Retinal pigment epithelial (RPE) adenoma mimicking retinoblastoma in a child

The authors report a rare case of retinal pigment epithelial (RPE) adenoma in a child who presented with blurred vision in the right eye for 5 years. Examination revealed visual acuity of light perception with a giant, retrolental yellowish-white mass in the right eye. Ultrasonography revealed an intraocular mass with areas of calcification within the lesion. MRI confirmed an intraocular lesion which was hyperintense on T1-weighted imaging and hypointense on T2-weighted imaging. A provisional diagnosis of right group E retinoblastoma (RB) was made, and enucleation was performed. Based on clinical and histopathology correlation, a definitive diagnosis of right primary RPE adenoma was established. The authors herein highlight the rare occurrence of RPE adenoma in the younger age group, its resemblance to RB and the significance of considering this entity among the differential diagnoses of leukocoria.