Rev. bras. ortop. vol.51 número6

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SOCIEDADE BRASILEIRA DE ORTOPEDIA E TRAUMATOLOGIA

w w w . r b o . o r g . b r

Case

Report

Monostotic

fibrous

dysplasia

of

the

metacarpal:

a

case

report

夽

Kátia

Tôrres

Batista

∗

_,

_Hugo

_José

_de

_Araújo,

_Ulises

_Prieto

_y

_Schwartzman

HospitalSarahBrasília,CirurgiaPlásticaeCirurgiadeMão,Brasília,DF,Brazil

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Articlehistory:

Received27October2015

Accepted8December2015

Availableonline26October2016

Keywords:

Disease Metacarpus

Cysticfibrousosteitis

Fibrousdysplasiaofbone

a

b

s

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Fibrousdysplasiaisabonediseasecharacterizedbyabnormaldifferentiationoffibrous

tis-sueinthebones;itisoftenasymptomatic.Itmayaffectonebone(monostotic)orseveral

bones(polyostotic).Themonostoticformprimarilyaffectstheribs,buthardlyeveraffects

thehand.Itisimportanttomakethedifferentialdiagnosiswithmalignantbonetumors.

Thisarticledescribesthetreatmentandoutcomeofararecaseofapatientadmittedwith

ahistoryoftumorgrowthintherighthand,diagnosedasfibrousdysplasiaoftheright

sec-ondmetacarpal.Malepatient,14yearsofage,admittedtotheSarahHospitalwithlesion

onthedorsumoftherighthandwithoutpaincomplaints,previoushistoryoftrauma,nor

localsignsofinflammation.Physicalexaminationrevealedswellingonthedorsumofthe

secondmetacarpal,painless,withunalteredmobilityandsensitivity.Radiography,

com-putedtomography,andmagneticresonanceimagingindicatedtheinvolvementoftheentire

lengthofthesecondmetacarpal:onlythedistalepiphysiswaspreserved,withareasofbone

lysis.Afterbiopsyconfirmation,thepatientunderwentsurgery,usingalongcorticalgraft

forreconstructingthemetacarpal.Duringthefollow-upperiodoffiveyearstherewereno

signsofrecurrence,andproperdigitalgrowthandfunctionalityoftheoperatedhandwere

observed.

Ltda.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://

creativecommons.org/licenses/by-nc-nd/4.0/).

Displasia

fibrosa

monostótica

em

metacarpo

–

Relato

de

caso

Palavras-chave:

Doenc¸a Metacarpo

Osteítefibrosacística

Displasiafibrosaóssea

r

e

s

u

m

o

Adisplasiafibrosaéumadoenc¸aósseaquesecaracterizapeladiferenciac¸ãoanormalde

tecidofibrosonosossoseémuitasvezesassintomática.Podeacometerumosso

(monos-tótica)ou vários ossos (poliostótica). A formamonostótica acomete principalmenteas

costelas,masraramenteacometeamão.Odiagnósticodiferencialcomtumoresósseos

malignosé importante.Oartigo descreveotratamento eevoluc¸ãodeum casoraro de

夽

StudyconductedatHospitalSarah,Brasília,DF,Brazil.

∗ _{Corresponding}_author_.

E-mail:katiatb@terra.com.br(K.T.Batista).

http://dx.doi.org/10.1016/j.rboe.2016.10.008

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pacienteadmitidocomhistóriadecrescimentotumoralnamãodireita,naqualfoi

diag-nosticadadisplasiafibrosadosegundometacarpodireito.Pacientedosexomasculino,14

anos,admitidonoHospitalSarahcomlesãonodorsodamãodireita,semqueixaálgica,

antecedentetraumáticooualterac¸ãoflogísticalocal.Noexamefísico,apresentavaaumento

de volume no dorso do II _metacarpo, _indolor, _mobilidade _e _{sensibilidade} _inalteradas.

Foramfeitosexamesderadiografia,tomografiaeressonânciamagnética,evidenciou-se

ocomprometimentodetodaaextensãodosegundometacarpo;apenas aepífisedistal

estavapreservada,comáreasdeliseóssea.Fez-setratamentocirúrgicoapósabiópsiade

confirmac¸ão,comousodeenxertolongocorticalparareconstruc¸ãodometacarpo.Durante

otempodeseguimentodecincoanosnãoforamverificadossinaisderecidiva;adequado

crescimentodigitalefuncionalidadedamãooperadaforamobservados.

EditoraLtda.Este ´eumartigoOpenAccesssobumalicenc¸aCCBY-NC-ND(http://

creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction

Theterm fibrous dysplasiawas introducedby Lichtenstain

in1938todescribetheanomalousreplacementofmedullary

bonebyfibroustissue.Itisabenignlesionthatmayinvolve

one(monostotic)ormorebones(polyostotic),orbe

accom-paniedbyothersystemicalterationsandendocrinedisorders,

suchasintheMcCune–Albrightsyndrome.1,2_The_etiology_has

beendescribedasamutationinthegeneencodingthe

sub-unit␣oftheGsproteinlocatedonchromosome20q13.213.3

The natural history of this lesion depends on its

presen-tation; many lesions are asymptomatic, while others may

causepain,bonedeformity,fractures,functionalandcosmetic

changes,andmalignantdegeneration.Themonostoticform

occurswithgreaterpredilectionforthelongbones,ribs,and

radius;few caseshave been described inthe hand.1–4 _The

authorsdescribethetreatmentresultsandevolutionofarare

caseofmonostotic fibrousdysplasialocatedon thesecond

metacarpaloftherighthand.

Case

report

Amale14yearoldpatientwasadmittedtotheSarahHospital

with a lesion on the dorsum of the right hand withslow

progressionduringthecourseoftwoyears;thepatienthad

nopaincomplaints, noprevioushistory oftrauma, andno

localsigns ofinflammation.Physical examinationrevealed

painless swellingon the dorsumofthe second metacarpal

withunalteredmobilityandsensitivity.Radiography,CTscan

andmagneticresonanceimagingindicatedtheinvolvement

oftheentirelengthofthesecondmetacarpal:onlythedistal

epiphysis was preserved with areas of bone lysis.(Fig. 1).

Thelevels ofC3 199.0, C4 38.5, and alkaline phosphatase,

aswell asthechestX-raywerenormal.Thepatient

under-went anincisional biopsy disclosingfibrous dysplasia. The

patient then underwent general anesthesia, plexus block,

blodemptyingontheupperlimbusinganEsmarchbandage

and tourniquet positioning with pressure of 200mmHg,

and removalofthelesion onthe second right metacarpal.

Reconstruction was made using a 5-cm bone graft taken

fromtherightfibula.Themetacarpalphalangealjointofthe

second fingerwas preserved,withalocalmargin from the

edgeof1mm;thefibulagraftwasproximallyfixatedwithtwo

transcorticaltitanium screwsand distallywithtwocrossed

1-mmKirschnerwires(Fig.2).Theprocedurewasbloodless.

Thematerialwassenttoforanatomopathological

examina-tion,culture,andantibiogram.Prophylacticantibioticswere

administeredfor48h.Patientwassubmittedtoradiographic

postoperativecontrol(Fig.3)andimmobilizationwithcircular

antebrachiopalmarplasterforsixweeks.Kirschnerwireswere

removedaftersixweeksafterbonegrafthealing,when

phys-icaltherapyprogramwasinitiated.Thetranscorticalscrews

wereremovedafterfiveyears,duetolocalpaincomplaints.

Theresultofthehistopathologicalexamindicatedfibrous

dys-plasiawith46,XY,add(6)(q27),t(14:21)(q22;p1?11.2)[4]/46,XY[12]

karyotype(Fig.4).Follow-upcontinuedforfiveyearswithout

recurrence,showingnormalfunctionoftheoperatedhand.

Discussion

Fibrousdysplasiarepresents7%ofbenignbonetumors,and

itsexactetiologyisunknown.Themonostoticformismore

commonandtheradiographicfindingsarenonspecific.5–7

Theetiologyofthetumorremainsunclear,butitappears

tobelinkedtoasinglenucleotidemutationintheGs␣gene

onthelongarmofchromosome20(20q13.2-3),whichresults

inadisturbanceofthetissuedifferentiationprocess.1,8_This

mutationoccursinsomaticcellssometimeafterfertilization,

andthereforeisnotinherited.Chromosome12hasalsobeen

implicatedinthepathogenesisoffibrousdysplasia;however,

to date, no chromosomal abnormalities have been

consis-tently demonstrated.Thelesions inthe longbonesusually

appear in the metaphysis as an intramedullary expansion

withcortexthinningandhazyaspect;however,dependingon

theextentofthefibroustissueanddysplasticchangesinbone,

aswellasthedegreeofcalcification,thefindingsmay vary

from sclerotic toradiolucent.3,7 _Clinically,_these_lesions_are

eithercharacterizedbyvolumeexpansionorasymptomatic.

Asinseveraltumors,thedifferentialdiagnosisshouldinclude

sarcomas.

Radiographically, the differential diagnosis may include

Paget’s disease, solitarybone cysts, aneurysmalbone cyst,

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Fig.1–Physicalandradiologicalexaminationshowingthetumoronthesecondmetacarpal.

osteosarcoma,osteofibrousdysplasia,andgiantcelltumor.3

Theradiological findings suggestive ofmalignancy include

lyticregionsinpreviouslymineralizedareas,intralesional

cal-cification, periosteal reaction, cortical disruption, and soft

tissue invasion. Some aspects of these alterations were

observed preoperatively inthe present case.Moreover, the

needforpreoperativebiopsyforthediagnosisofbonetumors

shouldbeemphasized.

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Fig.3–Radiologicalfollow-upofthehandinthepostoperativeperiod.

Malignanttransformationoccurswithrapidbonegrowthin

approximately0.5%ofpatientswithmonostoticfibrous

dys-plasiaandin4%ofthosewithMcCune–Albrightsyndrome,1,2

withosteosarcoma beingthemostcommon.Other tumors,

suchasfibrosarcoma,chondrosarcoma,ormalignantfibrous

histiocytoma,mayalsobeobserved.Histologically,low-grade

osteosarcoma ismorecellular, moreatypical,and presents

moremitosis, havingahigher activitythan fibrous

dyspla-sia.Furthermore,theregularlyspacedbonyspiculesseenin

fibrousdysplasiaarenotpresentinosteosarcoma.2

Thetreatmentoffibrousdysplasiaforasymptomaticand

stablelesionsisregularfollow-up.Surgeryisindicatedonlyfor

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confirmationbiopsy,correctionofdeformities,non-operative

therapy failure, preventionofpathological changes,and/or

eradicationofsymptomaticlesions.1,9–11_In_cases_of_fractures,

thetreatmentcanbedonewithclosedfixation.Other

treat-mentoptionsincludecurettage,curettageplusbonegraft,or

internalfixation.9–11_More_extensive_cases_may_require_bone

graftorvascularizedbonegraft.12

In the present case, three important aspects should be

highlighted:thefirstistheoccurrenceofsecondmetacarpal

monostoticdysplasiaintheupperlimb,alesscommonarea;

thesecondistheimportanceofthedifferentialdiagnosiswith

other lesions, including malignant degeneration; the third

aspectisthetreatmentusingfreecorticalbonegraft,allowing

foradequatebonelengthofthefingerandnormalfunctionof

thehand.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

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