Endocrine mucin-producing sweat gland carcinoma (EMPSGC) of the eyelid: Clinicopathologic features, immunohistochemical findings and review of literature

Mulay, Kaustubh; Menon, Vimla; Lahane, Sumeet; Sharma, Mukesh; Honavar, Santosh G

doi:10.4103/ijo.ijo_1745_18

Cited by 16 publications

(33 citation statements)

References 10 publications

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“…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%

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

Hadi

Barber

et al. 2021

J Cutan Pathol

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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.

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Section: Introductionmentioning

confidence: 99%

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

Hadi

Barber

et al. 2021

J Cutan Pathol

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“…These include neuroendocrine markers (synaptophysin, chromogranin, neuronspecific enolase, CD56, and CD57), estrogen receptor, progesterone receptor, epithelial markers (CK7, Cam5.2, epithelial membrane antigen, CK5/6, CK8, and CK18), and gross cystic disease fluid protein-15. 2,5,6,[11][12][13] CEA, CK20, TTF-1, and CDX2 are reported to be negative in such tumors. 2,5,6,[11][12][13] Our immunohistochemical results enforce these findings, though it did not cover the entire immunohistochemical list.…”

Section: Discussionmentioning

confidence: 98%

“…2,5,6,[11][12][13] CEA, CK20, TTF-1, and CDX2 are reported to be negative in such tumors. 2,5,6,[11][12][13] Our immunohistochemical results enforce these findings, though it did not cover the entire immunohistochemical list. In their original article, Abdulkader et al were the first to report GATA-3 positivity in 2 cases of EMPSGC/mucinous carcinoma of the eyelid.…”

Section: Discussionmentioning

confidence: 98%

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

Nasser

Siddiqui

2020

Int J Surg Pathol

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Endocrine mucin–producing sweat gland carcinoma (EMPSGC) is a rare low-grade sweat gland carcinoma. It is clinically often mistaken for basal cell carcinoma and presents a low rate of local recurrence. In this article, we present 2 new cases of EMPSGC arising in a similar location (left lower eyelid) and review the literature on this topic. One patient is a 72-year-old male with complete excision and no evidence of recurrence after 17 months of surveillance. The second patient is a 77-year-old female with incomplete initial excision and local recurrence after 38 months. p63 immunostain was negative in both cases. EMPSGC should be suspected in elderly patients with lesions of eyelids. Histologically, it should be considered and worked up if the lesion is separated from the overlying epidermis and presents a mucinous component. Complete excision is advised to avoid local recurrences.

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“…Other than MCC, cutaneous neoplasms with neuroendocrine features do not generally enter the differential diagnosis of a “small blue round cell” tumor. They include basal cell carcinoma, 58 apocrine adnexal carcinoma with neuroendocrine differentiation, 59 endocrine mucin‐producing sweat gland carcinoma, 60 undifferentiated large‐cell neuroendocrine carcinoma (LCNEC) of adnexal origin 61 and carcinoids 62 . Potential diagnostic pitfalls could occur in the setting of a poor biopsy of a BCC or through misinterpretation of a LCNEC as a variant of MCC.…”

Section: Differential Diagnosismentioning

confidence: 99%

Merkel cell carcinoma: A review

Walsh

Cerroni

2020

J Cutan Pathol

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Merkel cell carcinoma has been a focus of active scientific investigation in recent years and new information on the topic has emerged. Although uncommon, this primary cutaneous neuroendocrine carcinoma, usually involving the head/neck of elderly individuals, has a poor prognosis. Within the past two decades, an increase in the incidence of the tumor and the discovery of its link to the Merkel cell polyomavirus have focused medical attention on the lesion. The resulting studies have improved our understanding of the biology of the neoplasm and contributed to clinical care. Specifically, two pathogenic subsets of the tumor have come to light, the majority due to Merkel cell polyomavirus and the minority caused by ultraviolet radiation‐induced genetic damage. This dichotomy carries prognostic implications favoring the former subset. In addition, having capitalized on the known susceptibility of the tumor to immune influences, investigators have recently discovered its responsiveness to immune checkpoint inhibition. This revelation has constituted a therapeutic milestone at the clinical level. Herein we provide an overview of the topic, outline updates in the field and place an emphasis on dermatopathologic aspects of Merkel cell carcinoma.

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Endocrine mucin-producing sweat gland carcinoma (EMPSGC) of the eyelid: Clinicopathologic features, immunohistochemical findings and review of literature

Cited by 16 publications

References 10 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

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

Merkel cell carcinoma: A review

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