Extraocular sebaceous carcinoma in a patient with Muir-Torre syndrome

BACKGROUND. Sebaceous carcinoma is a rare, aggressive neoplasm that arises from the adnexal epithelium of sebaceous glands and is commonly associated with Muir-Torre syndrome. OBJECTIVE. The metastatic potential of extraocular sebaceous carcinoma warrants a thorough evaluation to establish the extent of disease. METHODS. We describe a 55-year-old man who presented with an asymptomatic abdominal wall mass 3 years after definitive diagnosis of Muir-Torre syndrome. RESULTS. A biopsy of the surgical specimen revealed sebaceous carcinoma. CONCLUSION. Dermatologists are crucial to the early recognition and diagnosis of extraocular sebaceous carcinoma. In our patient with documented Muir-Torre syndrome, continued surveillance allowed for prompt recognition and treatment of this associated cutaneous malignancy.