Research progress on primary orbital melanoma

Primary orbital melanoma (POM) is a sort of extremely rare orbital malignancy. It often associates with melanin lesion such as congenital ocular melanocytosis and blue nevus. The most common clinical manifestation of POM is progressive painless proptosis. There are some difficulties in diagnosing this disease. MRI shows characteristic expression of this disease as an important way to diagnose POM, while pathology is the gold standard. Surgery is the mainstay of treatment in patients with POM. In addition, adjuvant radiotherapy and chemotherapy should be considered. This article systematically reviews the research progress of pathogenesis, clinical manifestation, auxiliary examination, differential diagnosis and treatment of POM, in order to provide some thoughts for the future study of the rare malignancy. (Chin J Ophthalmol, 2021, 57: 861-864).