Medulloepithelioma is a congenital intraocular tumor that usually arises from the primitive medullary epithelium that is destined to form the nonpigmented ciliary epithelium of the ciliary body. It occurs most frequently in early childhood and is unilateral. This report documents a rare case of teratoid medulloepithelioma arising from the retina in a 9-year-old child. Gradual decrease in visual acuity (VA), strabismus and leukocoria in the left eye (LE) were observed in a 9-year-old girl. VA was 1.0 in the right eye (RE) and hand movement in the LE. Examination revealed a yellowish tumor in the retrolenticular space, apparently subretinal, vascularized, with cystic changes on its surface. Right eye was normal. Brain and orbital tomography was performed, and ocular ultrasonogram was obtained. The clinical diagnosis was retinoblastoma and the eye was enucleated. Histopathologic examination showed a benign teratoid medulloepithelioma arising from the retina. Most previously reported cases described medulloepitheliomas arising from the ciliary body. In our case, however, the tumor seemed to arise from the retina because on histopathology the ciliary body was preserved and the retina structure was not identified. Although the tumor presented was classified as benign, the fact that it is an extensive lesion with apparent recent growth justifies enucleation. The main objective of treatment is early total excision to avoid extraocular spread of the tumor.
Neuroectodermal tumors, primitive; Teratoma; Retinal neoplasms; Retinoblastoma; Child; Female
