How are intraocular tumors of the nonpigmented ciliary epithelium diagnosed?

Updated: Jun 25, 2020
  • Author: Andrew A Dahl, MD, FACS; Chief Editor: Hampton Roy, Sr, MD  more...
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Tumors of the nonpigmented ciliary epithelium

Medulloepithelioma, previously known as diktyoma, is an embryonic tumor that manifests in the first few years of life. These are solid or cystic lesions of the nonpigmented ciliary epithelium, which can extend into the anterior chamber and present with glaucoma, hyphema, and leukocoria.

In a large series from the Armed Forces Institute of Pathology, 46% of these patients had glaucoma. Glaucoma results from iris neovascularization, direct invasion of the angle structures, hyphema, or tumor-induced angle closure.

Adenomas and adenocarcinomas of the ciliary epithelium rarely are associated with glaucoma. However, in such rare instances, the presentation and management are similar to ciliary body melanoma.

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