title: [Muir-Torre syndrome. An interdisciplinary challenge].
abstract: Muir-Torre syndrome (MTS) is a rare autosomal dominant tumor syndrome characterized by the occurrence of tumors of the sebaceous glands and/or multiple keratoacanthomas in addition to internal neoplasia. Skin tumors include not only sebaceous adenomas and sebaceomas but also sebaceous carcinomas which are associated with colorectal carcinomas in over 50%, less commonly with carcinomas of the remaining gastrointestinal, urinary or genital tract. The underlying pathogenesis is a defect of the DNA mismatch repair system introducing microsatellite instability in tumor tissue.
authors.affiliation: Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universität Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Deutschland. Anders_D@klinik.uni-wuerzburg.de
authors.forename: D
authors.initials: D
authors.lastname: Anders
authors.forename: H
authors.initials: H
authors.lastname: Kneitz
authors.forename: E
authors.initials: E
authors.lastname: Kunstmann
authors.forename: E-B
authors.initials: EB
authors.lastname: Bröcker
authors.forename: S
authors.initials: S
authors.lastname: Benoit
mesh_terms: D001706:Biopsy; D002869:Chromosome Aberrations; D003299:Cooperative Behavior; D053843:DNA Mismatch Repair / Q000235:genetics; D003937:Diagnosis, Differential; D005799:Genes, Dominant / Q000235:genetics*; D006801:Humans; D033183:Interdisciplinary Communication; D008297:Male; D053842:Microsatellite Instability; D008875:Middle Aged; D055653:Muir-Torre Syndrome / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000235:genetics / Q000473:pathology; D012867:Skin / Q000473:pathology