Anders D, Kneitz H, Kunstmann E, Bröcker E-B, Benoit S
Klinik und Poliklinik für Dermatologie, Venerologie und Allergologie, Universität Würzburg, Josef-Schneider-Str. 2, 97080, Würzburg, Deutschland.
Hautarzt. 2012 Mar;63(3):226-9. doi: 10.1007/s00105-011-2213-y.
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.
穆尔-托雷综合征(MTS)是一种罕见的常染色体显性肿瘤综合征,其特征除了有内部肿瘤形成外,还会出现皮脂腺肿瘤和/或多发性角化棘皮瘤。皮肤肿瘤不仅包括皮脂腺腺瘤和皮脂腺瘤,还包括皮脂腺癌,其中超过50%的皮脂腺癌与结直肠癌相关,较少见的是与其余胃肠道、泌尿系统或生殖道的癌症相关。其潜在发病机制是DNA错配修复系统缺陷,导致肿瘤组织出现微卫星不稳定性。