Li-Fraumeni Syndrome | li-fraumeni syndrome

WhatisLi-Fraumenisyndrome?Li-Fraumenisyndromeisaconditionthatmakesapersonmorelikelytodeveloponeormorecancersintheseareasofthebody:BrainBreastsBonesBloodAdrenalgland(atinyorganthatislocatedontopofeachkidney)MusclesorconnectivetissuesPatientReferralsEmailThisPage RelatedTopicsCancerPredispositionProgramGeneticSyndromesDiseasesTreatedatSt.JudeLesscommonly,peoplewithLi-Fraumenisyndromedevelopothertypesofcancers,includingthoseaffectingtheseareasofthebody:SkinThyroidglandLungsDigestivesystem(esophagus,stomach,pancreasorcolon)Urinarysystem(kidney,bladder)Reproductivesystem(uterus,ovaries,prostate,gonads)WhatcausesLi-Fraumenisyndrome?Li-Fraumenisyndromeiscausedbychangesinageneknownas TP53.Genescarryimportantinformationthattellourbody’scellshowtofunction.The TP53 genecontrolshowcellsgrowanddivide.Oneofitsmainjobsistopreventcancersfromforming.Normally,yourcellscarrytwoworkingcopiesof TP53.Oneisinheritedfromyourmotherandonefromyourfather.CellsfrompeoplewithLi-Fraumenisyndromecarryoneworkingcopyof TP53 andonecopythatisaltered.Thisalterationcausesthegenetonotworkproperlyandiscalledamutation.Whentheremainingworkingcopyof TP53 becomesdamagedwithinacell,thiscanleadtoacancer.DownloadthisSummaryLi-FraumeniResourcesViewvideosfrompatientsandfamilieswhoaremanagingaLi-FraumeniSyndromediagnosis,featuredontogether.stjude.org/lfs.ViewVideosGeneticsHomeReference:Li-FraumeniSyndromeLi-FraumeniSyndromeAssociationLivingLFSAdditionalResourcesMakingSenseofYourGenes:AGuidetoGeneticCounselingYoungPeoplewithCancer:AParent’sGuideWhatisthecancerriskforchildrenwithLi-Fraumenisyndrome?Byage30,itisestimatedthathalfofallpeoplewithLi-Fraumenisyndromewilldevelopsomekindofcancer.Byage60,thelikelihoodofcancerforpeoplewithLi-Fraumenisyndromeincreasestoabout80%–90%.PeoplewithLi-Fraumenisyndromearemorelikelytodevelopmultiplecancersandcancerscausedbyradiation.BecauseofthewiderangeofcancertypesthatcanaffectchildrenwithLi-Fraumenisyndrome,itishardtopredictthekindsofcancerthatwilldevelop.Itisalsohardtoknowwhenthesec


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