Serleth H J, Kisken W A
Department of Surgery, Gundersen/Lutheran Medical Center, La Crosse, Wisconsin 54601, USA.
Am Surg. 1998 Apr;64(4):365-9.
The Muir-Torre syndrome is a rare autosomal-dominant disease involving sebaceous neoplasms as markers for multiple internal malignancies. Diagnostic criteria include at least one sebaceous gland adenoma, epithelioma, or carcinoma and at least one internal malignancy. The world literature contains 162 cases with 316 internal malignancies. Colorectal and urogenital malignancies predominate, and nearly half the patients had two or more internal cancers. The discovery of a Muir-Torre syndrome-associated sebaceous lesion is rare and should prompt an evaluation for internal malignancies. We report a family over five generations displaying this syndrome. The proband is a 44-year-old man with two skin and two colon malignancies who presented to our clinic with the chief complaint of an infected sebaceous cyst. The world literature is reviewed, and an emphasis on the surgeon's role in evaluation and treatment is discussed.
缪尔-托雷综合征是一种罕见的常染色体显性疾病，以皮脂腺肿瘤作为多种内脏恶性肿瘤的标志物。诊断标准包括至少一个皮脂腺腺瘤、上皮瘤或癌以及至少一种内脏恶性肿瘤。世界文献报道了162例病例，共伴有316种内脏恶性肿瘤。结直肠癌和泌尿生殖系统恶性肿瘤占主导，近一半的患者患有两种或更多种内脏癌症。发现与缪尔-托雷综合征相关的皮脂腺病变很罕见，应促使对内脏恶性肿瘤进行评估。我们报告了一个五代家族呈现这种综合征。先证者是一名44岁男性，患有两处皮肤和两处结肠恶性肿瘤，因皮脂腺囊肿感染为主诉前来我们诊所就诊。本文回顾了世界文献，并讨论了外科医生在评估和治疗中的作用。
