InternationalJournalofSurgeryCaseReports27(2016)96–101 Contents lists available at ScienceDirect InternationalJournalofSurgeryCaseReports journal homepage: www.casereports.com Pulmonaryextraskeletalmyxoidchondrosarcoma:Acasereportand literaturereview RicardoBalanzá(MD) a ,RodrigoArrangoiz(MSMDFACS) b,c,* , FernandoCordera(MDFACS) b,c ,ManuelMu˜ noz(MDFACS) c , EnriqueLuque-de-León(MD) c ,EduardoMoreno(MD) c ,LourdesMolinar(MD) d , NicoleSomerville(MedicalStudent) e a GeneralSurgeryResident,HospitalÁngelesLomas,Av.VialidaddelaBarrancas/n,Col.ValledelasPalmasHuixquilucan,EstadodeMéxico,C.P.52763, Mexico b DepartmentofSurgery,HospitalÁngelesLomas,Av.VialidaddelaBarrancas/n,Col.ValledelasPalmasHuixquilucan,EstadodeMéxico,C.P.52763, Mexico c DepartmentofSurgery,CentroMédicoABC,Av.CarlosGraefFernández154,Col.Tlaxala,Del.CuajimalpadeMorelos,MéxicoDistritoFederal,C.P.05300, Mexico d DepartmentofPathology,HospitalÁngelesLomas,Av.VialidaddelaBarrancas/n,Col.ValledelasPalmasHuixquilucan,EstadodeMéxico,C.P.52763, Mexico e UniversidadAnáhuacNorte,Av.UniversidadAnáhuac46,Col.LomasAnáhuac,Huixquilucan,EstadodeMéxico,C.P.52786,Mexico articleinfo Articlehistory: Received21June2016 Accepted11August2016 Availableonline26August2016 Keywords: Extraskeletalmyxoidchondrosarcoma Lungneoplasm Pulmonarylobectomy FusiongeneEWSR1-NR4A3 Lungcancer abstract BACKGROUND:Extraskeletalmyxoidchondrosarcoma(EMC)accountsforthe3%ofallsofttissuesar- comasandit’scategorizedasatumourofuncertaindifferentiation.Thisentityhasshowntohavethe recurrentbalancedchromosomaltranslocationt(9;22)(q22;q12.2),whichleadstotheoncogenicfusion geneEWSR1-NR4A3.Thissarcomausuallypresentsasaslowgrowing,palpablemassintheextremities. EMCarisingfromthelungisextremelyinfrequent.Wereportonecaseofpulmonaryextraskeletalmixoid chondrosarcomaandareviewoftheworldliterature. CASEREPORT:A69-year-oldmalepatientpresentedwithintermittenthemoptysisforthelast6months.A PET/CTscanshowedahypermetabolicsolidmasswithlobulatedbordersofapproximately29×26mmin theinferiorrightlobe.Weperformedarightthoracotomywithinferiorlobectomyandlymphadenectomy oflevelsVII,VIII,X,andXIlevels.Theneoplasmwasconstitutedbycordsofsmallcellswithsmall roundnucleusandscarcecytoplasmimmerseinanabundantmyxoidmatrix.Theimmunophenotype waspositiveforMUM-1,CDK4,MDM2,andshowedfocalexpressionforS-100proteinandCD56.The finalpathologyreportrevealedapulmonaryextraskeletalmixoidchondrosarcoma.Nofurthersurgical interventionsoradjuvanttherapieswereneeded. CONCLUSION:EMCisanintermediate-gradeneoplasm,characterizedbyalongclinicalcoursewithhigh potentialforlocalrecurrenceanddistantmetastasis.TreatmentforEMCissurgicalandnon-surgicaltreat- mentisreservedforrecurrenceormetastaticdisease.Pulmonaryextraskeletalmyxoidchondrosarcoma isarareneoplasmwithonlyisolatedcasereportsfoundintheliterature. ©2016PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.Thisisanopenaccessarticle undertheCCBY-NC-NDlicense(http://creativecommons.org/licenses/by-nc-nd/4.0/). 1.Introduction Extraskeletalmyxoidchondrosarcoma(EMC)isararetumour ofuncertaindifferentiationfirstdescribedbyStoutandVernerin 1953[1,2].Theclinicopathologicalfeaturesofthissarcomawere notdescribeduntil1972byEnzingerandShiraki[3].EMChas * Correspondingauthorat:DepartmentofSurgery,HospitalÁngelesLomas,Av. VialidaddelaBarrancas/n,Col.ValledelasPalmasHuixquilucan,EstadodeMéxico, C.P.52763,Mexico. E-mailaddress:rodrigo.arrangoiz@gmail.com(R.Arrangoiz). showntohavetherecurrentbalancedchromosomaltransloca- tiont(9;22)(q22;q12.2),whichleadstotheoncogenicfusiongene EWSR1-NR4A3[4].Thischimericgeneactivatesthetranscription oftargetgenesinvolvedincellproliferationandhasbeendetected inapproximately65%ofthecases[5].Histologically,EMChas amarkedphenotypicplasticitythatoverlapswithmesenchymal malignancies,vagueresemblancetohumancartilageandanuncer- tainhistiogenesis[6].ImmunohistochemicalstudiesinmostEMC arepositiveforvimentinandfocallypositiveforS100protein[7]. Thissarcomapredominantlyaffectsmenbeyondtheirfifthdecade andnormallydoesnotbehaveinanaggressiveway,nevertheless ithasahighrateoflocalrecurrenceanddistantmetastases[5]. http://dx.doi.org/10.1016/j.ijscr.2016.08.025 2210-2612/©2016PublishedbyElsevierLtdonbehalfofIJSPublishingGroupLtd.ThisisanopenaccessarticleundertheCCBY-NC-NDlicense(http://creativecommons. org/licenses/by-nc-nd/4.0/).