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.