstoo
soon.
Nodular PVNS is treated by simple excision with a marginal margin. Nodular lesions
rarelyrecuraftercompleteexcision,butwillalmostalwaysrecurifmacroscopicamountsof
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the lesionare leftbehindafter surgery. Surgical treatmentof diffuse PVNSis indicated for
bulkyorpainfullesionsandcaseswithboneorjointdamage,inordertopreventprogression.
Treatmentisbyaggressivemeans,eitheropenorarthroscopic,withoutadjuvanttherapy.There
is a 10% to 15% riskofrecurrence.23 Adjuvant treatmentsincluding radiation21 have been
used in severe and recurrent cases. In the ankle, arthrodesis or total arthroplasty with a
prostheticimplantmaybenecessaryincaseswithseverejointdamage.
24
GiantCellTumorofTendonSheath
ClinicalPresentation
Giantcelltumoroftendonsheathisarare,solitarybenignvariantofPVNSthatmayarisein
thetendonsheathtissuesofthehandandwristaswellastheankleandfoot.Mostcasesoccur
inthehand,wherelocalrecurrenceafterexcisionhasbeenreportedinupto40%ofcases.The
tumorcellsshowconsistentoverexpressionofCSF1,theligandofthetyrosinekinasereceptor,
aswellasfrequentchromosomaltranslocationsat1p13,thelocusoftheCSF1gene.
Clinically,thepatientsreportaslow-growing,painless,firmsolitarymassadjacenttothe
dorsalorplantartendons,themidfootjoints,ortheanklejoint,whichhasbeenpresentfor1to
2yearsonaverage.Theremaybeahistoryoftrauma,andneurologicsymptomsoccurrarely.
In onestudy, lesionsintheforefootoccurredinthefirst,second, andfifth rays exclusively,
indicatingthat there maybe some relationship between weight-bearing andthis tumor. The
tumormaycauseoraccentuateanangulardeformitysuchashalluxvalgus.
DiagnosticProcedure
On plain radiographs, there may be a visible soft tissue swelling, sometimes completely
encasingthebonyelementsoftheinvolveddigit,andthetumormayinvadetheadjacentbone
andcause cystic lesions that are clearlyvisible on X-ray. Approximately 10% involve the
bone. Thebone involvement anddestruction leads toconcernfor primarybonemalignancy,
and inappropriately aggressive treatments can result. CT scan will show the extent of the
tumor, and clearly delineate any bony involvement. Some of these tumors have small
calcifications, a feature shared with SS. MRI scans are helpful to define the extent of the
lesion,andcanbehelpfulinthepreoperativediagnosis.Hemosiderininthelesionmayresult
inverylowsignal intensities onsome sequences, andthe lesionsenhance onT1sequences
afteradministrationofgadopentetatecontrastagent,andthesefeatureshelpidentifythetumor.
Treatmentisbycomplete,meticulousexcisionoftheentirelesion.Awideorradicalmarginis
not necessary. Intralesional margins are acceptable, as long as complete excision is not
compromised.Recurrencehasbeenreportedinupto45%ofcases,butwithcarefulremoval,
recurrencecanbereducedto10%to20%.Inthelessertoes,wherethelesionhasextensively
invadedthesofttissuesandbone,amputationmaybepreferabletoexcision.Inthegreattoe,
effortsshouldbemadetopreservethemechanicalintegrityofthefirstray,includingcomplete
meticulous excision of the lesion, followed by bone grafts, skin grafts, and fusions as
necessary.
Aseparate,stagedbiopsyisrecommended.Thelesioncannotalwaysbecharacterizedby
preoperative studies, and aggressive or destructive features may be present that are also
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consistent withmalignancy. Opensurgical biopsywithfrozen section analysis is preferred,
performedthroughawell-plannedlongitudinalincisionthatavoidsanyinvolvementofnearby
neurovascularstructures.IfthelesioncanbecharacterizedbyMRI,andthelevelofconfidence
inthepreoperativediagnosisisveryhigh,thenexcisionalbiopsyisappropriate.
Schwannoma/NerveSheathTumors
ClinicalPresentation
Schwannomas/neurilemmomasandneurofibromasarepartofalargegroupoftumorsbelieved
toarisefromSchwanncells.Schwann cellsproducemyelinandmayalsoproducecollagen,
thustheneoplasmsthatarisefromthemhavearangeofhistologicfeatures.Thesetumorsmay
arise inboneas well asinsofttissues.Neurofibromasare more likely tooccur inyounger
individuals,are associated withneurofibromatosis,and carrya significantriskof malignant
degeneration.Schwannomas/neurilemmomasoccurinmiddle-agedandolderindividuals,are
rarely associated with neurofibromatosis, and carry an extremely small risk of malignant
degeneration. Numerous subtypes of nerve sheath tumors have been described, but an
exhaustivereviewis outside thescope ofthischapter.Mortonneuromais not a partofthis
groupoftumors.Mortonneuroma isnot a truetumor,butrather a fibrotic anddegenerative
thickeningoftheplantardigitalnerve.
Benignschwannoma/benignneurilemmomaisaslow-growingsolitarynervesheathtumor
that typically presents in adults between age 20 and 50. Schwannomatosis/multifocal
neurilemmomahasbeenobservedinthefootandankleandknee.
25,26
Thetypicalsolitarytumor
presentsasaslow-growingpainlessmassthatmayhavebeenpresentfor1to2yearsormore.
Someofthesetumorsareexquisitelypainfulandmaycausesevereradiatingneurogenicpain,
but others are completely painless. Schwannomas have been reported as a cause of tarsal
tunnelsyndrome.
27
Because ofprolonged growth ofthesetumors, theremaybelocal bonyimpingementand
boneremodelingduetopressurefromthetumor.However,thesebenigntumorsdonotinvade
thebone.MRIscansshowtypicalfeaturesforanindeterminatetumor,withlowsignalintensity
onT1-weightedsequencesandhighsignalintensityonT2-weightedsequences.Theanatomical
relationshipofthetumortothenerveoforiginmaybeobviousorsosubtleastobeimpossible
todetermine.
Because of theindeterminate MRI appearance of this tumor,a completeevaluation and
stagedbiopsyisrecommendedbeforedefinitivesurgicalremovalisplanned.Asmallnumber
oftumorscanbedefinitivelyidentifiedbasedonclinicalfindingsandMRIexamination.Ina
patient whodoesnothaveneurofibromatosis,thecombinationofatumorcausingsignificant
neurogenicpainandanMRIdemonstratingaclearanatomicalrelationshipbetweenthetumor
andaperipheralnerveisdiagnosticforbenignschwannoma/neurilemmoma.
Treatmentisby excisionwithamarginalmargin.Someofthesetumors havenoobvious
relationshiptoaperipheralnerve.Othersoccurinsidetheepineuriumofalargenervesuchas
the posterior tibial nerve.28 Intraneural schwannomas can usually be separated from the
surrounding normal nerve fibers without significant damage. Incomplete excision or
intralesional excision is acceptable for tumors that cannot be separated from a major
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peripheral nerve and where complete excision would cause significant morbidity and
permanentnervedamage.Recurrencefollowingresectionwithamarginalmarginisrare.
IntramuscularHemangioma
ClinicalPresentation
IMHstypicallypresentinthelowerextremitiesofchildrenandyoungadults.Thesevascular
lesions are divided into several types, according to their histologic features. Capillary
hemangiomaisthemostcommontype,consistingofsmallcapillariesthathavenormalsizeand
diameter,butthatareexcessiveinnumber.Cavernoushemangiomaismadeupoflargerdilated
blood vessels. Compound hemangiomas have features of both the capillary and cavernous
types.Lobularcapillaryhemangiomasaresmall,redbumpsoftenoccurringonthehands,face,
andarms, especiallyduring pregnancy. MostIMHs intheextremitiesare thecapillarytype.
IMHisabenignlesionwithaverylowriskofmalignantdegeneration.
IMHoccursinthelegmorecommonlythanthefoot.Patientspresentwithactivity-related
pain,nightpain,andlocalizedtendernessoverthelesion.Thetumorcancauseequinus,toe-
walking,and unilateral pes planus.29Lesions inthe heel maymimicplantar fasciitis. IMHs
neartheskinordermishaveavisiblebluecolor.
DiagnosticProcedure
Theappropriateage, location,history, andexaminationfindingsshould lead theclinicianto
suspectahemangioma.Phleboliths,whicharesmall,roundedcalcifiedintravascularthrombi,
areseenonplainfilmsin50%ofhemangiomas.
MRIscansareusuallyadequatetoconfirmthediagnosis.OnMRI,IMHisawell-defined
lobularheterogeneousintramuscularmass,isointenseonT1andhyperintenseonT2,withfat
andserpentinebloodvessels,containingsignalvoidsassociatedwiththephleboliths.TheMRI
appearance hasbeendescribed as a“bagof worms.” These lesionsare usuallydefiniteas
previouslydescribed,andmaybetreatedwithoutbiopsywhenthediagnosisiscertain.Itisnot
unusual for IMH to infiltrate both muscle and nearby structures, including skin, bone, and
neurovascularbundles.
In most cases, nonoperative treatment should be attempted. Activity restriction,
compression garments, nonsteroidal medications or acetaminophen are usually adequate to
control the symptoms. Ultrasound-guided or fluoroscopically guided sclerotherapy using
absolutealcoholorethanolamineoleatehasbeensuccessfullyemployedtotreatIMHinthe
footandankle.30Complicationsofsclerotherapyincludetendoncontracture,skinbreakdown,
reversiblenerveinjury,anddeepvenousthrombosis.
31
Localized, painful IMH that does not infiltrate critical structures can be treated with
surgery. Excision with an intralesional or marginal margin is effective for symptomatic
control.32Inpediatricpatients,IMHhasahighriskofrecurrence.Moreamplemarginsshould
beusedtolowertherateofrecurrenceandreoperations.33IMHmayextendintonearbytissues
suchasbone,skin,andvascularstructures.Resectionoftheaccessibleportionsofthelesion
combined with sclerotherapy, compressive garments, and medications is more suitable for
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complexlesions.
TumorMimics
Tumormimicsarenon-neoplasticlesions(nottruetumors)thatmaylookandactliketumors.
Gouty tophi, enlarged degenerated tendons, and focal collections of subcutaneous fat can
sometimesbemistakenfortumors.
GoutyTophi
Gout is an inflammatory arthropathy that occurs as a result of hyperuricemia. Gouty tophi
represent theaccumulationof calciumurate crystals withinsofttissue andbone. Tophi may
occurwithinthesubcutaneoustissues,synovium, subchondralbone,andtendons, resultingin
softtissuemasses,osseouserosion,tendonrupture,andtarsaltunnelsyndrome.Tophitypically
occuradjacenttothemetatarsalphalangealjointsandalongthelateralborderofthefoot.The
patient may not carry an established diagnosis of gout. In severe cases, there is usually a
historyofskinbreakdownandwhitepastymaterialdrainingfromthemass.Treatmentforgout
islargelymedical.Colchicineinhibitsthephagocytosisofuratecrystalsbypolymorphonuclear
cells and blocks the release of chemotactic factors. Nonsteroidal anti-inflammatory drugs
(NSAIDs) such as indomethacin can give patients relief from pain in 2 to 4 hours. Intraarticularsteroidinjectionscanalsogivereliefofacutegoutsymptoms.Extremelybulkygouty
tophicanbedebulked,butthe extensiveinfiltrationofcalcium uratecrystals withinthesoft
tissuesmakesitimpossibletoresectalltheabnormalmaterial.
Runner’sBump
Olderrunnerswhoaveragemorethan30milesperweekmaydevelopamassonthetibialis
anteriortendonsheaththatmaybemistakenforatumor.Thislesionisbecauseofdegeneration
ofthetibialisanteriortendonandafocaltenosynovialmassatthelocationofthedamage.On
examination,asoft,mobilelesionsurroundsthetibialisanteriortendonatthelevelwherethe
top of the shoelace rubs on the tendon. The tendon itself may retain normal caliber and
function, depending ontheseverity. Insome cases,the lesioncanbepartiallyeliminatedby
compressivemassage,butrecurspromptly.MRIshowsamildtomoderatedegree oftendon
diseasealongwithanintimatelyassociatedsynovialmass.Treatmentinvolvesrecognitionof
theoriginoftheproblem,educationoftherunner,andpadding,changing,orrepositioningthe
lacesandthetongueoftheshoetominimizetheforcesonthearea.Surgicaldebulkingofthe
excesssynoviumandtendonrepairshouldbereservedforseverecases.
LocalizedSinusTarsiFatCollection
Insomeoverweightorobeseindividuals,alocalized,well-definedsubcutaneouscollectionof
fat may occur anterior to the distal fibula in the area of the sinus tarsi. The patient is
asymptomatic, but maybe displeased withtheappearanceofthefatty collection. Themass
typicallymeasures 3 or 4 cm in proximal distal dimension and2 or 3 cm inmedial lateral
dimension,andconsistsofsoftcompressiblenontendersubcutaneousfat.Ifthereareatypical
featuresorclinical suspicion,an MRIis recommended. Thelesion consistsentirelyof fatty
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tissuethathasidenticalsignalcharacteristicstothatofnearbynormal subcutaneous fat.This
collectionofsubcutaneousfatisnotalipoma,andsurgicalremovalisnotrecommended.
SOFTTISSUESARCOMAINTHEFOOTANDANKLE
Introduction
Softtissuesarcomasareaheterogeneousgroupofmalignanciesthatarisefrommesenchymal
tissues. These rare tumors comprise less than 1% of all malignancies. Most sarcomas are
causedbymolecularanomaliesinthecellularDNA,suchaschromosomaltranslocationsand
mutations that activate, inhibit, or amplify the expression of the genetic material.
Environmental factors, familial cancer syndromes, herbicides suchas dioxin,radiation,and
immunedeficiencyarerarecausesofsarcomas.
34
Approximately8,700softtissuesarcomasarediagnosedperyearintheUnitedStates,with
anestimated10%oftheseoccurringinthedistallowerextremity.35Softtissuesarcomasare
morecommoninolder individuals.Approximatelyone-thirdofthese tumorsaresuperficial,
andthree-quartersarehistologicallyofhighgrade.Whenthesetumorsrecur,itismoreoftena
distal metastasis than a local recurrence.36 At least one-third of patients with soft tissue
sarcomaswilldieoftheirdisease.
TheAmericanJointCommitteeonCancer(AJCC)stagingsystemforsofttissuetumorshas
beenshowntohaveasignificantrelationshiptotheprognosis.Thestagingsystemincorporates
tumorsize,depth,thepresenceorabsenceofnodalordistantmetastasis,andhistologicgrade
(Table11-3).StagingisbasedondatafromtheCTorMRIofthetumoraswellasaCTofthe
chest.Thisinformationiscombinedwiththehistologicgrade,size,andtheintracompartmental
or extracompartmental extentofthe tumor. Prognosis is strongly related to grade, size,and
histologicsubtypeofsarcoma.
Treatmentofsofttissuesarcomasisdeterminedbythehistologicdiagnosisandstageofthe
tumor.Withrareexceptions,surgicalresectionwithawidemarginisthemosteffectivemethod
ofprimarytumorcontrol. Widemarginsreducethe chanceoflocal recurrence, whichhasa
strongnegativeimpactonsurvival.Limb-sparingsurgeryispreferable toamputationwhena
durable, functional, and pain-free extremity can be achieved. Because of the anatomical
constraintsofthefootandankle,itmaybedifficulttoachieveawidemarginwithoutresorting
to at least a partial amputation. Approximately 15% to 20% of patients with distal lower
extremitysarcomasrequiresomekindofamputation.
In adults with foot and ankle sarcoma, limb-sparing surgeryhas not been proventobe
betterthanamputationintermsoffunctional,psychosocial,orqualityoflifeoutcomes.37Limb
salvage is functionallysuperior to amputationinpediatric sarcoma patients. Thesoft tissue
coverage of the foot and ankle is scant and subjected to high loads and repetitive shear
stresses. Thebonyand articularelementsmustprovidedurablesupportandflexibilitywhile
withstandingthe stressesofweight-bearing. Limb-sparingsurgerymustbecarefullyweighed
againstotheroptionsfor tumorsofthefootandankle.38Tenpercentofpatients treatedwith
limb-sparingsurgeryareultimatelyamputatedfor reasons other than cancer.39Limbsalvage
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Table11-3.
forsarcomasinthe forefootusingfreevascularizedosteomyocutaneous fibularandscapular
grafts has beenreported to be successful. All patientsrequired multiple procedures due to
complications.40 In some series, less thanhalfthe patientstreatedwith limb salvageregain
normalfunctionalstatus.
38
PleomorphicSarcoma(PreviouslyIdentifiedasMalignantFibrous
Histiocytoma)
IntroductionandDefinition
PSisatermthatrecentlyreplacedMFH.MFHwas introducedasadiagnosis in1963, and
priortothattime,tumorsofthistypewereclassifiedasrhabdomyosarcomaorfibrosarcoma.
The classification continues to evolve, and this fact makes it difficult to interpret the
epidemiologic, clinical,andoutcomesdatafrom thepast.Thistumorisapleomorphichighgrade tumor of unknown origin composed of fibroblasts, myofibroblasts, and histiocytes.
PS/MFHisthemostfrequentsofttissuetumorinadultsinsomeseries.PS/MFHisfoundinthe
extremities70%to75% ofthetime,and50%ofallcases are inthelower extremity.Other
lesscommonsitesincludetheretroperitoneum,andtheheadandneck.Thehighestincidenceis
duringthefifthdecadeoflife,andthereisamaletofemaleratioof1.5:1.
IncidenceandDemographics
PS/MFHis secondarytoanotherprocess suchas radiation, surgery, fracture, osteonecrosis,
Pagetdisease,nonossifying fibroma, or fibrousdysplasia 20% ofthe time.PS/MFHarising
from a previous abnormality is usually more aggressive and has a poorer prognosis than
primaryPS/MFH.
TheAmericanJointCommitteeonCancerStagingSystem
AJCC,TheAmericanJointCommitteeonCancer;Superficial,abovethefascia;Deep,deeptofascia;G1,well
differentiated;G2,moderatelywelldifferentiated;G3,poorlydifferentiated;G4,undifferentiated.(AdaptedfromGreen
FL,PageDL,FlemingID,etal.AJCCCancerStagingHandbook.6thed.NewYork,NY:Springer-Verlag;2002:221–
225.)
SymptomsandPresentation
Clinically,PS/MFHpresentswithlocalpainandswelling.Thereisoftenahistoryofarapidly
enlargingmass.Itusuallypresentswithasofttissuemasswithorwithoutnearbyboneerosion.
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