Polyposis syndromes include: Familial adenomatous polyposis, MUTYH-associated polyposis, Serrated polyposis syndrome, Peutz-Jeghers syndrome, Juvenile ...SkiptomaincontentYouarehereHomeArchiveVolume10, Issue4PracticalmanagementofpolyposissyndromesEmailalertsArticleTextArticlemenuArticleTextArticleinfoCitationToolsShareResponsesArticlemetricsAlertsPDFColorectalReviewPracticalmanagementofpolyposissyndromesFreeRoshaniPatel1,2,http://orcid.org/0000-0002-9489-3190WarrenHyer11PolyposisRegistry,StMark'sHospital,Harrow,UK2ImperialCollegeLondonDepartmentofSurgeryandCancer,London,UKCorrespondencetoDrWarrenHyer,PolyposisRegistry,StMark'sHospital,Harrow,UK;polyposiswg{at}drhyer.co.ukAbstractHereditaryboweltumoursareusuallypartofadistinctsyndromewhichrequiremanagementofbothintestinalandextra-intestinaldisease.Polyposissyndromesinclude:Familialadenomatouspolyposis,MUTYH-associatedpolyposis,Serratedpolyposissyndrome,Peutz-Jegherssyndrome,JuvenilepolyposissyndromeandPTEN-hamartomatoussyndromes.Ofallcolorectalcancers(CRC),5%–10%willbeduetoanunderlyinghereditaryCRCsyndrome.Diagnosisandmanagementofpolyposissyndromesisconstantlyevolvingasnewscientificandtechnologicaladvancementsaremadewithrespecttoidentifyingcausativegenesandincreasedsophisticationofendoscopictherapytotreatpolyps.This,inadditiontodatayieldedfrommeticulousrecord-keepingbypolyposisregistrieshashelpedtoguidemanagementinwhatareotherwiserelativelyrareconditions.Thesedatahelpguideclinicalmanagementofpatientsandtheir‘at-risk’relatives.Diagnosisisbothgeneticwherepossiblebutclinicalrecognitioniskeyintheabsenceofanidentifiablecausativegene.Furthermore,somesyndromescanoverlapwhichcanadditionallycomplicatediagnosis.Theprinciplegoalsofpolyposismanagementarefirsttomanageandtreatthepresentingpatientandthentoidentify‘at-risk’patients,throughscreeningandpredictivegenetictesting,endoscopicsurveillancetoallowtherapyandguidesurgicalprophylaxis.Duetothecomplexityofdiagnosisandmanagement,patientsandtheirfamiliesshouldbereferredtoageneticsce