有内分泌特征并分泌黏蛋白的汗腺腺癌4例临床及组织病理学分析

Endocrine mucin-producingg sweat gland carcinoma:clinicopathological study of four cases

目的:探讨有内分泌特征并分泌黏蛋白的汗腺腺癌的临床及组织病理学特征。方法:收集2020年1月—2021年10月空军军医大学西京皮肤医院确诊的4例有内分泌特征并分泌黏蛋白的汗腺腺癌患者临床及病理资料进行回顾性分析。结果:4例患者中男性3例,女性1例,发病年龄37~65岁(平均年龄49.5岁)。2例发生于眼睑,1例发生于眼角,1例发生于头部,临床表现为丘疹或结节。组织病理分为两种模式,一种为真皮内界限清楚的多结节性肿瘤团块,伴有局灶性黏液沉积,瘤细胞排列成实性、乳头状、筛状或囊状结构。另一种类似黏液癌病理模式,表现为真皮大片黏液区域内大小不一的肿瘤细胞团块。肿瘤细胞呈多角形或椭圆形,胞质呈嗜酸性,染色质细腻、呈点彩状,核仁不明显,核分裂象少见,肿瘤细胞内及间质中可见黏液样物质沉积。肿瘤组织未见明显坏死。免疫组化显示:肿瘤细胞弥漫表达CK7、雌激素受体(ER)、孕激素受体(PR)及抗上皮细胞粘附分子单克隆抗体(BerEP4),不表达CK20;其中3例表达神经内分泌标记神经元特异性烯醇化酶(NSE),2例表达CD56及突触素(Syn),1例表达嗜铬蛋白A(CgA)。结论:有内分泌特征并分泌黏蛋白的汗腺腺癌是一种少见的表达神经内分泌标记的低度恶性的汗腺肿瘤,临床好发于眼周,但临床皮损不具有特异性。组织病理归纳为两种病理模式,免疫组化神经内分泌标记阳性可帮助诊断。

Objective:To study the clinicopathological features of endocrine mucin-producing sweat gland carcinoma(EMPS GC).Methods:Four cases of EMPSGC,who visited Department of Dermatology of Xijing Hospital from January 2020 to October 2021 were selected to study the clinicopathological features,retrospectively.Results:Of the 4 patients,3 were male,and 1 was female.The patient's onset age ranged from 37 years to 65 years(average age:49.5 years).Two patients had lesions on their eyelid,one patient had lesion on canthus,and one patient had lesion on head.The lesions were presented as papule or nodule clinically.Histologically,there were two different patterns.One manifested as well-circumscribed,multinodular tumors with focal mucin deposition in the dermis.The neoplastic cells were arranged in solid,partially cystic nodules,papillary architecture or cribriform patten.The other histopathological pattern was similar to that of mucinous carcinoma,with nests of tumor cells embedded in pools of mucin in the dermis.The tumor cells were uniform,small to medium-sized,oval to polygonal epithelial cells with pale eosinophilic cytoplasm.The nuclei of the tumor cells were bland with stippled chromatin and inconspicuous nucleoli.Mitoses were rare.Immunohistochemical study showed that the tumor cells were positive for CK7、ERPR and BerEP4,but negative for CK20.Three cases expressed neuroendocrine markers NSE,2 cases expressed Syn and CD56,and 1 case expressed CgA.Conclusions:EMPSCC is a rare,low-grade adnexal neoplasm characterized by immuno-expression of neuroendocrine markers.Clinically,the tumor commonly occurs in the periorbital region with non-specific manifestation.Histologically,EMPSCC can be classified into two histopathological patterns,and the distinctive expression of neuroendocrine markers is the key to diagnosis.