Sebaceous carcinoma is an uncommon and aggressive epithelial malignancy involving the sebaceous gland. While this malignancy is predominant in the head and neck region, it represents a rare malignant skin adnexal tumor typically observed in individuals in their 7th decade of life.

The World Health Organization classifies sebaceous carcinoma into 2 categories: periocular and extraocular. The former accounts for 75% of cases, primarily affects the eyelid, and exhibits a higher metastatic potential. Periocular sebaceous carcinoma also ranks as the 3rd most common eyelid malignancy after basal cell carcinoma (BCC) and squamous cell carcinoma (SCC). Notably, extraocular sebaceous carcinoma tumors demonstrate a significant association with visceral malignancies.

Within the spectrum of sebaceous carcinoma, the periocular subtype presents a distinct management challenge due to the increased likelihood of regional metastasis and poorer prognosis, largely stemming from the possibility of orbital invasion. These rare adnexal tumors, commonly misdiagnosed as chalazion, blepharitis, or nevus, are fast-growing and may lead to distant metastasis. Sebaceous carcinoma's association with Muir-Torre syndrome (MTS)—a condition characterized by cutaneous sebaceous tumors, visceral malignancies, and keratoacanthomas due to deoxyribonucleic acid microsatellite instabilities—adds another layer of complexity to its clinical presentation. Experts recommend screening patients with sebaceous carcinoma for MTS, emphasizing the importance of early detection and comprehensive management.

Historically, wide local excision (WLE) was the mainstay of treatment for sebaceous carcinoma. However, recent developments advocate Mohs micrographic surgery (MMS) as a more favorable option. MMS ensures clear margins before reconstruction and proves particularly attractive in the treatment of this tumor, allowing for tissue conservation in cosmetically sensitive areas and potentially lowering recurrence rates. Despite the reduced recurrence and metastatic rates, sebaceous carcinoma still has a high mortality and poor prognosis rate. A quicker and more accurate diagnosis can improve patient outcomes.