Sweat Gland Carcinoma of the Lower Eyelid

Figure

a) Well-defined mass and loss of eyelashes. b) Histology (and immunohistochemical confirmation: positivity for GATA-binding protein 3, estrogen receptor, progesterone receptor, partial positivity for the neuroendocrine markers INSM1, CD56, and synaptophysin, proliferation rate Ki-67 5%; partially preserved myoepithelial cell layer on cytokeratin 5/6 and p63 staining) showed areas of endocrine mucin-producing sweat gland carcinoma

A patient in their 50s presented with a more than 5-year history of a noticeable lesion on the right lower eyelid that was slowly increasing in size. Surgical removal of the 8-mm, round, elevated mass (Figure a) revealed a surprising finding: The rare endocrine mucin-producing sweat gland carcinoma (EMPSGC) was first described in 1997 as a cutaneous analog of the solid papillary carcinoma of the breast (Figure b). Approximately 90% of these low-grade adnexal neoplasms typically occur in the eyelid or periocular region. Following complete excision—which in this case yielded good functional and aesthetic results—the prognosis is excellent, since reports of metastatic spread are exceedingly rare. Loss of eyelashes is a sign indicative of malignancy and constitutes an argument for excision. This case underscores the need for histopathological analysis of even small, inconspicuous findings that are not necessarily suspicious for malignancy.

Prof. Dr. MP Schittkowski, Augenklinik, Universitätsmedizin Göttingen, & Pallas Kliniken Olten, [e-mail]
PD Dr. A Zettl, Pathologie Viollier AG Allschwil, Schweiz,
Prof. D. Goldblum, Pallas Kliniken Olten, Schweiz.

Conflict of interest statement: The authors state that no conflict of interest exists.

Translated from the original German by Christine Rye.

Cite this as: Schittkowski MP, Zettl A, Goldblum D: Sweat gland carcinoma of the lower eyelid. Dtsch Arztebl Int 2026; 123: 7. DOI: 10.3238/arztebl.m2025.0164