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Genitourinary tumors in men with the Muir-Torre syndrome

1995; Elsevier BV; Volume: 33; Issue: 5 Linguagem: Inglês

10.1016/0190-9622(95)90435-2

1097-6787

David A. Davis, Philip R Cohen,

Cancer Genomics and Diagnostics

The Muir-Torre syndrome is an autosomal dominant genodermatosis characterized by the occurrence of one or more sebaceous gland tumors (either adenoma, epithelioma, or carcinoma) and a single malignant internal neoplasm. To date, 133 cases of Muir-Torre syndrome have been described. Colorectal (49%) and genitourinary (21%) carcinomas are the most common initial neoplasms. The case of a man with a history of recurrent ocular sebaceous carcinoma who subsequently had seminoma is described, and previously published reports of men with Muir-Torre syndrome and genitourinary tumors are discussed. Including this report, an associated genitourinary tumor was the initial malignant internal neoplasm in 11 men with Muir-Torre syndrome. The detection of a genitourinary neoplasm preceded diagnosis of the patient's Muir-Torre syndrome-associated sebaceous gland tumor in five patients (45%). All patients with sebaceous gland tumors of the type associated with Muir-Torre syndrome warrant consideration of Muir-Torre syndrome and appropriate workup to detect asymptomatic malignant visceral neoplasms.