Department of Pathology and Laboratory Medicine, Queen Elizabeth II Health Sciences, Nova Scotia Health (Central Zone), Halifax, Nova Scotia, Canada ; and.
Departments of Pathology.
Am J Dermatopathol. 2022 May 1;44(5):321-326. doi: 10.1097/DAD.0000000000002132.
Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a low-grade carcinoma with predilection for the eyelid. It is analogous to solid papillary carcinoma of the breast with both expressing neuroendocrine markers and the potential to progress to invasive mucinous carcinoma (IMC). Although over 80 cases of EMPSGC have been reported, few multicentric cases have been described in the literature. In this article, we report 9 cases of EMPSGC including 3 with multicentric disease.
A computerized search was performed for EMPSGC and IMC of the eyelid from January 2000 to February 2021. Records were reviewed for age, sex, tumor location, and clinical impression.
Eight EMPSGC (7 associated with IMC) and 1 IMC of the eyelid were identified. Lesions were slightly more common in men (55%) than women. The mean age of presentation was 76 years (range, 59-98 years). Lesions ranged from 2.5 to 12 mm. Three cases had multicentric synchronous lesions on the skin. Histologically, these were well-circumscribed dermal tumors with solid or partially cystic nodules. Tested tumors expressed at least 1 neuroendocrine marker and were positive for CK7, ER/PR, 1 or more of GCDFP-15, mammaglobin, and GATA-3. One case had an associated IMC of the breast, and another case was associated with an intraductal papilloma of the breast in a man. There was no evidence of metastasis.
EMPSGC is a low-grade adnexal neoplasm, commonly affecting the eyelid of the elderly. Lesions often progress to IMC, metastases being exceptionally rare. EMPSGC can be bilateral and multicentric. Concurrence with breast neoplasms has been observed and deserves investigation.
内分泌黏液性汗腺癌（EMPSGC）是一种具有亲睑倾向的低度恶性癌。它类似于乳腺实性乳头状癌，两者均表达神经内分泌标志物，并有可能进展为浸润性黏液癌（IMC）。尽管已经报道了超过 80 例 EMPSGC，但文献中很少描述多中心病例。本文报道了 9 例 EMPSGC，其中 3 例为多中心疾病。
通过计算机检索 2000 年 1 月至 2021 年 2 月期间眼睑 EMPSGC 和 IMC 的病例。对患者的年龄、性别、肿瘤部位和临床印象进行了回顾。
共发现 8 例 EMPSGC（7 例与 IMC 相关）和 1 例眼睑 IMC。男性（55%）略多于女性。发病平均年龄为 76 岁（范围 59-98 岁）。病变大小从 2.5 至 12mm 不等。3 例患者的皮肤有同步多中心病变。组织学上，这些病变为界限清楚的真皮肿瘤，具有实性或部分囊性结节。所检测的肿瘤至少表达 1 种神经内分泌标志物，且 CK7、ER/PR、GCDFP-15、乳球蛋白和 GATA-3 阳性。1 例患者伴有乳腺 IMC，另 1 例患者为男性，伴有乳腺导管内乳头状瘤。无转移证据。
EMPSGC 是一种低级别附属器肿瘤，常见于老年人的眼睑。病变常进展为 IMC，转移极为罕见。EMPSGC 可双侧发生和多中心存在。与乳腺肿瘤并存的情况已被观察到，值得进一步研究。
