Multifocal primary mucinous carcinoma of the eyelids: Implications for management

Stiegler, Julia D.; Mogan, Sean; Pukhalskaya, Tatsiana; Verhave, Brendon; Ibrahim, Sherrif F.

doi:10.1016/j.jdcr.2021.01.001

Cited by 3 publications

(4 citation statements)

References 8 publications

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“…13,14 A new feature of interest, namely, a multicentric cutaneous origin of the tumor, has emerged in the form of isolated reports of this phenomenon. [15][16][17][18][19] In addition, the occasional concurrence of EMPSGC with an analogous tumor in the breast or with other extracutaneous hormonally sensitive neoplasms has raised the possibility of a biological susceptibility to the development of such neoplasms in some individuals.…”

Section: Discussionmentioning

confidence: 99%

“…Bertagnoli et al 16 and more recently Burris et al 17 described bilateral mucinous carcinoma of the eyelid. Stiegler et al 18 described multicentric primary mucinous carcinoma involving the left upper and lower eyelids as 3 distinct lesions. Jedrych et al 22 reported a case of EMPSGC of the left lower eyelid and an invasive mucinous ductal carcinoma of the breast associated with a focal in situ component.…”

Section: Discussionmentioning

confidence: 99%

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Endocrine Mucin-Producing Sweat Gland Carcinoma: Emerging Evidence of Multicentric Cutaneous Origin and Occasional Concurrence With Analogous Breast Tumors

Ravi

Walsh

Archibald

et al. 2022

The American Journal of Dermatopathology

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Introduction:Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is a low-grade carcinoma with predilection for the eyelid. It is analogous to solid papillary carcinoma of the breast with both expressing neuroendocrine markers and the potential to progress to invasive mucinous carcinoma (IMC). Although over 80 cases of EMPSGC have been reported, few multicentric cases have been described in the literature. In this article, we report 9 cases of EMPSGC including 3 with multicentric disease.Methods:A computerized search was performed for EMPSGC and IMC of the eyelid from January 2000 to February 2021. Records were reviewed for age, sex, tumor location, and clinical impression.Results:Eight EMPSGC (7 associated with IMC) and 1 IMC of the eyelid were identified. Lesions were slightly more common in men (55%) than women. The mean age of presentation was 76 years (range, 59–98 years). Lesions ranged from 2.5 to 12 mm. Three cases had multicentric synchronous lesions on the skin. Histologically, these were well-circumscribed dermal tumors with solid or partially cystic nodules. Tested tumors expressed at least 1 neuroendocrine marker and were positive for CK7, ER/PR, 1 or more of GCDFP-15, mammaglobin, and GATA-3. One case had an associated IMC of the breast, and another case was associated with an intraductal papilloma of the breast in a man. There was no evidence of metastasis.Conclusion:EMPSGC is a low-grade adnexal neoplasm, commonly affecting the eyelid of the elderly. Lesions often progress to IMC, metastases being exceptionally rare. EMPSGC can be bilateral and multicentric. Concurrence with breast neoplasms has been observed and deserves investigation.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Endocrine Mucin-Producing Sweat Gland Carcinoma: Emerging Evidence of Multicentric Cutaneous Origin and Occasional Concurrence With Analogous Breast Tumors

Ravi

Walsh

Archibald

et al. 2022

The American Journal of Dermatopathology

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“…6,7 Immune responses in humans are variable and influenced by cofactors and the dose and form of alpha-gal consumed; however, a majority of patients present with cutaneous symptoms characterized by urticaria, angioedema, and pruritis. 8,9 Diagnoses can be challenging given the delayed and variable presentation, particularly in patients with high-consumption of mammalian meat products. We aim to describe this newly recognized entity and shed light in its ophthalmic manifestations.…”

Section: Alpha-gal Syndrome: a New Etiology For Periorbital Edemamentioning

confidence: 99%

“…Recently, a case of 3 eyelid primary mucinous carcinomas occurring unilaterally was reported. 9 Two other cases of bilateral primary eyelid mucinous carcinoma have been reported; one with sequential development of the tumors over 1 year and the other occurred simultaneously. 5,10 None of the patients in these case reports were found to have occult primary malignancies.…”

Section: Fig 2 Histopathology and Immunohistochemistrymentioning

confidence: 99%

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

Wang¹,

Deng²,

LoBue³

et al. 2022

Ophthalmic Plastic &Amp; Reconstructive Surgery

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Endocrine mucin-producing sweat gland carcinoma (EMPSGC) is thought to be a precursor to mucinous carcinoma, both of which are rare. In this case report, the authors present a 55-year-old woman with concurrent EMPSGC and mucinous carcinoma manifesting with 2 distinct lesions on her left upper and lower eyelid and 1 lesion on her right lower eyelid. Biopsy and immunohistochemical staining supported primary cutaneous origin of the tumors. Systemic metastatic workup was initiated and thus far negative. The patient underwent staged slow Mohs micrographic surgery to remove one tumor at a time, with delayed reconstruction. This is the first reported case of EMPSGC and mucinous carcinoma presenting as distinct lesions both bilaterally and synchronously.E ndocrine mucin-producing sweat gland carcinoma (EMPSGC) is a rare entity and thought to be a precursor to primary mucinous carcinoma. We describe a patient presenting with 3 distinct, concurrent lesions involving both eyes. Two of the lesions were found to be EMPSGC, and 1 lesion was found to be mucinous carcinoma. This case supports the potential close relationship between EMPSGC and primary cutaneous mucinous carcinoma. Collection and evaluation of all protected health information was Health Insurance Portability and Accountability Act compliant and adhered to the tenets of the Declaration of Helsinki.

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Adnexal neoplasms of the eye

Drozdowski,

Grant-Kels,

Falcone

et al. 2024

Clinics in Dermatology

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Multifocal primary mucinous carcinoma of the eyelids: Implications for management

Cited by 3 publications

References 8 publications

Endocrine Mucin-Producing Sweat Gland Carcinoma: Emerging Evidence of Multicentric Cutaneous Origin and Occasional Concurrence With Analogous Breast Tumors

Endocrine Mucin-Producing Sweat Gland Carcinoma: Emerging Evidence of Multicentric Cutaneous Origin and Occasional Concurrence With Analogous Breast Tumors

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

Adnexal neoplasms of the eye

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