Rev. bras. ortop. vol.52 número3

r e v b r a s o r t o p . 2017;52(3):366–369

SOCIEDADE BRASILEIRA DE ORTOPEDIA E TRAUMATOLOGIA

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Case

report

Sarcomatous

transformation

of

a

hemosiderotic

fibrohistiocytic

lipomatous

tumor:

a

case

report

夽

Renata

Margarida

Etchebehere

,

Elia

Cláudia

Souza

Almeida

,

Carlos

David

Teixeira

Santos

_,

_Adilha

_Misson

_Rua

_Micheletti

_,

Antônio

Sebastião

Leitão

a_{UniversidadeFederaldoTriânguloMineiro(UFTM),HospitaldeClínicas,Servic¸odePatologiaCirúrgica,Uberaba,MG,Brazil}

b_{ConsultoriaemPatologia,Botucatu,SP,Brazil}

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Articlehistory: Received3March2016 Accepted14July2016

Availableonline16November2016

Keywords: Hemosiderin Histiocytes Adiposetissue Connectivetissue Sarcoma

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Hemosiderotic fibrohistiocytic lipomatous tumors are rare neoplasms that were first describedin2000.Initiallyconsideredabenignlipotamouslesionofthesofttissues, nowa-daystheyareconsideredtobealocallyaggressivetumor.Theyoccurmainlyinthefootand ankleofwomenintheirfifthandsixthdecades,althoughtheymaybefoundinanyplace inthelowerlimbsand,morerarely,inotherpartsofthebody.Histologically,hemosiderotic fibrohistiocyticlipomatoustumorsconsistofamixtureofmatureadiposetissue,fusiform cell fascicles, macrophages thatoften contain cytoplasmic hemosiderin, mononuclear inflammatoryinfiltrate,andstromathatmaybefocallymyxoid.Localrecurrenceisobserved innearlyone-thirdofallcases.Thereisnoconsensusintheliteraturewhetherthistumor isapartofaspectrumthatcomprisespleomorphichyalinizingangiectatictumorsand myxoinflammatoryfibroblasticmalignanttumors,orifitisanindependententity.

Theauthorsreportacaseofaneoplasiaafteradiagnosisofahemosiderotic fibrohistio-cyticlipomatoustumorina38-year-oldwoman,withtworecurrencesandlatersarcomatous transformation.Animmunohistochemicalstudyindicatedmyofibroblasticdifferentiation ofamalignantneoplasm.Tothebestoftheauthors’knowledge,thereareonlyfewreported casesofmalignanttransformationinhemosideroticfibrohistiocyticlipomatoustumors.

©2016SociedadeBrasileiradeOrtopediaeTraumatologia.PublishedbyElsevierEditora Ltda.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http:// creativecommons.org/licenses/by-nc-nd/4.0/).

夽

WorkperformedintheHospitaldeClínicas,UniversidadeFederaldoTriânguloMineiro(UFTM),Uberaba,MG,Brazil. ∗ _{Correspondingauthor.}

E-mail:renataetch@hotmail.com(R.M.Etchebehere).

http://dx.doi.org/10.1016/j.rboe.2016.11.002

rev bras ortop.2 0 1 7;52(3):366–369

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Transformac¸ão

sarcomatosa

de

tumor

lipomatoso

fibrohistiocítico

hemosiderótico:

relato

de

caso

Palavras-chave: Hemosiderina Histiócitos Tecidoadiposo Tecidoconjuntivo Sarcoma

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Ostumoreslipomatososfibrohistiocíticoshemosideróticossãoneoplasiasrarasqueforam descritaspelaprimeiraveznoano2000.Inicialmenteconsideradaumalesãolipomatosa benignadostecidosmoles,atualmenteéconsideradoumtumorlocalmenteagressivo.Estas lesõesocorremprincipalmentenopéenotornozelodasmulheresnaquintaesextadécadas devida,emborapossamserencontradosemqualquerlugarnosmembrosinferiorese,mais raramente,emoutraspartesdocorpo.Histologicamente,tumoreslipomatosos fibrohisti-ocíticoshemosideróticossãoformadosporumacombinac¸ãodetecidoadiposomaduro, fascículosdecélulasfusiformes,macrófagosquecontêmfrequentementehemosiderina citoplasmática,infiltradoinflamatóriomononucleareestroma,quepodeserfocalmente mixoide.Hárecidivalocalemquaseumterc¸odoscasos.Nãoexisteconsensonaliteratura seestetumorfazpartedeumespectroqueenvolveostumoresangiectásicoshialinizantes pleomórficoseostumoresfibroblásticosmixoinflamatóriosmalignos,ouseconfigurauma entidadeindependente.

Relatamosumcasodeumaneoplasiaapósodiagnósticodetumorlipomatoso fibro-histiocíticohemosideróticoemumamulherde38anos,comduasrecorrênciaseposterior transformac¸ãosarcomatosa.Umestudoimuno-histoquímicoindicoudiferenciac¸ão miofi-broblástica de uma neoplasia maligna. Para nosso conhecimento, há apenas poucos casosrelatadosdeumatransformac¸ãomalignaemtumoreslipomatososfibrohistiocíticos hemosideróticos.

©2016SociedadeBrasileiradeOrtopediaeTraumatologia.PublicadoporElsevier EditoraLtda.Este ´eumartigoOpenAccesssobumalicenc¸aCCBY-NC-ND(http:// creativecommons.org/licenses/by-nc-nd/4.0/).

Introduction

Hemosideroticfibrohistiocyticlipomatous tumor(HFLT)isa rareneoplasmthatwasinitiallythoughttobeareactive pro-cesssecondarytotraumaoranexcessivetissueresponseto chronicvenousstasis,whichwouldstimulatethe prolifera-tion ofelements seen inthe lesion.1 _{Browne and Fletcher} (2006)publishedaclassicreviewofthistumorinwhichdefined the clinical and histological characteristics.2 _{Initially, HFLT} wasbelievedtobeabenignlipomatousneoplasm.The con-temporaryunderstandingisthatHFLTisanunencapsulated, locallyaggressiveneoplasmthatiscomposedofadipocytes, hemosiderin-ladenspindlecellsandmacrophages,aswellas scatteredchronicinflammatorycells.Thecommonestsitefor HFLTisthedorsumofthefoot,followedbyotherankleand footsites,thedorsumofthehand,and,finally,thecalf,thigh, andcheek.HFLTismostfrequentlyfoundinwomenintheir fifthorsixthdecadeoflife.1–6

Radiologicimagesofthelesionrevealadiposetissue,often withseptation,andfeaturesmimickinglipomaorlow-grade sarcoma.7_{ClinicalandpathologicalfeaturesofHFLTare} sim-ilar to those ofpleomorphic hyalinizing angiectatic tumor (PHAT)andmyxoinflammatoryfibroblasticmalignanttumor (MIFS),leadingauthorstospeculatethatHFLTmaybeapart of a spectrum involving these two other tumor types.8–10 HFLTdoesnotproducemetastasesandisofuncertainlineage accordingtothesoft-tissuetumorclassificationsystemofthe WorldHealthOrganization.1

Therearefewreportsofthemalignanttransformationof HFLT into MIFS.9,10 _{Herein,wereporta publishedcaseofa} lesion thathad previouslybeen diagnosed,according toits morphologicalandbyimmunohistochemical(IHC)features, asHFLT.8_{Thelesionrecurredtwiceandlatershowed} malig-nanttransformationtoalow-gradeunclassifiedpleomorphic sarcoma,withanIHCstudysuggestingmyofibroblastic differ-entiation.

Case

report

ThiscasereportwasapprovedbytheEthicsCommitteeata tertiarypublichospital.A38-year-oldfemalepatientcameto ourclinicinDecemberof2010.Shecomplainedofapainful lesioninthebackareaofherleftthigh,inthevicinityofprior tumorsurgicalresections (performedinSeptemberof2006 and Januaryof2008).Previousresectionsledtoadiagnosis ofHFLT, basedonmorphologicalexaminationand comple-mentaryIHCstudy.Thepriorbiopsieswerereviewed,andthe diagnosiswasmaintained.ThiscaseofHFLThasbeen previ-ouslyreportedintheliterature.8

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Fig.1–(A)Hemosideroticfibrohistiocyticlipomatoustumors(HFLT)area(hematoxylin–eosin–HE,100×);(B)malignant

transformationareaofHFLTshowingincreasedcellularityandnuclearpleomorphism(HE,100×);(C)nuclearatypiainthe

malignanttransformationareaofHFLT(HE,400×).

tissuewereobtainedforhistologicalevaluation.Tissueshad hemorrhagicfocijointlymeasuring22cm×18cm×11cmand

weighing485g.

Microscopyshowedaninfiltrative,pleomorphicmalignant neoplasm,withirregularedgesinthesofttissuesand vari-ablecellularity.Theneoplasmwascomprisedofround,oval, and fusiformcells, withhyperchromaticnuclei, sometimes bizarreandeosinophiliccytoplasms,andraremitoticfigures, compatiblewithsarcomatoustransformationoftheHFLT.We alsonotedareaswithmatureandoftenhistiocyticadipose tissuecontaininghemosiderin(Fig.1).Thisfindingsuggested thatthesewereareasofaresidualHFLT.Nonecroticfociwere visible.

OnIHCanalysis,thelesionwasfocallypositiveforsmooth muscle actin and desmin, but negative for S100 protein and CD34,suggesting myofibroblasticdifferentiation ofthe sarcomatouscomponent.Acellproliferationindexof approx-imately5%(Ki67)wasobserved.Amputationwasproposed tothe patient, who refused thistreatment and underwent chemotherapyandradiotherapyatotherhospital.

Discussion

Wecomparedfindingsfrom theprevious resections,which yieldedadiagnosis ofHFLT,withthe currentmorphology,2 whichexhibitedmalignantfeatures.Thiscomparisonallowed ustoconcludethatthelesionhadundergonemalignant trans-formationtoalow-gradeunclassifiedpleomorphicsarcoma, withIHCfeaturessuggestingmyofibroblasticdifferentiation. Thepresenceofareaswithabenignpatternsimilartothe ini-tiallesionintermixedwithpleomorphicandinfiltrativeareas, aswellastheproliferativeindex,reinforcedthisconclusion.

Reportshavedescribed the presenceofgene rearrange-ments in tumors showing mixed features, which strongly suggestedarelationshipofHFLTwithPHATandMIFS.Previous authorshaveconcludedthatHFLT,PHAT,andMIFSrepresent morphologicvariantsofasingleentity,inwhichonlythelater hasacquiredthecapacitytometastasize.3,8,9_One histologi-calstudysuggestedthatmost,ifnotall,tumorsdiagnosedas PHATsmightactuallyrepresentexamplesofMIFSwith aber-rantangiectatichyalinizedvessels.Furthermore,thevascular changesinPHATmayrepresentonlyahistologicpatternand

notatrueneoplasm.Thus,MIFSandmostcasesofPHATmay represent different morphologic manifestations ofa single entity.10

Inthisreport,wepresentacaseofHFLTwithmalignant transformation.Thetumorretainedareassimilartothe origi-naltumor,reinforcingthepossibilitythatthisentityispartof aspectrumoflesionswiththepotentialformalignant evolu-tion.AftersurgicalresectionofHFLT,patientsshouldundergo rigorous andregular follow-upbecauseofthepossibilityof sarcomatoustransformation.

Conflicts

of

interest

Theauthorsdeclarenoconflictsofinterest.

Acknowledgements

WethankDrChristopherDMFletcherandDrFredEllingerfor reviewingthiscase.

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