EpithelioidsarcomaFromWikipedia,thefreeencyclopediaJumptonavigationJumptosearchEpithelioidsarcomaMicrographofanepithelioidsarcoma.H&Estain.SpecialtyOncology Epithelioidsarcomaisararesofttissuesarcomaarisingfrommesenchymaltissueandcharacterizedbyepithelioid-likefeatures.Itaccountsforlessthan1%ofallsofttissuesarcomas.ItwasfirstclearlycharacterizedbyF.M.Enzingerin1970.[1]Itcommonlypresentsitselfinthedistallimbs(fingers,hands,forearms,orfeet)ofyoungadultsasasmall,softmassoraseriesofbumps.Aproximalversionhasalsobeendescribed,frequentlyoccurringintheupperextremities.[2]Rarecaseshavebeenreportedinthepelvis,vulva,penis,andspine.Histologically,epithelioidsarcomaformsnoduleswithcentralnecrosissurroundedbybland,polygonalcellswitheosinophiliccytoplasmandperipheralspindling.[3]Epithelioidsarcomastypicallyexpressvimentin,cytokeratins,epithelialmembraneantigen,andCD34,whereastheyareusuallynegativeforS100,desmin,andFLI1(FLI-1).[3]TheytypicallystainpositiveforCA125.[4]Epithelioidsarcomamostcommonlystrikesyoungadults,yetnoagegroupisimmune.Thediseasehasatendencytodeveloplocalrecurrencesandmetastasisthereaftertoregionallymphnodes,lung,bone,brain,andotherlocations,includingthescalp.[3]Generallyspeaking,epithelioidsarcomahasahighrateofrelapseafterinitialtreatmentandtendstorecurlocally(atorneartheoriginaltumorsite).Epithelioidsarcomaalsodemonstrateslymphaticspread(in22-48%ofcases),andmetastasis(in21-63%ofcases).[5]Theseevents,aswellasadvancedstage(progression)andgrade(aggressiveness),arepredictiveofanoverallworseoutcome.Theoverallfive-yearsurvivalrateforepithelioidsarcomaisanywherefrom25to78%.[5]Importantly,the10-yearand15-yearsurvivalratedropsoffsignificantly.[6][7]Associatedwithamorepositiveoutcomeareyoungerage,femalevs.malesex,distalvs.proximallocation,smallertumorsize,andnegativemarginsupontumorresection.[1][7][8]Contents1Signsandsymptoms2Genetics3Molecularbiology3.1VEGF3.2MET3.3SonichedgehogandNotch3.4mTOR3.5EGFR3.6CD1093.7CyclinD14Diagnosis4.1Staging5Treatment6Prognosis7Research7.1C