Adenocarcinoma of the Ciliary Body Pigment Epithelium in a Child

Tumors arising from the pigment epithelium of the ciliary body are rare and most commonly occur in white adults. We describe a tumor that was clinically indistinguishable from a melanoma or medulloepithelioma occurring in a 7-year-old Japanese boy. Three months after initial evaluation, the eye was enucleated because of intractable intraocular pressure elevation and pain. On pathologic examination the tumor was found to fill approximately one third of the anterior chamber. Light microscopic and ultrastructural findings were consistent with an adenocarcinoma of the ciliary body pigment epithelium. To our knowledge, this is the youngest patient described with this tumor.