Genitourinary tumors in men with the Muir-Torre syndrome.
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.
Davis
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DA
Department of Medicine, University of Colorado Medical School, Denver, USA.
Cohen
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PR
D018266:Adenocarcinoma, Sebaceous / Q000175:diagnosis* / Q000556:secondary; D000328:Adult; D017809:Fatal Outcome; D006801:Humans; D008207:Lymphatic Metastasis; D008297:Male; D009364:Neoplasm Recurrence, Local / Q000175:diagnosis*; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis*; D009386:Neoplastic Syndromes, Hereditary / Q000175:diagnosis*; D012626:Sebaceous Gland Neoplasms / Q000175:diagnosis*; D018239:Seminoma / Q000175:diagnosis*; D013577:Syndrome; D014565:Urogenital Neoplasms / Q000175:diagnosis*