Consultant Histopathologist, Yashoda Hospitals, Secunderabad, Telangana, India.
Department of Pathology, Yashoda Hospitals, Secunderabad, Telangana, India.
Indian J Pathol Microbiol. 2021 Jan-Mar;64(1):180-182. doi: 10.4103/IJPM.IJPM_342_19.
Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare adnexal tumor with a predilection for the skin of the eyelid. It has also been reported in other areas of the face. Extra facial location has rarely been reported. They are twice as common in the females as compared to men and frequently affect the elderly between 50 and 80 years of age. It is a low-grade carcinoma with no reported cases of metastases, although a few cases with recurrences have been reported. Since it was first described by Flieder et al. in 1997, fewer than 60 cases have been reported in the literature. We describe one such case of EMPSGC in an adult male occurring at an unusual location, the peno-scrotal junction with systemic metastases to bilateral inguinal and iliac lymph nodes, multiple bones, and pancreas. Unlike previously reported cases, our patient worsened rapidly and succumbed to the disease six months after initiation of chemotherapy and radiotherapy. To the best of our knowledge, this is the first reported case of its kind in modern published literature.
内分泌黏液性汗腺癌（EMPSGC）是一种罕见的附属器肿瘤，好发于眼睑皮肤。也有报道发生在面部的其他区域。发生在面部以外的情况很少见。与男性相比，女性的发病率是男性的两倍，且常见于 50 至 80 岁的老年人。它是一种低级别癌，没有转移的报道，尽管有少数复发的病例。自 Flieder 等人于 1997 年首次描述以来，文献中报道的病例少于 60 例。我们描述了一例发生在成年男性不常见部位（阴茎阴囊交界处）的 EMPSGC 病例，该患者伴有双侧腹股沟和髂淋巴结、多处骨骼和胰腺的全身转移。与以往报道的病例不同，我们的患者病情迅速恶化，在开始化疗和放疗 6 个月后死于该病。据我们所知，这是现代已发表文献中首例此类病例的报道。
