Surgical resection of a retinal pigment epithelial hamartoma.

Several darkly pigmented tumors affecting the macular area have been described, including retinal pigment epithelial (RPE) hamartomas, combined retina-RPE hamartomas, melanocytic nevi of the RPE (congenital hypertrophy of the RPE), choroidal nevus, and melanomas invading the retina. Melanocytomas can rarely also be located in the macular area without involving the overlying retina. Retinal pigment epithelial hamartomas are focal, nodular, jetblack lesions that usually appear to involve the full thickness of the retina and to spill onto the inner retinal surface in an umbrella fashion, frequently in the macular area. Combined retina and RPE hamartomas can be papillary or nonpapillary, variably elevated pigmented lesions at the level of the RPE with a thin semitransparent membrane partly covering the tumor surface. There are various reports in the literature of vitrectomy to peel the epiretinal component of these lesions, with improvement of visual acuity. Melanocytoma is described as a darkly pigmented lesion of variable size and shape located partly or completely in the optic nerve head. Since the clinical and histopathologic nature of the lesion was clarified, similar tumors have been reported in other locations, including the macula, where they always involve the choroid only. We report an unusual pigmented macular tumor in a child that was surgically excised. It was also studied histopathologically and diagnosed as an RPE hamartoma.