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一名患有穆尔-托雷综合征患者的眼外皮肤皮脂腺癌:特别强调组织学和皮肤镜特征

Extraocular cutaneous sebaceous carcinoma in a patient with Muir-Torre syndrome: special emphasis on histologic and dermoscopic features.

作者信息

Savoia Francesco, Medri Matelda, Melandri Davide, Domeniconi Lucia, Crisanti Emilia, Sechi Andrea, Stanganelli Ignazio

机构信息

Skin Cancer Unit, IRCCS Istituto Romagnolo per lo Studio dei Tumori (IRST) "Dino Amadori", Meldola (FC).

Pathologic Anatomy, AUSL della Romagna, Ravenna.

出版信息

Dermatol Reports. 2023 Sep 13;16(3):9832. doi: 10.4081/dr.2023.9832. eCollection 2024 Sep 2.

Abstract

Cutaneous sebaceous carcinoma (CSC) can be classified into periocular and extraocular and can occur as part of Muir-Torre syndrome. It is usually a pink-red or yellow solitary nodule, mainly located in the head and neck region. According to the literature, dermoscopy is characterized in most cases by a yellow color, polymorphic vessels, and ulceration. We performed a review of the literature, and we found 14 papers describing the dermoscopic features of 33 sebaceous carcinomas, to which we added a case that we have recently observed. Compared to the data of the literature and in particular to the latest published reviews, we found that milky-red areas are frequently observed in CSC (47% of the cases) and can be added to the main dermoscopic features for the diagnosis. In our case, histology showed some features that were consistent with a CSC with a secretory pattern, and other features that were instead consistent with a non-secretory pattern. This was probably due to the fact that the lesion we observed was a moderately differentiated and not well-differentiated CSC. The presence of a CSC should always alert the clinician to the possible association with Muir-Torre syndrome, and immunohistochemistry for mismatch repair gene defects can help in the diagnostic pathway.

摘要

皮肤皮脂腺癌(CSC)可分为眼周型和眼外型,也可作为穆尔-托雷综合征的一部分出现。它通常是一个粉红色或黄色的孤立结节,主要位于头颈部区域。根据文献,在大多数情况下,皮肤镜检查的特征为黄色、多形性血管和溃疡。我们对文献进行了综述,发现有14篇论文描述了33例皮脂腺癌的皮肤镜特征,我们还补充了1例我们最近观察到的病例。与文献数据尤其是最新发表的综述相比,我们发现皮肤皮脂腺癌中经常观察到乳红色区域(占病例的47%),可将其添加到诊断的主要皮肤镜特征中。在我们的病例中,组织学显示一些特征与具有分泌模式的皮肤皮脂腺癌一致,而其他特征则与非分泌模式一致。这可能是因为我们观察到的病变是一个中度分化而非高分化的皮肤皮脂腺癌。皮肤皮脂腺癌的存在应始终提醒临床医生注意其与穆尔-托雷综合征的可能关联,错配修复基因缺陷的免疫组化检查有助于诊断。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/680a/11558309/d39c20e5825a/dr-16-3-9832-g001.jpg

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