Endocrine Mucin Producing Sweat Gland Carcinoma Treated With Mohs Micrographic Surgery: A Systematic Review

May 2025 | Volume 24 | Issue 5 | 8713 | Copyright © May 2025


Published online April 21, 2025

doi:10.36849/JDD.8713

Ashley Wittmer MSca, Neha Arora MBAa, Mojahed Mohammad K. Shalabi MDb, Benjamin Kelley MD MBAc, Stanislav N. Tolkachjov MDa,d-g

aTexas A&M College of Medicine, Bryan, TX
bBaylor Scott & White Medical Center, Department of Dermatology, Temple, TX
cBighorn Mohs Surgery and Dermatology Center, Scripps Clinic, La Jolla, CA
dBaylor University Medical Center, Division of Dermatology, Dallas, TX
eUniversity of Texas at Southwestern, Department of Dermatology, Dallas, TX
fEpiphany Dermatology, Dallas, TX
gVeteran Affairs Medical Center, Dallas, TX

Abstract
Background: Endocrine Mucin Producing Sweat Gland Carcinoma (EMPSGC) is a low-grade, cutaneous adnexal neoplasm. It is believed to have a potential for local recurrence as well as distant metastases in rare cases.
Objective: This study's purpose is to compare recurrence rates and outcomes between EMPSGC undergoing Mohs micrographic surgery (MMS) and traditional surgical excision.
Methods: A comprehensive review of the literature was performed using the following keywords: "Endocrine Mucin Producing Sweat Gland Carcinoma" and/or "EMPSGC" and/or "Mohs Micrographic Surgery" and/or "MMS" and/or "wide local excision" and/or "excision."
Results: There were 17 articles and 27 patients identified between the two databases. The procedures included 17 MMS and 13 surgical excisions. Patients treated with MMS typically had a lower incidence of recurrence (11.8%). In contrast, patients treated with surgical excision had a higher recurrence rate (46.2%).
Limitations: This study is limited by the small number of cases identified in our literature search and the variability in follow-up duration among the cases.
Conclusions: MMS demonstrated less recurrence of EMPSGC compared with surgical excision. Further research with large sample sizes would be beneficial in optimizing the surgical treatment approach for EMPSGC.

J Drugs Dermatol. 2025;24(5) doi:10.36849/JDD.8713

INTRODUCTION

Endocrine Mucin Producing Sweat Gland Carcinoma (EMPSGC) is a rare, low-grade neuroendocrine cutaneous adnexal neoplasm. This tumor is more common in women than men, and the median age at presentation is 70 years.1 It predominantly involves the eyelids but can occur in other areas of the body. EMPSGC is thought to be a precursor of primary mucinous carcinoma (PCMC), and these two neoplasms share several histologic and immunologic characteristics.1-3 On histopathology, EMPSGC can resemble ductal carcinoma in situ and mucinous carcinoma of the breast.1,4 The tumor appears as a well-circumscribed dermal proliferation of neoplastic cells that may be cystic, multinodular, and express neuroendocrine markers. Papillary patterns and cribriform patterns, in which the cells of the tumor grow in a pseudorosette or lacelike pattern, are often observed.1,5 Clinically, they typically present as an asymptomatic and slow-growing nodule or papule. These tumors can morphologically resemble hidrocystomas and basal cell carcinoma, as they may appear nodular, pearly, pink flesh-colored, or contain telangiectasias.1,6-8 In some cases, the lesions may mimic a chalazion in clinical appearance.9

EMPSGC is locally aggressive but typically carries a low potential for recurrence and/or metastasis.1,4 However, compared to other non-melanoma skin cancers, the rate of metastasis for EMPSGC is usually higher.9 Current management for EMPSGC usually includes a metastatic workup in addition to either surgical excision with margins greater than 5 mm or Mohs micrographic surgery (MMS). However, clinical guidelines for optimal treatment of EMPSGC remain unestablished.4 For the treatment of EMPSGC, there are limited studies that compare the recurrence rates between MMS and traditional surgical excision. The aim of this study was to perform a systematic review of the literature to identify cases where EMPSGC was treated with MMS or surgical excision and to compare their recurrence rates.