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[多发性皮脂腺肿瘤伴结肠癌——穆尔-托雷综合征]

[Multiple sebaceous tumors with colon cancer--Muir-Torre syndrome].

作者信息

Vajda Adrienne, Scharrer Krisztina, Baló-Banga J Mátyás

机构信息

Bórgyógyászati Osztály és Rendelóintézet, Magyar Honvédség Központi Honvédkórház, Budapest.

出版信息

Orv Hetil. 2003 May 18;144(20):985-9.

Abstract

The Muri-Torre syndrome is an autosomal-dominant genodermatosis. The criteria of the diagnosis is the occurrence of at least one sebaceous skin tumour and at least one malignant internal tumour. Skin tumours must be considered as very important signs because they may precede visceral malignancies. The authors present the case of a 62 years old man. Three tumours were excised from three different locations of his trunk in the Plastic Surgery Ward of our hospital. The tumours proved to be sebaceous adenoma, sebaceous epithelioma and sebaceous carcinoma by histology. Because of this result, we had conducted a detailed tumour screening which found a non-symptomatic malformation of the proximal colon tract. Histology of the resected colon part revealed adenocarcinoma. With the presentation of this case the authors call attention on the fact, that in case of the diagnosis of a sebaceous skin tumour, careful tumour screening could be lifesaving.

摘要

穆里-托雷综合征是一种常染色体显性遗传性皮肤病。诊断标准是至少出现一个皮脂腺皮肤肿瘤和至少一个恶性内脏肿瘤。皮肤肿瘤必须被视为非常重要的体征,因为它们可能先于内脏恶性肿瘤出现。作者介绍了一名62岁男性的病例。在我院整形外科病房,从他躯干的三个不同部位切除了三个肿瘤。组织学检查证实这些肿瘤分别为皮脂腺腺瘤、皮脂腺上皮瘤和皮脂腺癌。基于这一结果,我们进行了详细的肿瘤筛查,发现近端结肠有一个无症状的畸形。切除的结肠部分的组织学检查显示为腺癌。通过展示这个病例,作者提醒注意这样一个事实,即在诊断出皮脂腺皮肤肿瘤时,仔细的肿瘤筛查可能会挽救生命。

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