Hartig C, Stieler W, Stadler R
Hautklinik, Minden.
Hautarzt. 1995 Feb;46(2):107-13. doi: 10.1007/s001050050218.
We report on a 63-year-old female patient with Muir-Torre syndrome (MTS). In the course of this disease two carcinomas of the colon, a kerato-acanthoma and multiple sebaceous gland tumours, including four sebaceous carcinomas, appeared. This case is thought to be a hereditary form as one of daughters was also found to have a sebaceous epithelioma. MTS is a mostly autosomal-dominant disease with the association of sebaceous gland tumours and internal carcinomas. As the malignant tumours only show slight aggressiveness the prognosis is quite favourable. Oral isotretinoin therapy was successfully used for the inhibition of sebaceous gland proliferation. A narrower definition is presented and an updated survey of the published cases is given. Furthermore, the histopathologic peculiarities of sebaceous gland tumours, especially of sebaceous gland carcinomas, are discussed and compared to sebaceous gland tumours not connected with MTS. A total number of 100 of the 135 published cases of MTS were included and analysed regarding sebaceous gland tumours and other skin tumours. The data on internal carcinomas were taken from the work of Cohen et al. (1991) and 11 current cases were added.
我们报告了一例63岁患有穆尔-托雷综合征（MTS）的女性患者。在该疾病过程中，出现了两处结肠癌、一个角化棘皮瘤和多个皮脂腺肿瘤，其中包括四个皮脂腺癌。由于发现患者的一个女儿也患有皮脂腺上皮瘤，因此该病例被认为是遗传性的。MTS是一种主要为常染色体显性遗传的疾病，伴有皮脂腺肿瘤和内脏癌。由于恶性肿瘤的侵袭性较弱，预后相当良好。口服异维A酸疗法成功用于抑制皮脂腺增生。本文给出了一个更狭义的定义，并对已发表病例进行了最新综述。此外，还讨论了皮脂腺肿瘤，尤其是皮脂腺癌的组织病理学特征，并与不伴有MTS的皮脂腺肿瘤进行了比较。在已发表的135例MTS病例中，共纳入100例，对其皮脂腺肿瘤和其他皮肤肿瘤进行了分析。内脏癌的数据取自科恩等人（1991年）的研究，并增加了11例当前病例。
