Davis D A, Cohen P R
Department of Medicine, University of Colorado Medical School, Denver, USA.
J Am Acad Dermatol. 1995 Nov;33(5 Pt 2):909-12. doi: 10.1016/0190-9622(95)90435-2.
The Muir-Torre syndrome is an autosomal dominant genodermatosis characterized by the occurrence of one or more sebaceous gland tumors (either adenoma, epithelioma, or carcinoma) and a single malignant internal neoplasm. To date, 133 cases of Muir-Torre syndrome have been described. Colorectal (49%) and genitourinary (21%) carcinomas are the most common initial neoplasms. The case of a man with a history of recurrent ocular sebaceous carcinoma who subsequently had seminoma is described, and previously published reports of men with Muir-Torre syndrome and genitourinary tumors are discussed. Including this report, an associated genitourinary tumor was the initial malignant internal neoplasm in 11 men with Muir-Torre syndrome. The detection of a genitourinary neoplasm preceded diagnosis of the patient's Muir-Torre syndrome-associated sebaceous gland tumor in five patients (45%). All patients with sebaceous gland tumors of the type associated with Muir-Torre syndrome warrant consideration of Muir-Torre syndrome and appropriate workup to detect asymptomatic malignant visceral neoplasms.
穆尔-托里综合征是一种常染色体显性遗传性皮肤病，其特征为出现一个或多个皮脂腺肿瘤（腺瘤、上皮瘤或癌）以及单一的恶性内脏肿瘤。迄今为止，已报道了133例穆尔-托里综合征病例。结直肠癌（49%）和泌尿生殖系统癌（21%）是最常见的首发肿瘤。本文描述了一名有复发性眼部皮脂腺癌病史、随后患精原细胞瘤的男性病例，并讨论了之前发表的关于患有穆尔-托里综合征和泌尿生殖系统肿瘤男性的报告。包括本报告在内，11名患有穆尔-托里综合征的男性中，相关泌尿生殖系统肿瘤是最初的恶性内脏肿瘤。5名患者（45%）在诊断出与穆尔-托里综合征相关的皮脂腺肿瘤之前就检测到了泌尿生殖系统肿瘤。所有患有与穆尔-托里综合征相关类型皮脂腺肿瘤的患者都需要考虑穆尔-托里综合征，并进行适当检查以检测无症状的恶性内脏肿瘤。
