[Muir-Torre syndrome and Turcot syndrome].
Fiche publication
Date publication
février 2017
Journal
Annales de dermatologie et de venereologie
Auteurs
Membres identifiés du Cancéropôle Est :
Pr CRIBIER Bernard
Tous les auteurs :
Velter C, Caussade P, Fricker JP, Cribier B
Lien Pubmed
Résumé
Lynch syndrome (LS) is a syndrome that carries a genetic predisposition to certain cancers associating, either in a single individual or in a family, a visceral tumour, mainly colorectal, with a high risk of other synchronous or metachronous cancers. LS is linked with mutations in the genes coding for proteins in the DNA repair system. Phenotypic variants of SL exist, including Muir-Torre syndrome (MTS) and Turcot syndrome (TS), both of which predispose to colorectal cancer. They may be distinguished by the presence of benign or malignant sebaceous tumours in MTS, and tumours of the central nervous system in TS.
Mots clés
DNA mismatch repair genes, Génétique, Instabilité des microsatellites, Microsatellite instability, Muir-Torre syndrome, Sebaceous gland neoplasms, Syndrome de Muir-Torre, Syndrome de Turcot, Tumeurs sébacées, Turcot syndrome
Référence
Ann Dermatol Venereol. 2017 Feb;: