PDF(Pubmed)
Abstract：
(1) Background: Endocrine Mucin-Producing Sweat Gland Carcinoma (EMPSGC) is a rare, low-grade, neuroendocrine-differentiated, cutaneous adnexal tumor, officially recognized by the World Health Organization (WHO) Skin Tumors Classification in 2018 as a separate entity and homologue of endocrine ductal carcinoma in situ (eDCIS)/solid papillary carcinoma of the breast. Although it is more frequent in the female sex, between 60 and 70 years old, in the peri-orbital region, EMPSGC has also been described in the male sex, in subjects under 60 and over 80, and in extra-eyelid localizations (cheek, temple, scalp), but also in extra-facial localizations (chest and scrotum). (2) Methods: We present the clinical case of a 71-year-old woman with an undated lesion of the scalp, which presented as a nodule, skin-colored, and 2.5 cm in maximum diameter. We also conduct a comprehensive literature review from 1997 to the end of 2022, consulting PubMed, Scopus, Web of Science (WoS), and Google Scholar using the following keywords: \"Endocrine mucin-producing sweat gland carcinoma\" and/or \"EMPSGC\" and/or \"skin\" and \"cutaneous neoplasms\". In addition, we followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 253 patients were recorded; 146 were females (57.7%) and 107 were males (42.2%). The vast majority of the lesions were in the eyelids (peri-ocular region), and only a minority of cases involved the cheeks, supra-auricular, retro-auricular, and occipital region, with very rare cases in the scalp, to which the present is also added. (4) Conclusions: The morphological and immunophenotypical features are essential both for the correct diagnosis and to be able to classify this lesion among the corresponding eDCIS/solid papillary carcinoma of the breast, with neuroendocrine differentiation. Recent papers have attempted to shed light on the molecular features of EMPSGC, and much remains to be conducted in the attempt to subtype the molecular profiles of these entities. Future studies with large case series, and especially with molecular biology techniques, will be needed to further add information about EMPSGC and its relationship in the PCMC spectrum.
摘要：
(1)背景:内分泌粘蛋白分泌型汗腺癌(EMPSGC)是一种罕见的,低档,神经内分泌分化，皮肤附件肿瘤，2018年被世界卫生组织（WHO）皮肤肿瘤分类正式认可为内分泌导管原位癌（eDCIS）/乳腺实体乳头状癌的单独实体和同源物。虽然它在女性中更常见，在60到70岁之间，在轨道周围区域，EMPSGC也被描述为男性，在60岁以下和80岁以上的受试者中，以及眼睑外定位(脸颊，寺庙，头皮),而且在面部外定位（胸部和阴囊）。（2）方法：我们介绍了一名71岁女性头皮未满病变的临床病例，表现为结节，肤色,和2.5厘米的最大直径。我们还从1997年到2022年底进行了全面的文献综述，咨询PubMed，Scopus,WebofScience(WoS),和谷歌学者使用以下关键词：\"产生内分泌粘蛋白的汗腺癌\"和/或\"EMPSGC\"和/或\"皮肤\"和\"皮肤肿瘤\"。此外,我们遵循系统评价和荟萃分析（PRISMA）指南的首选报告项目。共记录了253例患者；女性146例（57.7%），男性107例（42.2%）。绝大多数病变在眼睑（眼周区域），只有少数病例涉及脸颊，耳上，复古耳廓,和枕骨区域，头皮上的病例非常罕见，现在也加入了。（4）结论：形态学和免疫表型特征对于正确诊断和能够将该病变分类为相应的eDCIS/乳腺实体乳头状癌至关重要。神经内分泌分化。最近的论文试图揭示EMPSGC的分子特征，在尝试对这些实体的分子谱进行亚型化时，还有许多工作要做。未来的研究与大案例系列，尤其是分子生物学技术，将需要进一步添加有关EMPSGC及其在PCMC频谱中的关系的信息。
