(149) Double Trouble: Endocrine Mucin-producing Sweat Gland Carcinoma of Bilateral Temples

Monday, October 2, 2023

2:45 PM – 3:45 PM East Coast USA Time

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Presenting Author(s)

Zachary S. Burgess, MD, MPH

Resident Physician
University of Texas Medical Branch, Department of Otolaryngology-Head and Neck Surgery
Galveston, Texas, United States

Co-Author(s)

Md

Maria M. de la Garza Bravo, MD

Houston Methodist Hospital, USA
PC

Patricia Chévez-Barrios, MD

Houston Methodist Hospital, USA

Senior Author(s)

Nadia G. Mohyuddin, MD, FACS

Associate Professor of Clinical Otolaryngology, Residency Program Director
Houston Methodist Hospital
Houston, Texas, United States

Disclosure(s):

Zachary S. Burgess, MD, MPH: No relevant relationships to disclose.

Nadia G. Mohyuddin, MD, FACS: No relevant relationships to disclose.

Introduction: Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade cutaneous adnexal carcinoma with predilection for the periorbital skin of elderly women. EMPSGC typically presents as a solitary lesion with an indolent course that is frequently mistaken for other cutaneous or glandular lesions. To the authors’ knowledge, this case report describes the first known case of EMPSGC affecting symmetric locations on the bilateral temples.

Methods: The case of a 59 year-old woman presenting with recurrent bilateral temple lesions identified as EMPSGCs at Houston Methodist Hospital in May of 2021 is herein described. The patient’s course and the relevant histopathologic findings are discussed. Images of the lesions and pathologic slides were obtained. A brief literature review on this disease process was performed.

Results: Imaging workup for breast cancer and metastatic disease was negative and the bilateral temple lesions underwent wide local excision. The patient’s pathologic specimens were diffusely pan-cytokeratin, epithelial membrane antigen (EMA), estrogen receptor (ER), and progesterone receptor (PR) positive. The lesions were diagnosed as EMPSGC. A 2mm foci of EMPSGC in the left-sided lesion was associated with invasive mucinous carcinoma.

Conclusions: EMSPGC was identified in 1997 with histopathology identical to papillary breast carcinoma. Since then, only 190 cases have been reported as of 2021, and EMPSGC has been identified as a precursor lesion to the more aggressive neuroendocrine-type mucinous sweat gland carcinoma (MSC). In cases of sub-total excision, EMPSGC can recur with MSC and locoregional spread. Given histopathologic similarity to papillary carcinoma of the breast, diagnostic workup to rule out breast cancer as the etiology of the lesion is necessary for definitive diagnosis of EMPSGC. While lesions are frequently managed by ophthalmologists and dermatologists, isolated facial EMPSGC is becoming more frequently identified, necessitating increased awareness and management by head and neck surgeons.