[Adenoma of the retinal pigment epithelium]

We report 3 cases of macular or perimacular adenomas of the R.P.E. These tumors are small, deeply pigmented and arise abruptly like a pyramid from a flat base over laying retina. There is no intra-tumoral vascularisation during angiography. They typically grow very slowly or not. Metastasis from them has never been described like a certainty with histological evidence. Clinical course, histological aspects are very similar to a carcinoma of R.P.E. They have a tubular or cord-like arrangement of proliferated R.P.E. cells. The main problem is to differentiate these tumors from first malignant melanomas and other uveal pigmented tumors and second others nonneoplastic proliferations of R.P.E.: essentially unifocal congenital hypertrophy and reactive hyperplasia. Management of these tumors depends of clinical characteristics and often behavior of the tumor with periodical photographic or ultrasonic supervision to detect evidence of growth which could lead to enucleation to obtain histologic evidence for fear of uveal malignant tumor.