Endocrine Mucin–Producing Sweat Gland Carcinoma: Two New Eyelid Cases and Review of the Literature

Nasser, Haitham; Siddiqui, Sami

doi:10.1177/1066896920913940

Cited by 8 publications

(15 citation statements)

References 22 publications

(46 reference statements)

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Endocrine mucin‐producing sweat gland carcinoma (EMPSGC) is thought to be a rare, low‐grade cutaneous adnexal carcinoma most commonly arising on the face of individuals in the sixth or seventh decades of life 1‐3 . The clinical presentation is most often that of a skin‐colored, slowly expanding nodule 4,5 .…”

Section: Introductionmentioning

confidence: 99%

“…EMPSGC has, as of yet, an unclear cell of origin, with apocrine, eccrine, or neuroendocrine lineages all proposed as possible candidates 7 . Given morphologic similarities, EMPSGC is often likened to lesions encountered in the breast, 3 including solid papillary carcinoma of the breast and endocrine ductal carcinoma in situ 1,10,11 . Overlapping histopathologic features have also been described with other salivary gland and sweat gland neoplasms (eg, pleomorphic adenoma/benign mixed tumors and hidradenoma) 8 .…”

Section: Introductionmentioning

confidence: 99%

See 1 more Smart Citation

A case of endocrine mucin‐producing sweat gland carcinoma with distant metastasis

Hadi

Barber

et al. 2021

J Cutan Pathol

View full text Add to dashboard Cite

Endocrine mucin‐producing sweat gland carcinoma (EMPSGC) is a rare cutaneous adnexal neoplasm typically arising on the face of older individuals, most commonly around the eyelids. Histopathologic features include a circumscribed proliferation of low‐grade epithelioid cells with areas of cystic and cribriform growth, foci of intracytoplasmic and extracellular mucin, and coexpression of endocrine, neuroendocrine, and cytokeratin markers by immunohistochemistry. Given histopathologic and immunohistochemical similarities, EMPSGC is often likened to solid papillary carcinoma of the breast and endocrine ductal carcinoma in situ, and is thought by many to represent a forme fruste of mucinous carcinoma of the skin. To date, the vast majority of reported cases of EMPSGC have been described as having indolent behavior, with no cases of distant metastasis yet reported. Here we report a unique case of EMPSGC that recurred over several years following standard surgical excision and Mohs micrographic surgery, with subsequent metastasis to the parotid gland and axial skeleton.

show abstract

Section: Introductionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

A case of endocrine mucin‐producing sweat gland carcinoma with distant metastasis

Hadi

Barber

et al. 2021

J Cutan Pathol

View full text Add to dashboard Cite

show abstract

“…Importantly, however, EMPSGC shows substantial histomorphologic overlap with metastatic adenocarcinoma, which must carefully be excluded by evaluation of both the histomorphology and immunophenotype. 5,6 EMPSGC is described as a circumscribed or multilobulated dermal tumor showing solid, cribriform, cystic, and or papillary architecture. Mucin is present both intracellularly and extracellularly, and its bland, monomorphous nuclei possess inconspicuous nucleoli, occasionally stippled chromatin, and generally sparse mitotic figures.…”

Section: Introductionmentioning

confidence: 99%

“…Thus, the clinical differential diagnosis is quite broad. Importantly, however, EMPSGC shows substantial histomorphologic overlap with metastatic adenocarcinoma, which must carefully be excluded by evaluation of both the histomorphology and immunophenotype 5,6 . EMPSGC is described as a circumscribed or multilobulated dermal tumor showing solid, cribriform, cystic, and or papillary architecture.…”

Section: Introductionmentioning

confidence: 99%

Comparative performance of insulinoma‐associated protein 1 (INSM1) and routine immunohistochemical markers of neuroendocrine differentiation in the diagnosis of endocrine mucin‐producing sweat gland carcinoma

et al. 2020

View full text Add to dashboard Cite

Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare cutaneous adnexal malignancy with predilection for the eyelids of older adults. It must be distinguished from metastatic adenocarcinomas of extracutaneous origin and from benign adnexal proliferations on partial samples when a solid growth component and mucin production are not evident. Thus, demonstration of neuroendocrine differentiation can help to ensure a correct diagnosis. Insulinoma-associated protein 1 (INSM1) is a novel neuroendocrine marker that has recently shown greater sensitivity than synaptophysin (SYN) and chromogranin (CHR) in the diagnosis of various neuroendocrine neoplasms. We compared the performance of these three markers across 10 examples of EMPSGC. All EMPSGCs expressed INSM1. Eight of ten were also immunoreactive for SYN; however, INSM1 staining was generally more intense and stained a greater proportion of the tumor cells. CHR staining was weak and focal in most cases. INSM1 staining was present in hidrocystoma-like components of cystic EMPSGC. These findings suggest that INSM1 may be more sensitive than SYN and CHR and thus valuable for establishing a diagnosis of EMPSGC.

show abstract

“…However, in situ carcinoma component morphology and discernable myoepithelial cells at the tumor periphery, when present, are helpful in confirming primary cutaneous adnexal origin 30 . Cutaneous metastasis from low‐grade neuroendocrine tumors (carcinoids) can also resemble EMPSGC and should also be excluded clinically 19 …”

Section: Discussion With Review Of the Literaturementioning

confidence: 99%

Endocrine mucin‐producing sweat gland carcinoma and associated primary cutaneous mucinous carcinoma: Review of the literature

Bundele

2021

J Cutan Pathol

View full text Add to dashboard Cite

Background Endocrine mucin‐producing sweat gland carcinoma (EMPSGC) is a rare, low‐grade, cutaneous adnexal carcinoma with neuroendocrine differentiation. It is considered to be a precursor of invasive neuroendocrine type primary cutaneous mucinous carcinoma (PCMC). Objective To review clinicopathological literature summary of EMPSGC and associated neuroendocrine PCMC from all reported cases and compare its behavior vs non‐neuroendocrine PCMC data reported in the literature. Methods A review of English literature of all EMPSGC cases with and without associated PCMC was carried out. Results EMPSGC was associated with invasive neuroendocrine type PCMC in 35.7% of cases. We found the recurrence rate of PCMC associated with EMPSGC of about 12.3%, which is much less than the 30% recurrence rate reported for the non‐neuroendocrine subtype of PCMC. The non‐neuroendocrine subtype of PCMC shows a 4% and 11% rate of distant and lymph node metastasis, respectively, vs EMPSGC‐associated neuroendocrine type of PCMC, which is very indolent and only one recent case of locoregional metastasis out of 190 EMPSGC cases has been reported so far. Limitation Limitation of our study includes data derivation from case reports and case series in the literature. Conclusion The prognostic benefits for this cohort of patients may be considered in their overall management.

show abstract

Endocrine Mucin–Producing Sweat Gland Carcinoma: Two New Eyelid Cases and Review of the Literature

Cited by 8 publications

References 22 publications

A case of endocrine mucin‐producing sweat gland carcinoma with distant metastasis

A case of endocrine mucin‐producing sweat gland carcinoma with distant metastasis

Comparative performance of insulinoma‐associated protein 1 (INSM1) and routine immunohistochemical markers of neuroendocrine differentiation in the diagnosis of endocrine mucin‐producing sweat gland carcinoma

Endocrine mucin‐producing sweat gland carcinoma and associated primary cutaneous mucinous carcinoma: Review of the literature

Contact Info

Product

Resources

About