Recent updates on the management of ocular sebaceous gland carcinoma

Abstract

Ocular sebaceous gland carcinoma (SGC) is a relatively rare, slow growing, but most aggressive and life-threatening tumor. It accounts for around 1% of all cutaneous malignancies. In Caucasians, SGCs are rare accounting for 1-5.5% of eyelid malignancies with a high incidence rate (28-60%) reported in the Asian population. In most SGCs no obvious etiology has been identified but few cases are associated with Muir-Torre syndrome. The dysregulation of several cell signaling pathways has been reported in tumorigenesis of SGC. Recently genome sequencing of periocular SGC revealed several gene mutations like TP53 and RB1 genes. Ocular SGC is known as the ‘great masquerader’ as it mimics several benign and inflammatory conditions like chalazion and chronic blepharitis/ blepharoconjunctivitis which may be responsible for delayed diagnosis and high mortality. Clinico-pathologically ocular SGC can be broadly categorized into nodular and pagetoid subtypes. The latter is more aggressive and associated with a high rate of lymph node metastasis and recurrence hence requiring aggressive multimodal treatment. More aggressive features associated with poor prognosis include involvement of both eyelids, infiltrative growth pattern, multicentric in origin with a pattern of spread to surrounding structures like pagetoid spread, vascular, lymphatic and orbital invasion. Although wide surgical excision with tumor-free margin is the gold standard treatment for the localized nodular type of ocular SGC, but the management of advanced-stage disease, invasive or aggressive lesions and recurrence is challenging and often needs a multidisciplinary approach that can reduce the mortality rate in patients with SGC. In this review article, we report recent research in molecular pathogenesis, clinicopathological features, the importance of TNM staging, sentinel lymph node biopsy, map biopsy and immunohistochemical evaluation of tumor markers like p,Ki-67, bcl-1, and p. We also emphasized the treatment of ocular SGC, i.e. surgical excision & reconstruction, topical therapy, neoadjuvant chemotherapy, targeted therapy, and radiation therapy.