Sebaceous carcinoma is a rare, aggressive adnexal tumor of unknown etiology; 75% of these tumors arise in the periocular region while 25% arise at extraocular sites. This cutaneous tumor is a diagnostic sign of Muir-Torre syndrome, a disorder associated with visceral malignancies and gene abnormalities. Patients with sebaceous carcinoma should therefore be carefully evaluated; a detailed personal and family history of cancer, a thorough physical examination, additional tests where appropriate, and close monitoring will all be required. We report 2 cases of extraocular sebaceous carcinoma and review the literature, focusing on the association between sebaceous carcinoma and Muir-Torre syndrome.
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