Tumors of the Ciliary Epithelium

The diagnosis of ciliary body tumors is difficult because of their location behind the iris, the complexity in differentiating between benign and malignant tumors, the remarkable cellular polymorphism, and the possibility of dealing with either a congenital or acquired tumor. Medulloepithelioma should be considered in the differential diagnosis of a child with leukocoria, especially if there is a gray translucent cystic ciliary body mass, iris neovascularization, lens coloboma, or cataract. Medulloepithelioma should be also considered in a child with a history of pleuropulmonary blastoma and vice versa. Acquired neoplasms of the pigment or nonpigmented ciliary epithelium can be benign (adenoma) or malignant (adenocarcinoma); the clinical differentiation between the two may be impossible. Both adenoma and adenocarcinoma appear as a solid ciliary body mass simulating a ciliary body melanoma. These tumors may grow slowly and destroy the ocular structures, but they only very rarely metastasize or cause death. These tumors are usually managed by resection (iridocyclectomy), episcleral brachytherapy, or enucleation.