THE MUIR-TORRE SYNDROME - A VARIANT OF HEREDITARY NONPOLYPOSIS COLORECTAL-CANCER SYNDROME

The Muir-Torre syndrome (MTS) is a variant of the hereditary nonpolypo sis colorectal cancer syndrome (HNPCC). The MTS has to be redefined cl inically to include all the potential cutaneous phenotypes of sebaceou s neoplasms including keratoacanthoma as the latter, MTS and HNPCC hav e all been identified with the genetic molecular findings of chromosom al abnormalities of DNA mismatched repair (MMR) sequences. Retrospecti ve pedigree investigations for systemic cancer occurrences and cutaneo us stigmata of MTS, followed by gene testing for abnormal DNA MMR, wil l identify kindreds and their members with high hereditary cancer risk s for HNPCC.