Turcot syndrome is one of the variations in polyposis syndromes. It is characterized by multiple colonic polyps and an increased risk of colon and primary brain ...×ArticlesLogInCasesSignUpCoursesQuizBlogDonateAbout×MenuSearchADVERTISEMENT:Radiopaediaisfreethankstooursupportersandadvertisers.BecomeaGoldSupporterandseenoads.ArticlesCasesCoursesLogInLoginSignup{"url":"/signup-modal-props.json?lang=us\u0026email="}ArticlesCasesCoursesQuizAboutBlogGoad-freeSearchTurcotsyndromeDrAmmarHaouimi◉andAssocProfFrankGaillard◉◈etal.Turcotsyndromeisoneofthevariationsinpolyposissyndromes.Itischaracterizedbymultiplecolonicpolypsandanincreasedriskofcolonandprimarybraincancers.EpidemiologyTurcotsyndromeisararedisease.Patientstypicallypresentintheseconddecade3.PathologyTurcotsyndromeischaracterizedby:intestinalpolyposisCNStumors:mostcommonlyglioblastoma ormedulloblastomaGeneticsItisthoughttocarryanautosomalrecessiveinheritance. Two-thirdsofpatientshavemutationsintheAPCgene,thesamegeneticdefectasinfamilialadenomatouspolyposis(FAP).Thesepatientshavemultiplecolonicadenomas,andvirtuallyalldevelopcolorectalcarcinomabytheageof40.Thecommonintracranialtumorinthissubtypeismedulloblastoma. TheotherthirdhavemutationsintheHNPCCgenes.Colonicmalignancyisnotascommoninthistypebuttendstodevelopatayoungerage.Mostdevelopglioblastomas.HistoryandetymologyItisnamedafterJacquesTurcot(1915-1977)5, Canadiansurgeon. References1.RobbinsSL,KumarV,AbbasAKet-al.RobbinsandCotranpathologicbasisofdisease.W.B.SaundersCompany.(2010)ISBN:1416031219.ReaditatGoogleBooks-FinditatAmazon2.RadinDR,FortgangKC,ZeeCSet-al.Turcotsyndrome:acasewithspinalcordandcolonicneoplasms.AJRAmJRoentgenol.1984;142(3):475-6.AJRAmJRoentgenol(citation)-Pubmedcitation3.HarnedRK,BuckJL,OlmstedWWet-al.Extracolonicmanifestationsofthefamilialadenomatouspolyposissyndromes.AJRAmJRoentgenol.1991;156(3):481-5.AJRAmJRoentgenol(abstract)-Pubmedcitation4.KoontzNA,HessCP.AJRteachingfile:braintumorinapatientwithfamilialadenomatouspolyposis.AJRAmJRoentgenol.201