Histology and prognosis in rhabdomyosarcoma: a report of the Intergroup Rhabdomyosarcoma Study [Abstract]. Proc SIOP 1981; 13: 113. Google ...SkiptomaincontentThankyouforvisitingnature.com.YouareusingabrowserversionwithlimitedsupportforCSS.Toobtainthebestexperience,werecommendyouuseamoreuptodatebrowser(orturnoffcompatibilitymodeinInternetExplorer).Inthemeantime,toensurecontinuedsupport,wearedisplayingthesitewithoutstylesandJavaScript.AdvertisementnaturemodernpathologyreviewarticlesarticlePathologicClassificationofRhabdomyosarcomasandCorrelationswithMolecularStudiesDownloadPDFAbstractRhabdomyosarcoma,themostcommonsoft-tissuemalignancyofchildhoodandadolescence,comprisesagroupofdifferingpathobiologicentitieslinkedbytheircommonpropensityforformationofneoplasticskeletalmuscle,afeaturethatresultsfrombiologicalforcesrelatedtoaberranttranscriptionsignalsandtheresultantproductionofmyogenicproteins.Atamolecularlevel,however,rhabdomyosarcomasformaheterogeneousgroupthatcanbesubdividedintodistinctclinicopathologicentitiesbasedonmorphologicappearanceandgeneticmakeup.Thesedifferingmorphologicfeatureswererecognizedinthemid-1900sbyHornandEnterlinewiththeirdivisionofrhabdomyosarcomasintoembryonal,alveolar,botryoid,andpleomorphicsubtypes.Morerecenthistologicandbiologicstudieshaveresultedindescriptionofadditionalentities,suchasspindlecellandanaplasticrhabdomyosarcoma,andrefinementsinrecognitionoftheoriginalentities,suchassolid-alveolarrhabdomyosarcoma.Familiaritywithnewerclassificationsandtheirrelationshiptomolecularaberrationsiskeytostratifyingpatientsoncurrenttherapeuticprotocolsandproposedinnovativegenetictherapies.DownloadPDFINTRODUCTIONAlthoughrelativelyrareinadults,rhabdomyosarcomasarethemostcommonsoft-tissuemalignancyinchildrenandadolescents,inwhomtheycompriseapproximately60%ofsarcomasreportedperannum(1).Inmanyways,thehistologyoftheseneoplasmsisanalogoustomyogenesisinthedevelopingembryo,yieldingcluestothebiologyoftheselesions.Forthisreason,itismorecorrecttodefinerhabdomyosarcoma