Abstract
The iris, ciliary body and retinal pigment epithelium (RPE) can undergo reactive hyperplasia, but true neoplasms of the retinal pigment epithelium are rare. RPE tumors can be benign or malignant. Even malignant variants rarely metastasize locally or distantly. Recognition of RPE tumors is important as they can be clinically confused with uveal melanomas and the prognosis and treatment of these two conditions are completely different. They can be distinguished from melanoma by their clinical, imaging and cytopathological features. While observation is usually sufficient in small and asymptomatic RPE tumors, more aggressive treatments (vitreoretinal surgery, plaque radiotherapy, etc.) may be required depending on the conditions they rarely cause. In this review, it is aimed to convey current literature information under 8 sub-titles by quoting references from relatively new diagnostic tests and researches on the subject.
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