Definition of Muir-Torre syndrome

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Muir-Torre syndrome: A genetic syndrome characterized by a combination of sebaceous tumors of the skin and one or more internal malignancies, most often colon cancer. The types of sebaceous skin neoplasia include sebaceous adenoma, sebaceous epithelioma, sebaceous carcinoma, and keratoacanthoma.

The Muir-Torre syndrome is inherited in an autosomal dominant manner. It is due to mutation in the mismatch repair genes responsible for the hereditary nonpolyposis colon cancer (HNPCC) and is now sometimes considered part of HNPCC.