Endocrine mucin-producing sweat gland carcinoma of the eyelid: A clinical and histological conundrum

A 60-year-old male presented with a 6-month-old history of a left upper lid mass. The mass was excised, and histopathological evaluation showed a well-circumscribed, multinodular, intradermal tumor consisting of round-to-oval cells with round nuclei and mucin filled cysts. On immunohistochemical analysis, the tumor cells stained positively for cytokeratin (CK)-7, CK-8, estrogen receptor (ER), progesterone receptor (PR), mucicarmine, synaptophysin, gross cystic disease fluid protein-15 (GCDFP-15), and neuron-specific enolase (NSE). A diagnosis of endocrine mucin-producing sweat gland carcinoma (EMPSGC) of the eyelid was made and at 6-month follow-up, no recurrence was noted. In this communication, we discuss the pathology and treatment options of EMPSGC of the eyelid. Although an uncommon entity, EMPSGC may be considered as a differential when encountered with a suspicious, potentially malignant eyelid mass.