Epithelioid sarcoma of the vulva and deep pelvic soft tissue is a rare malignant neoplasm characterized by SMARCB1 / INI1 deletion · Most vulvar ...AuthorsBlogBoardReviewCommentContactUsNewslettersStatisticsTestimonialsSeeElsevier'snewvideoabouttheirClinicReviewArticlesonourhomepageorclickhere.JobsFellowshipsConferences/WebinarsBooksCMECasesAdvertiseGrantsMedicalNewsLibrariesRocheCompanionDiagnosticLibraryRocheNTRKIHCDiagnosticLibraryCOVID-19LibraryPandemicreliefmusicawardHome>Vulva,vagina&femaleurethra>EpithelioidsarcomaVulva,vagina&femaleurethraMesenchymalneoplasmsEpithelioidsarcomaAuthors: DavidB.Chapel,M.D.,JenniferA.Bennett,M.D.EditorialBoardMember: RicardoR.Lastra,M.D.Editor-in-Chief: DebraL.Zynger,M.D.TopicCompleted:30November2020Minorchanges:1February2021Copyright:2002-2021,PathologyOutlines.com,Inc.PubMedSearch:"Epithelioidsarcoma"vulvaPageviewsin2020:2,238Pageviewsin2021todate:668TableofContentsDefinition/general|Essentialfeatures|ICDcoding|Epidemiology|Sites|Pathophysiology|Clinicalfeatures|Diagnosis|Radiologydescription|Radiologyimages|Prognosticfactors|Casereports|Treatment|Clinicalimages|Grossdescription|Grossimages|Microscopic(histologic)description|Microscopic(histologic)images|Virtualslides|Cytologydescription|Positivestains|Negativestains|Electronmicroscopydescription|Molecular/cytogeneticsdescription|Molecular/cytogeneticsimages|Videos|Samplepathologyreport|Differentialdiagnosis|Additionalreferences|Boardreviewstylequestion#1|Boardreviewstyleanswer#1|Boardreviewstylequestion#2|Boardreviewstyleanswer#2Citethispage:ChapelDB,JenniferB.Epithelioidsarcoma.PathologyOutlines.comwebsite.https://www.pathologyoutlines.com/topic/vulvaepithelioidsarcoma.html.AccessedApril13th,2021.Definition/generalEpithelioidsarcomaofthevulvaanddeeppelvicsofttissueisararemalignantneoplasmcharacterizedbySMARCB1/INI1deletionMostvulvarandpelvicepithelioidsarcomasareoftheproximaltypeandshowmoreaggressiveclinicalbehaviorthandistaltype(classical)epithelioidsarcomaEssentialfeatures