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穆尔-托里综合征:一例病例报告及文献综述

Muir-Torre syndrome: a case report and review of the literature.

作者信息

Okan Gökhan, Vural Pervin, Ince Ümit, Yazar Aziz, Uras Cihan, Saruç Murat

机构信息

Department of Dermatology, Medical Park Bahçelievler Hospital, İstanbul, Turkey.

出版信息

Turk J Gastroenterol. 2012 Aug;23(4):394-8. doi: 10.4318/tjg.2012.0411.

Abstract

Muir-Torre syndrome is a rare autosomal dominant genodermatosis characterized by the occurrence of sebaceous gland neoplasm associated with visceral malignancies. Most patients present with sebaceous adenomas, but cystic sebaceous neoplasms have been reported as specific markers of the syndrome. Gastrointestinal and genitourinary cancers are the most common internal malignancies. Colorectal cancer is the commonest visceral neoplasm in Muir-Torre syndrome patients. In this case report, we describe a rare case of Muir-Torre syndrome associated with colon cancer, and we demonstrate the important role of the dermatopathologist in alerting the clinician to the possibility of Muir-Torre syndrome when the diagnosis of sebaceous neoplasm is made.

摘要

穆尔-托雷综合征是一种罕见的常染色体显性遗传性皮肤病,其特征是出现与内脏恶性肿瘤相关的皮脂腺肿瘤。大多数患者表现为皮脂腺腺瘤,但囊性皮脂腺肿瘤已被报道为该综合征的特异性标志物。胃肠道和泌尿生殖系统癌症是最常见的内部恶性肿瘤。结直肠癌是穆尔-托雷综合征患者中最常见的内脏肿瘤。在本病例报告中,我们描述了一例与结肠癌相关的罕见穆尔-托雷综合征病例,并展示了皮肤病理学家在诊断皮脂腺肿瘤时提醒临床医生注意穆尔-托雷综合征可能性方面的重要作用。

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