[Muir-Torre syndrome]

The Muir-Torre syndrome (MTS) is an autosomal dominant disease defined by the coincidence of at least one sebaceous skin tumor and one internal malignancy. We describe an additional case and give a review of the literature. Over a period of 7 years, 19 skin tumors were excised in a 50 year old male patient. A total of 3 colonic carcinomas, one gastric carcinoma and one laryngeal carcinoma were operated successfully. The underlying defective mutation in the hMSH2 gene and the microsatellite instability were demonstrable. MTS is graded as a subgroup of hereditary non-polyposis colorectal cancer (HNPCC). In patients with MTS and with family members with known defective mutation, regular follow-up and search for new malignancies are mandatory.