[Muir-Torre syndrome].
Muir-Torre syndrome (MTS) is an autosomal dominant genodermatosis that is characterized by the presence of at least one sebaceous gland tumor with or without keratoacanthoma, and a minimum of one internal malignancy. The most commonly associated neoplasms were colorectal (51%) and genitourinary (25%). MTS shares a number of clinical and pathological presentations with hereditary nonpolyposis colorectal carcinoma (HNPCC), including: tendency to develop right-sided colon cancers; predisposition for the development of extracolonic tumors; and prolonged survival after surgical treatment. These family members should be monitored to detect cancers preliminaly and enrolled in life-long surveillance.
Second Department of Surgery, Hyogo College of Medicine.
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Yoshikawa
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Utsunomiya
D015179:Colorectal Neoplasms / Q000235:genetics; D003123:Colorectal Neoplasms, Hereditary Nonpolyposis / Q000235:genetics; D005260:Female; D006801:Humans; D007636:Keratoacanthoma / Q000235:genetics; D008297:Male; D012626:Sebaceous Gland Neoplasms / Q000235:genetics; D013577:Syndrome; D014565:Urogenital Neoplasms / Q000235:genetics