A MUIR-TORRE-SYNDROME FAMILY

The Muir-Torre syndrome is a rare autosomal-dominant disease involving sebaceous neoplasms as markers for multiple internal malignancies. Di agnostic criteria include at least one sebaceous gland adenoma, epithe lioma, or carcinoma and at least one internal malignancy. The world li terature contains 162 cases with 316 internal malignancies. Colorectal and urogenital malignancies predominate, and nearly half the patients had two or more internal cancers. The discovery of a Muir-Torre syndr ome-associated sebaceous lesion is rare and should prompt an evaluatio n for internal malignancies. We report a family over five generations displaying this syndrome. The proband is a 44-year-old man with two sk in and two colon malignancies who presented to our clinic with the chi ef complaint of an infected sebaceous cyst. The world literature is re viewed, and an emphasis on the surgeon's role in evaluation and treatm ent is discussed.