Printable version | Epithelioid sarcoma pathology
SurgicalPathologyCriteriauseyourbrowserbackarrowtoreturnEpithelioidSarcomaDefinitionKeratinpositivesofttissuetumorcomposedoflargeepithelioidcellsalmostalwaysinvolvingdistalextremities(seealsoproximaltypeepithelioidsarcoma)Alternate/HistoricalNamesClassicaltypeepithelioidsarcomaLargecellepithelioidsarcomaDiagnosticCriteria95%keratinpositiveCD3450%AlmostalwaysinvolvesdistalextremitiesofyoungadultsandchildrenPoorlycircumscribed,oftenmultinodularSeparateandfusednodulesandsheetsofcellsFrequentcentralnecrosisMayappearhistologicallyasnecroticgranulomasBrightlyeosinophiliccollageninandaroundnodulesMediumtolargeepithelioidandspindledcellsRarelypredominantlyspindledAbundantbrightlyeosinophiliccytoplasmFrequentlywithsharpcellbordersNucleimaybeeccentricModeratenuclearpleomorphismMitoticfiguresinfrequenttonumerousOccasionalfeaturesPerineuralinvasionVascularinvasionMyxoidareasorextensivehyalinizationCalcificationorossificationHemorrhageCystformationSmallcellpatternwithscantcytoplasmFibroma-likevariantPurespindledcellsOtherthanthename,classicaltypeepithelioidsarcomasharesonlythedistinctiveimmunophenotypeofproximaltypeepithelioidsarcoma.RichardLKempsonMDRobertVRouseMDDepartmentofPathologyStanfordUniversitySchoolofMedicineStanfordCA94305-5342Originalposting::July27,2007Latestupdate:December12,2008 SupplementalstudiesImmunohistologyCD3450%Keratin95%LowmolecularweightkeratinCK8ispositivein95%Highmolecularweightkeratinsrareto50%CK722%andCK200-15%INI1lossin91%EMA95%Vimentin>95%CA12590%Actin(MSAorSMA)30-40%Desmin15-70%S100RareCD31NegativeFactorVIIINegativeHigherrateofdesminreactivityreportedinproximaltypediseaseGeneticstudyLossofheterozygosityonchromosome22hasbeendetectedinsomecasesDifferentialDiagnosisPalisadednecroticgranulomaSquamouscarcinomaSweatglandcarcinomaSynovialsarcomaMelanomaEpithelioidhemangioendotheliomaSclerosingepithelioidfibrosarcomaIschemicfasciitisCentralnecrosisofepithelioidsarcomamaysimulategranulomatousdisease,infectiousorautoimmunePalisadedNecroticGranulomaEpithelio
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