Congenital and other pigmented fundus lesions

Purpose: To describe congenital and pigmented fundus tumors except nevi and melanomas. Methods: We analyse diagnostic approaches of thess tumors, their characteristics, the differential diagnosis and the follow up. Results: The main tumors include congenital tumors such as congenital hypertrophy of the retinal pigment epithelium (RPE), hamartoma of the RPE, combined hamartoma of the RPE and sensory retina. The other pigmented tumors are melanocytoma, adenoma or adenocarcinoma of RPE and pigmented choroidal metastasis. The differential diagnosis must be made from RPE hyperplasia, peripheral exsudative haemorrhagic chorioretinopathy and pseudo tumoral age related macular degeneration. Conclusions: These tumors must be known to be differenciated from melanomas and choroidal nevi. Moreover, although these lesions are mainly benign in their clinical behavior, some cases can have ocular complications, slow growth or malignant transformations.