MUIR-TORRE SYNDROME - DIAGNOSTIC-CRITERIA AND REVIEW OF THE LITERATURE

We report on a 63-year-old female patient with Muir-Torre syndrome (MT S). In the course of this disease two carcinomas of the colon, a kerat o-acanthoma and multiple sebaceous gland tumours, including four sebac eous carcinomas, appeared. This case is thought to be a heriditary for m as one of daughters was also found to have a sebaceous epithelioma. MTS is a mostly autosomal-dominant disease with the association of seb aceous gland tumours and internal carcinomas. As the malignant tumours only show slight aggressiveness the prognosis is quite favourable. Or al isotretinoin therapy was successfully used for the inhibition of se baceous gland proliferation. A narrower defination is presented and an updated survey of the published cases is given. Furthermore, the hist opathologic peculiarities of sebaceous gland tumours, especially of se baceous gland carcinomas, are discussed and compared to sebaceous glan d tumours not connected with MTS. A total number of 100 of the 135 pub lished cases of MTS were included and analysed regarding sebaceous gla nd tumours and other skin tumours. The data on internal carcinomas wer e taken from the work of Cohen et al. (1991) and 11 current cases were added.