GENITOURINARY TUMORS IN MEN WITH THE MUIR-TORRE SYNDROME

The Muir-Torre syndrome is an autosomal dominant genodermatosis charac terized by the occurrence of one or more sebaceous gland tumors (eithe r adenoma, epithelioma, or carcinoma) and a single malignant internal neoplasm. To date, 133 cases of Muir-Torre syndrome have been describe d Colorectal (49%) and genitourinary (21%) carcinomas are the most com mon initial neoplasms. The case of a man with a history of recurrent o cular sebaceous carcinoma who subsequently had seminoma is described, and previously published reports of men with Muir-Torre syndrome and g enitourinary tumors are discussed. Including this report, an associate d genitourinary tumor was the initial malignant internal neoplasm in 1 1 men with Muir-Torre syndrome. The detection of a genitourinary neopl asm preceded diagnosis of the patient's Muir-Torre syndrome-associated sebaceous gland tumor in five patients (45%). All patients with sebac eous gland tumors of the type associated with Muir-Torre syndrome warr ant consideration of Muir-Torre syndrome and appropriate workup to det ect asymptomatic malignant visceral neoplasms.