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1、CHAPTER33SOFTTISSUESARCOMASSamuelSingerMostcasesofsofttissuesarcomaarethoughttobesporadicdistribution,age,andcausesandtheircauseisunknown.Inrarecases,geneticfactors,envi-moleculargeneticsronmentalfactors,priorradiationtherapy,viralinfections,andevaluationimmunodefic
2、iencyhavebeenassociatedwiththedevelopmenthistologicgradingandprognosticfactorsforofsarcoma.Inaddition,sarcomashavebeenreportedtoariseinoutcomescartissue,fracturesites,oranatomicregionsassociatedwithstagingpriorsofttissuetrauma.Geneticsyndromessuchasneurofibro-manage
3、mentmatosis,familialadenomatouspolyposis,andtheLi-Fraumenisummarysyndromehaveallbeenshowntobeassociatedwiththedevelop-mentofsofttissuesarcoma.Desmoidtumorsoccurinpatientswithfamilialadenomatouspolyposis,adisordercausedbytheSofttissuesarcomasarerareandunusualneoplasm
4、s,accountinggermlineadenomatouspolyposiscoli(APC)genemutation.forapproximately1%ofadulthumancancersand15%ofMalignantperipheralnervesheathtumorsdevelopinabenignpediatricmalignancies.Sarcomasaffectmorethan10,600nervesheathinapproximately2%ofpatientswithneurofibro-pati
5、ents/yearintheUnitedStatesandapproximately4000matosis.TheLi-Fraumenisyndromeisarare,highlypenetrant,patientswilldieeachyearfrominoperableformsofsofttissuefamilialcancerphenotypeusuallyassociatedwithgermline12sarcoma.Sarcomascontinuetocarrybiologicandclinicalinter-mu
6、tationsinthep53tumorsuppressorgene.Ofpatientswithestandsignificancedisproportionatetotheirclinicalfrequencythissyndrome,80%developcancerbyage45andsofttissueorbecauseoftheiroftenclearlydefinedmoleculargeneticsandthebonesarcomasofdiversehistologyariseastheindextumorin
7、vastexpansionofcytogeneticandmoleculargeneticinformation36%ofpatients.Heritableretinoblastomagene(RB1)muta-thathasbeendiscoveredoverthepast10years.Softtissuesar-tionsareassociatedwithanincreasedriskofboneandsofttissuecomasmaydevelopinanyanatomicsiteandthisfeature,sa
8、rcoma.Forexample,patientswithRB1mutationshavea36%togetherwiththemorethan50histologictypesandsubtypes,cumulativeincidenceover50yearsofdevel
