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The report describes a patient who developed five distinct EMPSGC and MCS lesions over eight years, involving both periorbital and lower-face sites. Targeted next-generation sequencing of two tumors identified identical ATRX missense variants, supporting molecular characterization and enabling future genetic classification of these multicentric, asynchronous neoplasms.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/multicentric-asynchronous-endocrine-mucin-producing-sweat-gland-carcinoma-and-mucinous-carcinoma-of-the-skin-case-study/358002/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/multicentric-asynchronous-endocrine-mucin-producing-sweat-gland-carcinoma-and-mucinous-carcinoma-of-the-skin-case-study/358002.png","ImageObject",300,407,{"name":92,"@type":93},"hdkawk","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-25","2026-09-23",true,{"@type":102,"interactionType":103,"userInteractionCount":34},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"What is endocrine mucin-producing sweat gland carcinoma (EMPSGC) and how is it related to MCS?","Question",{"text":112,"@type":113},"EMPSGC is an adnexal neoplasm typically occurring on periorbital skin. It shows overlapping histopathologic features with primary mucinous carcinoma of the skin (MCS).","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What did the next-generation sequencing show in this case?",{"text":117,"@type":113},"Targeted next-generation sequencing of two discrete tumors identified identical missense variants within the ATRX gene (NM_000489.6: c.2924A>G, p.D975G).",{"name":119,"@type":110,"acceptedAnswer":120},"How were the patient’s lesions managed and what was the follow-up outcome?",{"text":121,"@type":113},"Multiple lesions were treated with Mohs micrographic surgery (MMS). After the last MMS, the patient remained under active surveillance with no metastasis or local recurrence reported within 12 months.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},358002,1790179331,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":34,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":29,"language":139,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":140,"faqs":141,"seo_title":142,"seo_description":67,"update_tm":143,"read_time":144},3985747859855,"https://ap-avatar.wpscdn.com/davatar_276721f389ce27ea32af1340a28f341c","Journal of Cutaneous Pathology  \nCASE STUDY  OPEN ACCESS   \nMulticentric Asynchronous Endocrine Mucin-Producing Sweat Gland Carcinoma and Mucinous Carcinoma of the Skin  \nIkuko Hirai1,2  | Joseph F. Sobanko1 | Tara C. Mitchell3 | Jacquelyn Roth4 | Rosalie Elenitsas1 | Emily Y. Chu1   \n1Department of Dermatology, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA | 2Department of Dermatology, Keio University of School of Medicine, Tokyo, Japan | 3Division of Hematology/Oncology, Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA | 4Department of Pathology and Laboratory Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pennsylvania, USA  \nCorrespondence: Emily Y. Chu ([emily.chu@pennmedicine.upenn.edu](emily.chu@pennmedicine.upenn.edu))  \nReceived: 28 October 2025 | Revised: 6 February 2026 | Accepted: 6 April 2026  \nKeywords: endocrine mucin-producing sweat gland carcinoma | mucinous carcinoma of the skin | multicentric | sweat gland tumor  \nABSTRACT  \nEndocrine mucin-producing sweat gland carcinoma (EMPSGC) is an adnexal neoplasm which typically occurs on periorbital skin and demonstrates overlapping histopathologic features with primary mucinous carcinoma of the skin (MCS) . Herein, wereport a patient who developed five distinct lesions of EMPSGC and MCS over an eight-year period, some of which occurred on periorbital sites and others which occurred on the lower face. We further sought to better characterize these tumors on a molecular basis; targeted next generation sequencing analysis of two discrete tumors revealed identical missense variants within the ATRXgene. Our report expands on the rare prior reports of multicentric, asynchronous EMPSGC and MCS, and also provides a basis for additional genetic classification of these neoplasms.  \n1 | Introduction  \nEndocrine mucin-producing sweat gland carcinoma (EMPSGC) was first described by Flieder et [al. in](al. in) 1997 as an adnexal neoplasm with a predilection for the periorbital skin and morphologically analogous to solid papillary carcinoma of the breast (SPC) [1]. Both are low-grade solid/cystic adenocarcinomas with mucin production, express neuroendocrine markers in various degrees and demonstrate histopathologic overlap with invasive mucinous carcinoma.  \nSeveral recent reports have highlighted an uncommon presentation of these tumors: multicentricity [2–7] . The mechanism underlying multicentric and asynchronous EMPSGCs is unknown. A next-generation sequencing (NGS) study (MSK-IMPACT, 368 genes) identified somatic mutations in DNA repair and tumor suppressor pathways such as BRD4, PPP4R2, and TP53 in  \nEMPSGC, which may be implicated in the multistep molecular pathogenesis of EMPSGC [8] . However, to our knowledge, no studies have focused on the clonality of multiple EMPSGC/mucinous carcinoma of the skin (MCS) lesions in an individual via the homogeneity and heterogeneity of genetic profiles.  \nHerein, we report a patient with an 8-year history of five lesions of EMPSGC and MCS widely distributed beyond the periorbital region on the face, along with the NGS results from several lesions.  \n2 | Case Report  \nA 55-year-old woman was diagnosed with MCS on her left chin 2 years prior to initial presentation in our clinic. The lesion was treated by Mohs micrographic surgery (MMS), resulting in clear  \nThis is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.  \n© 2026 The Author(s). Journal of Cutaneous Pathology published by John Wiley & Sons Ltd.  \nJournal of Cutaneous Pathology, 2026; 53:901–906 901  \n[https://doi.org/10.1111/cup.70122](https://doi.org/10.1111/cup.70122)  \nmargins (Figures 1A and 2A,B) . She was previously healthy aside from obesity with obstructive sleep apnea and had no personal or family history of developmental delay.  ","cbCaicu1roOTKaOv","https://ap.wps.com/l/cbCaicu1roOTKaOv","pdf",2142107,"English","# Abstract\n# Introduction\n# Case Report\n# Discussion","[{\"question\":\"What is endocrine mucin-producing sweat gland carcinoma (EMPSGC) and how is it related to MCS?\",\"answer\":\"EMPSGC is an adnexal neoplasm typically occurring on periorbital skin. It shows overlapping histopathologic features with primary mucinous carcinoma of the skin (MCS).\"},{\"question\":\"What did the next-generation sequencing show in this case?\",\"answer\":\"Targeted next-generation sequencing of two discrete tumors identified identical missense variants within the ATRX gene (NM_000489.6: c.2924A\\u003eG, p.D975G).\"},{\"question\":\"How were the patient’s lesions managed and what was the follow-up outcome?\",\"answer\":\"Multiple lesions were treated with Mohs micrographic surgery (MMS). After the last MMS, the patient remained under active surveillance with no metastasis or local recurrence reported within 12 months.\"}]","Multicentric Asynchronous Endocrine Mucin-Producing Sweat Gland Carcinoma and Mucinous Carcinoma of the Skin - Case Study | PDF",1790132494,15]