| Summary: | Yangfan Xu,1,2,* Fang Li,1,2,* Renbing Jia,1,2 Xianqun Fan1,2 1Department of Ophthalmology, Shanghai Ninth People’s Hospital, Shanghai Jiao Tong University School of Medicine, Shanghai, People’s Republic of China; 2Shanghai Key Laboratory of Orbital Diseases and Ocular Oncology, Shanghai, People’s Republic of China *These authors contributed equally to this work Abstract: Ocular sebaceous carcinoma (SC) is an exceedingly rare but aggressive malignancy that can impair patients’ visual acuity or even cause tumor-specific mortality. This tumor usually mimics chronic benign eyelid lesions, thus leading to delayed diagnosis, thereby causing high recurrence and metastasis. Ophthalmologists should be more aware of SC in order to offer correct diagnosis and treatment at the onset of symptoms. Prompt surgical excision with frozen section margin control is the mainstay of SC management after patient evaluation and accurate biopsy. Mohs micrographic surgery has been associated with better tumor control outcomes than wide local excision. Radiation therapy should be considered as adjuvant treatment for locally advanced (stage T3a or higher) or high-risk (pagetoid spread) SC, nodal metastasis, or palliative care. Cryotherapy and topical chemotherapy are used for pagetoid spread. Targeted therapy has an emerging role in more complicated cases. For lymph node and distant metastasis, combination treatments should be provided, including lymph node and neck dissection, radiation therapy, systemic chemotherapy, and even orbital exenteration. The rarity of ocular SC precludes a comprehensive perspective on standard treatment. This paper offers a brief review of recent advances in the clinical diagnosis and management of ocular SC based on current scientific literature. Keywords: sebaceous carcinoma, periocular cancers, therapy, Mohs micrographic surgery
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