AuthorsBlogBoardReviewCommentContactUsNewslettersStatisticsTestimonialsSeeElsevier'snewvideoabouttheirClinicReviewArticlesonourhomepageorclickhere.JobsFellowshipsConferences/WebinarsBooksCMECasesAdvertiseGrantsMedicalNewsLibrariesRocheCompanionDiagnosticLibraryRocheNTRKIHCDiagnosticLibraryCOVID-19LibraryPandemicreliefmusicawardHome>Softtissue>EpithelioidsarcomaSofttissueUncertaindifferentiationEpithelioidsarcomaAuthors: FarresObeidin,M.D.,BorislavA.Alexiev,M.D.Editor-in-Chief: DebraL.Zynger,M.D.TopicCompleted:30March2020Minorchanges:20January2021Copyright:2002-2021,PathologyOutlines.com,Inc.PubMedSearch:Epithelioidsarcoma[title]Softtissuefreefulltext[sb]Pageviewsin2020:20,492Pageviewsin2021todate:6,798TableofContentsDefinition/general|Essentialfeatures|Terminology|ICDcoding|Epidemiology|Sites|Pathophysiology|Etiology|Clinicalfeatures|Diagnosis|Radiologydescription|Radiologyimages|Prognosticfactors|Casereports|Treatment|Clinicalimages|Grossdescription|Grossimages|Microscopic(histologic)description|Microscopic(histologic)images|Virtualslides|Cytologydescription|Positivestains|Negativestains|Electronmicroscopydescription|Molecular/cytogeneticsdescription|Samplepathologyreport|Differentialdiagnosis|Additionalreferences|Boardreviewstylequestion#1|Boardreviewstyleanswer#1|Boardreviewstylequestion#2|Boardreviewstyleanswer#2Citethispage:ObeidinF,AlexievB.Epithelioidsarcoma.PathologyOutlines.comwebsite.https://www.pathologyoutlines.com/topic/softtissueepithelioidsarcoma.html.AccessedApril13th,2021.Definition/generalAmalignantmesenchymalneoplasmthatexhibitsepithelioidcytomorphologyandapredominantlyepithelialphenotypeEssentialfeaturesMalignantepithelioidtumoroccurringinpediatricandadultpopulationsUnpredictableclinicalcourse;betterprognosisinpediatricpatientsTwotypicalmorphologies,includingclassictypewithepithelioidtospindledcellswithcentralpseudogranulomatousarchitecture;andproximaltypewithpredominantepithelioidandrhabdoidcellsIHC:Pancytokeratin+,INI1lostMolecular:MutationsinI