Overview
Cancers of the eye and orbit are associated with diverse pathological conditions, disease prognosis, and significant mortality. The survival rates vary among patients from different countries and have been reported to be significantly lower in those from developing countries compared to those noted in the United States owing mainly to delay in diagnosis associated with advanced‐stage disease and unavailability of treatments. In this article, we provide an overview of the most common and rare ophthalmic malignancies that include ocular surface, external and adnexal diseases, and orbital and intraocular malignancies. We summarize the incidence, histopathology, risk factors, molecular alterations, clinical presentation, diagnostic evaluation, and therapeutic approaches for these diseases. We also describe the first‐line and salvage treatments and their complications. These treatments depend on disease stage at presentation and include pathology‐guided excision, Mohs microsurgery, chemo‐reduction, chemoprophylaxis, adjuvant chemotherapy after enucleation, and/or neoadjuvant chemotherapy. Complications include symblepharon, scarring, and limbal stem cell deficiency while incomplete excision of eyelid tumors may result in recurrence of larger and more aggressive tumors. Currently, identification of genetic abnormalities identified in genomic profiling of patient tumors improves treatment selection. Early diagnosis and involvement of an ocular oncologist at the time of diagnosis will optimize the therapeutic management of patients with these diseases.