RhabdomyosarcomaHighQualityPathologyImagesofSoftTissue:MyogenicofRhabdomyosarcomaAdvertisementFollowus:        11065Images:LastWebsiteUpdate:March2,2021HomeAboutUsAcknowledgementsFeedbackContactUsSiteMapHighQualityPathologyImagesofBenignandMalignantNeoplasmsandRelatedEntitiesJanuary 2020RhabdomyosarcomaReviewers:JayalakshmiVenkateswaran,MD;DharamRamnani,MD|HomeSoftTissueMyogenicRhabdomyosarcomaRhabdomyosarcomas(RMS)aremalignantsofttissuetumors,exhibitingskeletalmuscledifferentiation.Thereisabimodalagedistribution,between2-6yearsandasecondpeakbetween10-18years;itisuncommonafter45years.Commonlocationsincludeheadandneck(26%),genitourinarytract(17%)andextremities(15%).Therearefourmajorsubtypes-Embryonal,Alveolar,Pleomorphic,&SpindleCell/Sclerosing.SarcomabotryoidesisavariantofEmbryonalRMS.Grossly,theirsizeisvariableandtheymaybewell-circumscribedandmultinodularorlesswelldefinedwithinfiltrativeborders.Sectioningrevealsglistening,gelatinous(mucoid)orgray-whiteorpink-tanfleshyandbulgingappearance.Areasofcystformation,hemorrhageornecrosismaybepresent.Microscopically,thetumorcellsrangefromsmallprimitivehyperchromaticround/spindle/stellatecellstolargedifferentiatedstrap/ribbon/tadpole/racket-shapedcellswithabundanteosinophiliccytoplasmandcross-striations(rhabdomyoblasts).Thenucleiarelarge,round,andvesicularwithaprominentnucleolus.Immunohistochemically,theyarepositiveforMyoD1,MyogeninA,MuscleSpecificActin(MSA)orDesmin.Translocationt(2;13)(q35;q14)isfoundinthemajorityofalveolarRMScasesandat(1;13)(p36;q14)isnotedinasmallersubset.Thecongenital/infantileformofsclerosing/spindlecellRMShasgenefusionsinvolvingVGLL2andNCOA2orCITED2genes.UsuallyRMSistreatedwithsurgeryandchemotherapy,withorwithoutradiationtherapy.Thehistologictypeandlocationofthetumorinfluencesurvival.ThebotryoidvariantofembryonalRMShasthebestprognosis,whilethepleomorphicsubtypeisoftenfatal.References:1)Goldblum,J.R.,Weiss,S.W.,&Folpe,A.L.(2019).Enzinger&Weiss'sSoftTissueTumors-SeventhEdition.Philadelphia,PA.Elsevier.2)G