An Update on Endocrine Mucin-producing Sweat Gland Carcinoma

Abstract

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade adnexal neoplasm with predilection for the periorbital skin of older women. Histologically and immunophenotypically, EMPSGC is analogous to another neoplasm with neuroendocrine differentiation, solid papillary carcinoma of the breast. Both lesions are spatially associated with neuroendocrine mucinous adenocarcinomas of the skin and breast, respectively. EMPSGC is ostensibly a precursor of neuroendocrine-type mucinous sweat gland adenocarcinoma (MSC), a lesion of uncertain prognosis. Non-neuroendocrine MSC has been deemed locally aggressive with metastatic potential, and previous works speculated that EMPSGC-associated (neuroendocrine-type) MSC had similar recurrence and metastatic potential with implications for patient follow-up. Only 96 cases of EMPSGC have been reported (12 cases in the largest case series). Herein, we present 63 cases diagnosed as “EMPSGC” in comparison with aggregated results from known published EMPSGC cases. We aim to clarify the clinicopathologic features and prognostic significance of the neuroendocrine differentiation of EMPSGC and its associated adenocarcinoma and to determine the nosological relevance of EMPSGC association in the spectrum of MSC histopathogenesis. Results established an overall female predominance (66.7%) and average presenting age of 64 years. EMPSGC lesions were associated with adjacent MSC in 33.3% of cases. The recurrence rate for neuroendocrine-type MSC was ~21%, less than the reported 30% for non-neuroendocrine MSC. There were no cases of metastasis. EMPSGC and neuroendocrine-type MSC are distinct entities with more indolent behavior than previously reported, supporting a favorable prognosis for patients.

Cytological findings in a case of endocrine mucin‐producing sweat gland carcinoma with metastasis to salivary glands

“…To date, fewer than 200 cases have been reported in the literature, with the majority occurring in the facial tissue of elderly female patients, particularly in the eyelid region. EMPSGC of the eyelid is generally considered an indolent disease, with an overall local recurrence rate of 8.4%‐14.3% 1,6 . The first eyelid EMPSGC with metastasis was described in 2020 by Friehilich et al, 2 and only two other cases have been reported in the past 2 years 3,4 .…”

“…It usually affects elderly patients, is more frequently observed in women, and has a predilection for the periorbital skin. It shares the same histological and immunohistochemical characteristics as solid papillary carcinoma (SPC) of the breast 1 . Local recurrences are common in EMPSGC, but metastases have not been reported until recently; 2–4 to date, only three cases have been reported, all of which involved ipsilateral parotid glands.…”

Bilateral Concurrent Endocrine Mucin-Producing Sweat Gland Carcinoma and Mucinous Carcinoma of the Eyelids

“…In 2018, the World Health Organization (WHO) Classification of Skin Tumors recognized EMPSGC as a low-grade carcinoma and recommended that lesions containing in situ and invasive components be diagnosed as mucinous carcinoma (with neuroendocrine features). 4 Not all reported cases of EMPSGC have a myoepithelial rim, suggesting that EMPSGC may best be defined as invasive tumors themselves. Further, there have been recent reports of EMPSGC metastasizing to the parotid glands and axial skeleton, supporting the malignant nature of the tumor.…”

¿Carcinoma de mama o carcinoma de glándula sudorípara? Presentación de dos casos y análisis de la literatura

“…Los marcadores que suelen ser positivos (mas no específicos) son el CK7, CK8, CK18, AE1/AE3, CAM5.2, EMA, GCDFP-15, estrógenos y progesterona. Lo más característico es la expresión de marcadores neuroendocrinos como la sinaptofisina y la cromogranina 10 .…”