Li-Fraumeni Syndrome | li-fraumeni syndrome

Li-Fraumeni syndrome (LFS) is a cancer predisposition syndrome associated with high risks for a diverse spectrum of childhood- and ...NCBISkiptomaincontentSkiptonavigationResourcesHowToAboutNCBIAccesskeysMyNCBISignintoNCBISignOutNCBIBookshelf.AserviceoftheNationalLibraryofMedicine,NationalInstitutesofHealth.AdamMP,ArdingerHH,PagonRA,etal.,editors.GeneReviews®[Internet].Seattle(WA):UniversityofWashington,Seattle;1993-2021.GeneReviews®[Internet].ShowdetailsAdamMP,ArdingerHH,PagonRA,etal.,editors.Seattle(WA):UniversityofWashington,Seattle;1993-2021.GeneReviewsbyTitleSearchtermGeneReviewsAdvancedSearchHelpLi-FraumeniSyndromeKatherineSchneider,MPH,KristinZelley,MS,KimENichols,MD,andJudyGarber,MD,MPH.AuthorInformationKatherineSchneider,MPHDanaFarberCancerInstituteBoston,MassachusettsEmail:ude.dravrah.icfd@redienhcs_enirehtakKristinZelley,MSChildren'sHospitalofPhiladelphiaPhiladelphia,PennsylvaniaEmail:ude.pohc.liame@kyellezKimENichols,MDStJudeChildren'sResearchHospitalMemphis,TennesseeEmail:[email protected],MD,MPHDanaFarberCancerInstituteBoston,MassachusettsEmail:ude.dravrah.icfd@rebrag_ydujInitialPosting:January19,1999;LastUpdate:November21,2019.Estimatedreadingtime:30minutesSummaryClinicalcharacteristics.Li-Fraumenisyndrome(LFS)isacancerpredispositionsyndromeassociatedwithhighrisksforadiversespectrumofchildhood-andadult-onsetmalignancies.ThelifetimeriskofcancerinindividualswithLFSis≥70%formenand≥90%forwomen.FivecancertypesaccountforthemajorityofLFStumors:adrenocorticalcarcinomas,breastcancer,centralnervoussystemtumors,osteosarcomas,andsoft-tissuesarcomas.LFSisassociatedwithanincreasedriskofseveraladditionalcancersincludingleukemia,lymphoma,gastrointestinalcancers,cancersofheadandneck,kidney,larynx,lung,skin(e.g.,melanoma),ovary,pancreas,prostate,testis,andthyroid.IndividualswithLFSareatincreasedriskforcancerinchildhoodandyoungadulthood;survivorsareatincreasedriskformultipleprimarycancers.Diagnosis/testing.ThediagnosisofLFSisestablishedinaproban


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