Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans.docVIP

Fibrosarcomatous changes and expression of CD34+ and apolipoprotein-D in dermatofibrosarcoma protuberans.doc

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FibrosarcomatouschangesandexpressionofCD34andapolipoprotein-Dindermatofibrosarcomaprotuberans

Palmerinietal.ClinicalSarcomaResearch2012,2:4 /content/2/1/4 CLINICAL SARCOMA RESEARCH RESEARCH OpenAccess Fibrosarcomatouschangesandexpressionof CD34+andapolipoprotein-Din dermatofibrosarcomaprotuberans EmanuelaPalmerini1*,MarcoGambarotti2,EricLStaals3,LiccianaZanella2,GabrielaSieberova2,AlessandraLonghi1, MarilenaCesari1,StefanoBonarelli1,PieroPicci4,PietroRuggieri3,MarcoAlberghini2andStefanoFerrari1 Abstract Background:Dermatofibrosarcomaprotuberans(DFSP)isarelativelycommonsoft-tissuetumor.Amore aggressiveappearingfibrosarcomamayariseinDFSP,changingitsbiologicalbehavior.CD34andapolipoprotein-D arehighlyexpressedinDFSP,buttheirprognosticsignificanceisuncertain. Methods:DFSPandfibrosarcomatous-DFSP(FS-DFSP)patientsreferredtoourinstitutebetween1982and2009 wereidentified.Fibrosarcomatouschanges,expressionofCD34andapolipoprotein-Dwereevaluated. Results:40patients,(medianage43years,55%males)wereidentified.Tumorwaslocatedinthelimbsin60%,in thetrunkin40%.Thirty-sevenpatientshadlocalizedand3hadmetastaticdisease.Thirteen(32%)patientswereFS- DFSP.Allbutoneunderwentsurgerywithadequatesurgicalmarginsin72%.7FS-DFSPreceivedalsoradiotherapy (RT).Chemotherapywasadministeredto3patientswithFS-DFSP.Withamedianfollow-upof49months,the5-OS was90%.Localrecurrenceratewas23%:42%FS-DFSP,15%DFSP.MetastasesdevelopedinthreeFS-DFSPpatients. The5-yearEFSwas70%inlocalizedpatients.Histology(DFSP75%vs.FS-DFSP52%,p=0.002),surgicalmargins (adequate74%vs.inadequate55%,p=0.02),site(limb47%vs.trunk100%),CD34expression(CD34positive:70% vs.CD34negative:33%,p=0.05),andapolipoprotein-Dexpression(Apo-Dpositive:73%vs.Apo-Dnegative:33%, p=0.02)influencedthe5-yearEFS,whereassex,useofRTornumberofprevioussurgicaltreatmentsdidnot. Conclusions:PatientswithDFSPhaveahighsurvivalprobability.Site,adequatesurgicalmargins,presenceofthe fibrosarcomatouscomponent,lackofCD34expressionandapolipoprotein-Dinfluenceoutcome. Keywords:dermatofibrosarcomaprotuberans,softtissuesarcoma,Apolipoprotein-D,CD34 Background shapedcells

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