Endocrine mucin-producing sweat gland carcinoma: a systematic review and meta-analysis

Froehlich, Michael; Conti, Keith; Norris, Ivy I.; Allensworth, Jordan J.; Ufkes, Nicole; Nguyen, Shaun A.; Bruner, Evelyn; Cook, J.; Day, Terry A.

doi:10.1080/09546634.2021.1937479

Cited by 3 publications

(5 citation statements)

References 43 publications

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“…Endocrine mucin-producing sweat gland carcinoma was previously believed to be a low-grade neuroendocrine neoplasm with an indolent clinical course and a low rate of recurrence (<10%) following excision [2,3,5,12]. There have now been seven reported cases since 2020 of metastases, including this case, with the majority metastasizing to the parotid gland and none metastasizing to the lungs [3,7,8].…”

Section: Discussionmentioning

confidence: 77%

“…Although initially described in 1997, EMPSGC continues to be a rare entity, with only a low number of reported cases in the literature [5]. This histogenesis of EMPSGC is incompletely understood to date.…”

Section: Discussionmentioning

confidence: 99%

“…This histogenesis of EMPSGC is incompletely understood to date. The majority of reported cases have occurred within the last five to ten years, presumably due to their increased recognition [2,5,16]. As this is a rare tumour, there continues to be a lack of agreement as to whether this is a precursor to primary cutaneous mucinous carcinoma, on the spectrum of neuroendocrine mucinous carcinoma with minimal mucin, or a separate entity altogether.…”

Section: Discussionmentioning

confidence: 99%

“…Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare low-grade cutaneous neuroendocrine neoplasm believed to be of sweat gland origin, the cutaneous analogue to solid papillary adenocarcinoma of the breast, and a potential precursor to mucinous adenocarcinoma of the skin [1][2][3]. This entity was first described in 1997 [4], and to date there have only been just over 110 cases reported, with a predilection for females in their sixth to seventh decades of life and arising mainly in the periorbital skin [2,3,5]. The current suggested treatment is surgical excision alone [6].…”

Section: Introductionmentioning

confidence: 99%

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Metastatic Endocrine Mucin-Producing Sweat Gland Carcinoma to the Lung: A Case Report

Fournier,

Russell,

Hossain

2023

Cureus

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Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare, low-grade neuroendocrine neoplasm previously believed to be indolent in nature. There have only previously been six reported cases of metastases and none of thoracic structures. This case shows a metastatic EMPSGC in a 72-year-old male with a complex oncologic history and is the first reported case of metastases to the lung. As increased recognition of this entity continues to grow, it is important to consider it as part of the differential in mucinous and/or neuroendocrine neoplasms for appropriate management. This case adds to the oncologic literature by demonstrating a rare cutaneous neoplasm and emphasizing its metastatic potential.

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Section: Discussionmentioning

confidence: 77%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Metastatic Endocrine Mucin-Producing Sweat Gland Carcinoma to the Lung: A Case Report

Fournier,

Russell,

Hossain

2023

Cureus

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“…With regard to hormone receptors, almost all EMPSGC lesions were positive for at least one of ER, PgR, and AgR. In almost all published cases, immunostaining for p63 and/or SMA was performed to allow the study of the myoepithelium and to understand whether the lesion was in situ or invasive [ 6 , 7 ]. In a paper [ 8 ], the diagnostic usefulness of immunohistochemical staining for MYB in 11 cases was reported, while a very recent paper [ 9 ] analyzed the use of a new neuroendocrine differentiation marker, Insulinoma-associated protein 1 (INSM1), which was extremely specific and more sensitive than the routinely used neuroendocrine markers, such as Syn and CgA.…”

Section: Case Presentationmentioning

confidence: 99%

Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature

Cazzato,

Bellitti,

Trilli

et al. 2023

Dermatopathology

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(1) Background: Endocrine Mucin-Producing Sweat Gland Carcinoma (EMPSGC) is a rare, low-grade, neuroendocrine-differentiated, cutaneous adnexal tumor, officially recognized by the World Health Organization (WHO) Skin Tumors Classification in 2018 as a separate entity and homologue of endocrine ductal carcinoma in situ (eDCIS)/solid papillary carcinoma of the breast. Although it is more frequent in the female sex, between 60 and 70 years old, in the peri-orbital region, EMPSGC has also been described in the male sex, in subjects under 60 and over 80, and in extra-eyelid localizations (cheek, temple, scalp), but also in extra-facial localizations (chest and scrotum). (2) Methods: We present the clinical case of a 71-year-old woman with an undated lesion of the scalp, which presented as a nodule, skin-colored, and 2.5 cm in maximum diameter. We also conduct a comprehensive literature review from 1997 to the end of 2022, consulting PubMed, Scopus, Web of Science (WoS), and Google Scholar using the following keywords: “Endocrine mucin-producing sweat gland carcinoma” and/or “EMPSGC” and/or “skin” and “cutaneous neoplasms”. In addition, we followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. A total of 253 patients were recorded; 146 were females (57.7%) and 107 were males (42.2%). The vast majority of the lesions were in the eyelids (peri-ocular region), and only a minority of cases involved the cheeks, supra-auricular, retro-auricular, and occipital region, with very rare cases in the scalp, to which the present is also added. (4) Conclusions: The morphological and immunophenotypical features are essential both for the correct diagnosis and to be able to classify this lesion among the corresponding eDCIS/solid papillary carcinoma of the breast, with neuroendocrine differentiation. Recent papers have attempted to shed light on the molecular features of EMPSGC, and much remains to be conducted in the attempt to subtype the molecular profiles of these entities. Future studies with large case series, and especially with molecular biology techniques, will be needed to further add information about EMPSGC and its relationship in the PCMC spectrum.

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Challenges in the diagnosis and treatment of the malignant adnexal neoplasms of the head and neck

Wierzbicka

Kraiński

Bartochowska

2023

Current Opinion in Otolaryngology &Amp; Head &Amp; Neck Surgery

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Purpose of reviewThe current review is to present the current knowledge regarding epidemiology, diagnostics, and management of malignant adnexal neoplasms (MANs).Recent findingsImmunotherapy and gene-related therapies are still being developed as the methods of salvage treatment in advanced and disseminated cases: CACNA1S, ATP2A1, RYR1, and MYLK3, as well as p53 or the JAK/STAT pathways, may be therapeutic targets; the efficiency of talimogene laherparepvec and nivolumab is assessed.SummaryMANs are rare tumors, but due to the aging of population their incidence is increasing. Their clinical presentation is unspecific, which makes the diagnosis challenging. Histopathological assessment is difficult even for experienced pathologists. Mohs micrographic surgery or wide local excision are recommended to treat primary lesions. Adjuvant radiotherapy may be beneficial in case of insufficient or positive surgical margins, in nodal metastases, in selected types of MANs like sebaceous, trichilemmal, and pilomatrix carcinomas, and as the induction treatment in large tumors located in medically fragile or cosmetically important regions. The role of chemotherapy is not well defined; however, it is recommended in distant metastases. Immunotherapy can improve the prognosis in advanced stage of the disease.

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Endocrine mucin-producing sweat gland carcinoma: a systematic review and meta-analysis

Cited by 3 publications

References 43 publications

Metastatic Endocrine Mucin-Producing Sweat Gland Carcinoma to the Lung: A Case Report

Metastatic Endocrine Mucin-Producing Sweat Gland Carcinoma to the Lung: A Case Report

Endocrine Mucin-Producing Sweat Gland Carcinoma: Case Presentation with a Comprehensive Review of the Literature

Challenges in the diagnosis and treatment of the malignant adnexal neoplasms of the head and neck

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