Sebaceous gland tumors and internal malignancy in the context of Muir-Torre syndrome. A case report and review of the literature

Abstract

Background: The Muir-Torre syndrome is a rare autosomal dominant condition and is currently considered a subtype of the more common hereditary nonpolyposis colorectal cancer syndrome, in which multiple primary malignancies occur together with sebaceous gland tumors.

Case presentation: We describe a case of a 62-year-old woman with three primary colorectal tumors, genital tumor, and sebaceous adenomas and present her family history of three generations. Our case represents the first case reported from Greece in the international literature.

Conclusion: Recognition of the syndrome in patients with sebaceous gland tumors should facilitate early detection of subsequent malignancies if the patient is entered into appropriate screening programs.

Figure 1

Condensed pedigree of four generations (I to IV) of the family. Patients II-2 and III-2 had four and three primary gastric and colorectal carcinomas respectively, as well as other forms of cancer. Squares: men, circles: women, diamonds: persons of either sex