Abstract:

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 both expressing neuroendocrine markers and progressing to invasive mucinous carcinoma (IMC). While-over 80 cases of EMPSGC have been reported, few multicentric cases have been described in the literature. Herein, we report nine cases of EMPSGC including three with multicentric disease. Methods: A computerized search was performed for EMPSGC and IMC of the eyelid from January 2000 February 2021. Records were reviewed for age, gender, tumor location, clinical impression. Results: Eight EMPSGC and one IMC of eyelid were identified. Lesions were slightly more common in men (55%) than women (45%). Mean age of presentation was 76 years (range, 59-98). Lesions ranged from 2.5mm - 12mm. Three cases had multicentric synchronous lesions on the skin. Histologically, these were well circumscribed dermal-tumors with solid or partially cystic nodules. All tumors tested, expressed at least one neuroendocrine marker and were positive for CK7, ER/PR, and one or more of GCDFP-15, mammaglobin and GATA-3. 7/9 were associated with progression to IMC. One had an associated IMC of the breast. Another case, in a male, was associated with intraductal papilloma of the breast. There was no evidence of metastasis. Conclusion: EMPSGC is a low-grade adnexal neoplasm, commonly affecting the eyelid of elderly. Lesions, often progress to IMC, but do not metastasize. Occasionally, EMPSGC can be bilateral and multicentric. Concurrence with breast neoplasms has been rarely observed and deserves further investigation.

Financial Disclosure:
No current or relevant financial relationships exist.

Menu

Priyanka Ravi, MBBS, MD