Muir-Torre综合症（MTS）是一种罕见的常染色体显性遗传病，由微卫星不稳定性引起，其原因是DNA错配修复基因的突变。这种疾病易于导致皮肤肿瘤和内脏恶性肿瘤，并可能在免疫受损或移植患者中发生。MTS需要对患者和家族进行密切的癌症监测，因为存在发展侵袭性内部恶性肿瘤和皮脂腺癌的趋势。免疫组化和/或遗传测试可以确认MTS的诊断。本综述提供了MTS指南、诊断和治疗的最新更新，同时提供了一个重要但不太知名的综合征的独特视角。©2023.作者（们），在Springer Nature Switzerland AG独家许可下。

Muir-Torre syndrome (MTS) is a rare autosomal dominant genetic condition resulting from microsatellite instability which is caused by mutations in DNA mismatch repair genes. This disorder predisposes individuals to skin tumors and visceral malignancies and may be precipitated in immunocompromised or transplant patients. MTS requires close cancer surveillance for the patient and family because of the tendency to develop aggressive internal malignancies and sebaceous carcinoma. Immunohistochemistry and or genetic testing can confirm the diagnosis of MTS. This review offers an update to the guidelines, diagnosis, and management of MTS while offering a unique perspective on an important but lesser-known syndrome.© 2023. The Author(s), under exclusive licence to Springer Nature Switzerland AG.