What is juvenile polyposis syndrome?Juvenile polyposis syndrome (JPS) is a hereditary condition that is characterized by the presence of hamartomatous ...SkiptoContentSearchMenuYouarehereHome>TypesofCancer>JuvenilePolyposisSyndromeRequestPermissionsJuvenilePolyposisSyndromeApprovedbytheCancer.NetEditorialBoard,11/2020Whatisjuvenilepolyposissyndrome?Juvenilepolyposissyndrome(JPS)isahereditaryconditionthatischaracterizedbythepresenceofhamartomatouspolypsinthedigestivetract.Hamartomasarenoncancerous(benign)massesofnormaltissuethatbuildupintheintestinesorotherplaces.Thesemassesarecalledpolypsiftheydevelopinsideabodystructure,suchasthestomachorintestines.Theterm“juvenilepolyposis”referstothetypeofpolyp(juvenilepolyp)thatisfoundafterexaminationofthepolypunderamicroscope,nottheageatwhichpeoplearediagnosedwithJPS.PolypsmayfrequentlydevelopinapersonwithJPSbyage20.Thenumberofpolypsapersonhasduringhisorherlifetimecanrangefromaround5tomorethan100.Mostjuvenilepolypsarenoncancerous,butthereisanincreasedriskofcancerofthedigestivetract,suchasstomach,smallintestine,colon,andrectumcancers,infamilieswithJPS.JPSissuspectedwhenaperson’ssymptomsandfamilyhistoryfitanyofthefollowingcategories:Morethan5juvenilepolypsofthecolonand/orrectumMultiplejuvenilepolypsthroughoutthedigestivetractAnynumberofjuvenilepolypsandafamilyhistoryofjuvenilepolypsWhatcausesJPS?JPSisageneticcondition.Thismeansthattheriskforpolypsandcancercanbepassedfromgenerationtogenerationinafamily.Basedoncurrentresearch,2geneshavebeenlinkedtoJPS.TheyarecalledBMPR1AandSMAD4.AgeneticalterationthatdisruptsthefunctionofeithertheBMPR1AgeneortheSMAD4genemakesapersonmorelikelytodevelopjuvenilepolypsandcancerofthedigestivetractoverhisorherlifetime.Thistypeofchangetoagenecanalsobecalledageneticmutation,genealteration,pathogenicorlikelypathogenicgermlinevariant,oradisruptivegenechange.NotallfamiliesthathaveJPSwillhaveidentifiablegeneticalterationsinBMPR1AorSMAD4.OthergenesarebeingstudiedregardingtheirlinktoJPS.HowisJPSinherited?Normall