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Файл:Ординатура / Хирургия / Библиотека им академика М.И. Перельмана / Книга_2821_Библиотеки_им_академика_М_И_Перельмана
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lesionaspossiblefollowedbyradiationtothetumorsitehasbeenassociatedwithacceptable
tumorcontrol. There is concern aboutsecondarymalignancyarising inirradiatedgiantcell
tumors.Avarietyofreconstructivemethodsareutilizeddependingontheextentofbonydefect,
ornoreconstructionmaybenecessary.Chemotherapyisnotused.
OutcomesofTreatmentandPrognosis
Following surgery, patients should be made aware ofthe ongoing risk of local recurrence.
Patientsshouldbefollowedonaregularbasisforthefirst2yearsatleast.Metastasistothe
lungsismorecommoninaxiallesions,locallyaggressivelesions,andrecurrentlesions.Local
recurrenceofgiant cell tumor should trigger a complete workup for metastasis for the lung
includingCTscanofthechest.
Osteochondroma
IntroductionandDefinition
Osteochondroma,orosteocartilaginousexostosis,isthemostcommonskeletalneoplasm.This
cartilage-capped subperiosteal bone projection accounts for 20% to 50% of benign bone
tumors and 10% to 15% of all bone tumors. The cause of solitary osteochondromas is
unknown. Hereditary multiple osteochondroma (HMO), also termed hereditary multiple
exostoses,isanautosomaldominantbonedisorderinwhichmostindividualsshowmutations
oftheEXT1orEXT2genesonchromosomes8and11.Thediseasemanifestationsencompass
multiplebenigncartilage-cappedtumors,shortstature,growthdisturbanceanddeformity,joint
dysfunction,prematureosteoarthritis, and malignant degenerationof the osteochondromas in
1%to4%ofindividuals.
59,60
Osteochondromascanbeeitherflattened(sessile)orstalk-like
(exostosis)andappearinajuxta-epiphyseallocation.Osteochondromascanoccurasaresult
ofradiationtherapyinchildren.Aftertheclosureofthegrowthplateinlateadolescence,there
isnormallynofurthergrowthoftheosteochondroma(Fig.11-2).
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FIGURE11-2.Asolitarypedunculatedosteochondromaonthemetatarsalofa17-year-oldgirl.Therehadbeenan
injurytotheareaatage12.
IncidenceandDemographics
The lesions occur only in bones that develop from cartilage (endochondral ossification).
Osteochondromasarefoundmostofteninlongbones,especiallythedistalfemurandproximal
tibia, with40% of thetumors occurring around the knee. The distal tibia and fibula are a
relatively common site. Osteochondroma is uncommon in the bones of the foot. It usually
occursintheforefootonametatarsal.Osteochondromasoccurmostfrequentlyinthefirsttwo
decadesoflife,witharatioofmalestofemalesof1.5:1.
SymptomsandPresentation
Clinically,osteochondromaspresentwithpainduetomechanicalirritationorapainlessmass.
Afracturecanoccurthroughthestalkofthelesion,whichalsocausespain.Osteochondromas
arerelativelycommononthedistalfibulaandtibia,wheretheycancausegrowthdisturbance,
mechanicalimpingement,recurrentsprains,anklestiffness,subluxationoftheankletendons,or
a palpable mass. In HMO, differential longitudinal growth of the tibia and fibula leads to
valgusdeformityoftheankle.PatientswithHMOmaypresentinearlyadulthoodwithankle
pain,instability,andvalgus.
X-rayAppearanceandAdvancedImagingFindings
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Plainfilmsarenormallyenoughtodiagnoseosteochondromas.Sessile lesionscoverawide
area andasaresultcausemetaphysealwideningora“trumpet-shapeddeformity”onX-ray.
Lesions with stalksare often foundmore distally. Thelesion appears as apedunculatedor
sessilematurebonymassonthesurfaceofthemetaphysealportionofthebone.Thecortexof
thelesioniscontinuouswiththenearbynormalcortex.CTscanscanbehelpful,becausethe
pathognomonic appearance ofthelesion canbeverified. TheCT scanshould show thatthe
cortex and medullary cavity of the underlying bone are continuous with the cortex and
medullary cavity of the lesion. MRI scans are helpful in evaluating the thickness of the
cartilagecap.Growthofthecartilagecaporgrowthofthelesionafterskeletalmaturityisa
worrisomefinding andshouldbeinvestigatedasapotentialsignofmalignant degeneration.
Therelationshipofthelesiontootherstructuresandthethicknessofthecartilagecapisbest
delineatedwithMRI.
HistopathologyFindings
Ongrossexamination,anosteochondromaisanirregularbonymasswithabluish-graycapof
cartilage.Opaqueyellowcartilagehascalcificationwithinthematrix.Thebaseofthelesion
hasarimofcorticalboneandcentralcancellousbone.Occasionally,abursadevelopsoveran
osteochondroma. Normally, the cartilage cap ranges from 1 to 6 mm thick. Over 2 cm of
cartilage thickness or renewed growth of a dormantlesionis a sign ofpossible malignant
transformation. Under themicroscope, an osteochondroma has endochondral ossificationon
the basal surface of hyalinecartilage so it resembles a normal, albeit disorganized growth
plate.Thebenigncartilageislessspatiallyorganized,hasbinucleatechondrocytesinlacunae,
andiscoveredwithathinlayerofperiosteum.
TreatmentOptionsforThisTumor
Thereisnotreatmentnecessaryforasymptomaticosteochondromas.Symptomaticlesionsmay
betreatedwithsimpleexcisionbyosteotomythroughthebaseofthelesion.Thesurgeondoes
not need to remove every vestige of the lesion to achieve resolution of the symptoms.
Treatmentshould bedelayeduntilskeletalmaturityinallcases. Atthattime the lesionsare
better defined and easier to remove, and the active cartilaginous portion of the lesion is
locatedfartherfromtheunderlyingbone.Iftheentireactivecartilaginousportionofthelesion
isnotremoved,recurrencemayfollow.Distalfibularandtibiallesionsoftenimpingeonthe
adjacenttibiaorfibulaandrequireremoval.
InHMO,multiplelesionsinthedistallegresultindifferentiallongitudinalgrowthofthe
tibiaandfibulaleadingtoanklevalgus.61Epiphysiodesisofthemedialmalleolusisperformed
to reduce or correct valgus. Supramalleolar osteotomy of the tibia for correction of ankle
valgusisperformedinsevereorneglectedcases.Aftertibialepiphysiodesisforcorrectionof
anklevalgus,removalofhardwarepriortoskeletalmaturityhasbeenassociatedwithrebound
ofvalgusdeformity.
62
OutcomesofTreatmentandPrognosis
Aslongas theentire cartilagecap isremoved,thereshould be norecurrence.Patientswith
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many,especiallylarge,osteochondromasshouldhaveregularscreeningexamsandradiographs
todetectmalignanttransformationearly.
Enchondroma
IntroductionandDefinition
Enchondromais a solitary, benign, intramedullary cartilagetumor that is often foundinthe
short tubular bones of the hands and feet, distal femur, and proximal humerus. The peak
incidenceisinthethirddecadeandisequalbetweenmenandwomen.
Multiple enchondromatosis is a nonheritable condition also known as Ollier disease.
Multiple enchondromas and hemangiomas of soft tissue constitute a condition known as
Maffuccisyndrome(Fig.11-3).
IncidenceandDemographics
Thepeakincidenceisinthethirddecadeandisequalbetweenmenandwomen.Itisthemost
commonprimarytumorinthehandandisnormallyfoundinthediaphysis.Thematurehyaline
cartilagelocatedcentrallywithinshorttubularbonesusuallypresentsclinicallyasafracture
due to an enlarging lesion. Enchondromas are also found incidentally in long bones and
undergomalignanttransformationinlessthan1%ofcases.Approximately8%ofthesetumors
occurinthebonesofthe foot.Thepeakageatdiagnosisis aroundthe middleofthe fourth
decade, but thetumor maypresentatvirtuallyany age. Enchondroma usuallyoccurs inthe
metatarsalsorphalangesofthelessertoes.Thehindfootisrarelyinvolved.
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FIGURE11-3.APandlateralradiographsshowinganexpansileenchondromaofthedistalphalanxina34-year-old
womanwithmildactivity-relatedpainandenlargementofthetoe.
SymptomsandPresentation
Mostpatientshavenosymptoms.Themostcommonpresentationisapatientwhohasinjured
their ankle or foot and who has an X-ray, leading to the discovery of the previously
asymptomaticlesion.Lesionsinthehandsandthefeetmayweakentheboneandcausepain,
swelling, and small pathologic fractures duringactivities. Patientspresentwith painduring
activities or after aninjury, butthere is rarelyany mass palpable on physical examination.
Occasionally, pathologic fracture through the lesion will cause the patient to seek medical
care.
X-rayAppearanceandAdvancedImagingFindings
Enchondromasareusuallyelongated,ovalwithwell-definedmargins,andmayhavepunctate
orringandarc-likecalcifications.Inlargerlesionsorinsmallerbones,thelucentdefecthas
endosteal scalloping and the cortex is expanded and thinned. Calcifications throughout the
lesion can range from punctate to rings. In the small bones of the foot, particularly the
phalanges,benignenchondromasmayhaveworrisomeX-rayfeaturesthatareassociatedwith
chondrosarcomainother locations.Inthephalanges,enchondromamaycausedramatic bone
expansion,bonedeformation,corticaldestruction,orasofttissuemassoutsidethebone.Ina
large bone, these X-ray findings would indicate that the tumor is likely to be malignant
chondrosarcoma,butinsmallbonesthesebehaviorsareconsistentwithabenigntumor.
CTis useful for detecting matrixmineralization andcortex integrity. MRI is helpful for
describing the nonmineralized portion of the lesion and visualizing any aggressive or
destructive features. Radiographic and imaging featuresofenchondroma thatare considered
worrisomebecauseofanobservedhigherincidenceofmalignancyincludelargesize,alarge
unmineralized component, significant thinning of the adjacent cortex,and bone scanactivity
greater than that ofthe anterior superior iliac spine.Features of enchondroma that are very
stronglyassociatedwithmalignanttransformationareprogressivedestructionofthechondroid
matrixbyanexpanding,nonmineralizedcomponent,anenlarginglesionassociatedwithpain,
oranexpansilesofttissuemass.
LaboratoryFindings
Nolaboratoryexaminationishelpful.
DifferentialDiagnosis
Othercartilaginouslesions,suchaslow-gradechondrosarcoma,CMF, andchondroblastoma,
andlesions withscattered densificationsuch as fibrous dysplasia should be considered. If
thereisdefinitegrowthofthelesion,adefinitepainfromthelesion,oralesioninalargebone
is actinginan aggressivemanner,such as damaging thecortex or breakingoutintothesoft
tissues,adifferentdiagnosisshouldbeconsidered.
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PreferredBiopsyTechniqueforThisTumor
Minimally invasive techniques are sufficient inlesionsthat are documentedtobe latent by
radiologicexamination.
HistopathologyFindings
On gross examination, an enchondroma consists of bluish-gray lobules of fine translucent
tissue.Thedegreeofcalcificationofthe lesiondeterminesiftheconsistencyisgritty.Under
the microscope,athinlayer oflamellarbonesurroundingthe cartilagenodulesisapositive
signthatthelesionisbenign.Atlowpower,therearelobulesofdifferentsizes.Bloodvessels
are surrounded by osteoid. Enchondromas have chondrocytes without atypia inside hyaline
cartilage.Thenucleiaresmall,round,andpyknotic.Thecellularityvariesbetweenlesionsand
within the same lesion. Each potential enchondroma needs to be evaluated for cellularity,
nuclear atypia, double nucleated chondrocytes,andmitotic activity ina viablearea without
calcificationstodistinguishitfromlow-gradechondrosarcoma. Smallperipherallesionsare
morelikelytobebenignthanlargeaxiallesions.Thepathologicdiagnosisissodifficultthatit
alwaysneedstobemadeinconjunctionwiththeradiologistandthesurgeon.
TreatmentOptionsforThisTumor
Asymptomatic,latentlesionsthatdonotcausebonefragilitymaybeobservedwithoutbiopsy.
This type is commonly encountered in thedistal femurandproximal humerus. In the small
bonesofthefoot,enchondromasaremorelikelytobecomesymptomaticduetoexpansionand
weakeningofthecortexandpathologicfracture.Painfulorproblematicenchondromascanbe
treated with simple curettage and packing with bone graft. Recurrence is rare. Extremely
expansile lesions may require complete excision and substitution of a structural allograft.
Large lesions in the distal phalanges that have dramatically expanded the bone should be
considered for partial amputation of the toe, because the functional and cosmetic result of
curettageandbonegraftingmaybeunacceptable.
All specimens must be analyzed carefully for malignancy. Small, peripheral cartilage
tumors tend to be benign, whereas large central cartilage lesions are more likely to be
malignant. Reliable differentiation of benign from malignant cartilage tumors is difficult.
Tumorsthatarelarger,tumorslocatedinthehindfootormidfoot,ornewtumorspresentingina
patient with aknownhistoryofenchondromatosis (Ollier disease)haveanincreasedriskof
malignancy.
PreferredMarginforThisTumor
Intralesional.
OutcomesofTreatmentandPrognosis
Removal ofenchondromais curative.The lesionsdo not grow; therefore, recurrence is not
expected.Recurrenceofthelesionsconsideredtobeanenchondromamaybeasignthatthe
lesionisactuallyalow-gradechondrosarcoma.
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SpecialandUnusualFeatures
MultipleenchondromatosisisanonheritableconditionalsoknownasOllierdisease.Multiple
enchondromasandhemangiomasofsofttissueareotherwiseknownasMaffuccisyndrome.In
bothconditions,menareaffectedmorethanwomen,andthediseaseprocessoftenonlyaffects
onesideofthebody.Inbothdiseases,thereisa30%riskofmalignanttransformationofthe
enchondromas.63 Chondrosarcoma is much more common in older patients, so large
enchondromasinolderindividualsdemandacarefulworkup.
ChondromyxoidFibroma
CMFisararebenigncartilagetumorthatalsohasmyxoidandfibrouselements.Itisextremely
uncommonandaccountsforlessthat1%ofallbonetumors.CMFhasapredilectionforthe
bonesofthelowerextremityandthefoot.64CMFisfoundmostofteninthemetaphysisaround
thekneeintheproximaltibia,proximalfibula,ordistalfemur.Recentstudieshavepointedto
anomaliesinchromosome6asapossiblegeneticfactorinCMF.Thebreakpointonthelong
armofchromosome6appearstoinvolveCOL12A1gene,acollagengenethatmayplayarole
inanothertumor,subungualexostosis(Fig.11-4).
IncidenceandDemographics
Itpresentsinthesecondtothirddecadeandhasamaletofemaleratioof2:1.Thisrarebenign
tumorhasapredilectionforthebonesofthetibiaandthefoot.Mostpatientsareyoungerthan
30.It ismorecommoninmales, typicallyinthe secondorthird decade oflife.Aboutonequarterofallofthesetumorsinvolvethefoot,withthemetatarsalsthemostcommonlocation.
SymptomsandPresentation
Theclinicalpresentationisusuallychronicpain,swelling,andpossiblyapalpablesofttissue
mass or restrictionofmovement. Only5% ofpatients with CMF presentwith a pathologic
fracture.
FIGURE11-4.Chondromyxoidfibromainthe5thmetatarsalofa15-year-oldboy.
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X-rayAppearanceandAdvancedImagingFindings
Radiologic findings demonstrate an eccentrically placed lytic lesion with well-defined
margins. It is an expansile, lobulated, lytic lesion in the metaphysis with partial cortical
erosionandlocalextensionintothesofttissue.Theremaybeascleroticmargin,andthelong
axisistypicallyparalleltothebone.Thelesionusuallyhasascleroticmarginofboneanda
lobulated contour. Ridges and grooves that appear in the margins secondary to scalloping
falselyappeartobetrabeculae.CThelpsdefinecorticalintegrityandconfirmsthatthereisno
mineralizationofthematrix,unlikeothercartilagetumors.CMFhasthesame appearanceon
MRI as other cartilage tumors, which is decreased signal on T1-weighted images and
increasedsignalonT2-weightedimages.MRIishelpfulinpreoperativeplanningandstaging.
DifferentialDiagnosis
Giant cell tumor, ABC, unicameral bone cyst (UBC), chondroblastoma, fibrous dysplasia,
osteosarcoma.
PreferredBiopsyTechniqueforThisTumor
Openincisional.
HistopathologyFindings
CMFresemblesfibrocartilagegrossly.Ithasasharpborderoftenwithanoutersurfaceofthin
boneorperiosteum.Theglisteninggrayish-whitelesionisfirmandlobulated.Itmayalsohave
small cystic foci or areas of hemorrhage. Histologically, CMF appears very similar to
chondrosarcoma. Theyaresocloseinhistology thatoftenradiology helpstomakethefinal
diagnosis. The predominant features of CMFare thezonal architectureand lobular pattern.
Nodulesofcartilagearefoundinbetweenfibromyxoidareas.Insomefields,theloosemyxoid
dominatesandinothersthedensechondroiddominates.Thechondrocytesareplumptospindly
inshapeandhaveindistinctcellbordersinsparselycellularlobulesofmyxoidorchondroid
matrix.Therearealsomorecellularzonesofthetumor,withsomegiantcellsattheedges.The
sharpbordersofeachlobuleandthelesionitselfhelptodifferentiateitfromchondrosarcoma.
TreatmentOptionsforThisTumor
Curettagemaybeadequate,butmayresultinlocalrecurrenceinasmanyasaquartertoonethirdofpatients.Unlikegiantcelltumor,thistumorhasapropensitytorecurduetoseedingof
the soft tissues during curettage. Local recurrence can be treated with meticulous repeat
curettageandexcisionoftheentiresofttissuemass.Marginalorwideexcisionandsubstitution
oftheinvolvedbone with a structural bone graftis usuallycurative.If possible, the initial
treatmentofCMFshouldfavorenblocexcision.
OsteoidOsteoma
IntroductionandDefinition
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Osteoidosteomaisabenignbonelesionwithanidusoflessthan2cmsurroundedbyazoneof
reactivebone.Thislesionaccountsforapproximately10%ofbenignbonetumors(Fig.11-5).
FIGURE11-5.Osteoidosteomaofthetalarneck.
IncidenceandDemographics
Thetumoroccursmostfrequentlyintheseconddecade,withapeakageintheearlytwenties,
andaffectsmalestwiceasoftenasfemales.Theproximalfemuristhemostcommonlocation
followed bythe tibia,posterior elements ofthespine,andthe humerus. Osteoid osteoma is
foundinthediaphysisorthemetaphysisoftheproximalendofthebonemoreoftenthanthe
distalend.Approximately11%ofthesepainful,benigntumorsoccurinthebonesofthefoot.
Theneckofthetalusisthemostcommonlocation.
SymptomsandPresentation
Patientspresentwithpersistentpainandswelling,whichisunrelatedtoactivity.Thepainmay
be more intense at night. In most cases, NSAIDs give substantial relief, and any historyof
takingthesemedicinesshouldbecarefullyreviewed.Thepainisoftendescribedasdulland
decreaseswithin20to30minutesoftreatmentwithNSAIDs.Ifthereisabsolutelynoreliefof
painfromtakingNSAIDs,thediagnosisofosteoidosteomaislesslikely.Lesionsadjacenttoa
joint may cause ankylosis or mimic a pauciarticular inflammatory arthritis, such as Reiter
disease.Localsymptomscanincludeanincreaseinskintemperatureandincreasedsweating
andtenderness.Epiphyseallesionscancauseabnormalgrowth.Thelocalswelling,erythema,
andtendernesscanmimicinfection.Whenthereissignificantinvolvementofanearbyjoint,the
relieffromNSAIDscanbelessdramatic.
X-rayAppearanceandAdvancedImagingFindings
Theclassicradiologicpresentationofanosteoidosteomaisaradiolucentnidussurroundedby
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a dramatic reactive sclerosis in the cortexof the bone.Thecenter canrange from partially
mineralizedtoosteolytictoentirelycalcified.Thelesioncanoccuronlyinthecortex,inboth
thecortexandmedulla,oronlythemedulla.Thereactivesclerosismaybepresentorabsent.
Thefourdiagnosticfeaturesinclude(1)asharproundorovallesionthat(2)islessthan2cm
in diameter, (3) has a homogeneous dense center, and (4) has a 1 to 2 mm peripheral
radiolucent zone. Bone scan shows a small, very intense focus of abnormal uptake. MRI
findingsarenonspecific.CTisthepreferredmethodofevaluation,especiallyifthelesionisin
the cortex or obscuredby reactive sclerosis. Characteristic plainradiograph,CT scan, and
bonescanfindingsaresufficienttoconfirmthediagnosisofthistumor.
DifferentialDiagnosis
Osteoblastoma, osteomyelitis (Brodie abscess), arthritis, stress fracture and enostosis/bone
island.
HistopathologyFindings
On gross examination,osteoid osteoma is a brownish-red, mottled, and grittylesion thatis
distinct from the surrounding bone. It can be present in the cortex or medullary canal.
Osteoclastsarepresent.Thenidusissurroundedbyscleroticbonewiththickenedtrabeculae.
Microscopically,thenidusconsistsofacombinationofosteoidandwovenbonesurroundedby
osteoblasts.Theoval-shapednidusiswellvascularizedandclearlyseparatefromthereactive
wovenorlamellarbone.
TreatmentOptionsforThisTumor
Surgicalremovalisnotmandatoryforthislesion.Patientswhosepainanddysfunctionrespond
welltoNSAIDsandaspirinmaybesuccessfullytreatedwiththesemedicationsuntilthelesion
disappears. Theaveragetime toresolutionis 22 months. Some patientswill not be able to
toleratemedicaltreatment,andrequestsurgicalremoval.Forthesecases,thegoaliscomplete
removal of the lesion by the least invasive means possible. Occasionally, the tumor is an
unexpectedfindingduringanarthroscopyoftheankle.Juxta-articularandsubperiosteallesions
ofthetalushavebeentreatedwithunplannedarthroscopicexcisionusingamotorizedshaver,
withvariableresults.
Forlesionsinthedistaltibiaandfibula,hindfoot,and midfoot,radiothermalablationby
CT-guidedneedleistherecommendedtechnique.65Duringradiothermalablation,thetipofa
radiofrequencygeneratorelectrodeisplacedintothecenterofthelesionunderCTguidance
andgeneralanesthesia.Aradiofrequencygeneratorformsanalternatinghigh-frequencyradio
wavethatpassesfromtheelectrodetipintothesurroundingtissue,whereenergyisdissipated
asheat.Thetissueitselfisheated,nottheradiofrequencyprobe.Aspherewithadiameterof1
cmcanbeeffectivelytreatedinthismanner,makingthistreatmentidealforosteoidosteoma.In
ordertobetreatedwithradialthermalablation,thediagnosisshouldbeconfirmedbasedonthe
imagingstudieswithahighdegreeofconfidence.Thereshouldbesufficientdistancebetween
the lesionand any major neurovascular structure. The lesionshould have a clearlyformed
niduslessthan1cminlargestdimension.Althoughradiothermalablationwaspreviouslyonly
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