Fetal adenoma of the pigmented ciliary epithelium associated with persistent hyperplastic primary vitreous.
A 1.5-year-old girl presented with a peripheral iris mass. When the girl was 3 years old, the lesion was excised after it had manifested significant growth. A stalk of fibrovascular tissue was noted to extend from the lesion to the optic disc. Histopathologically, the tumor was a well-circumscribed, pigmented ciliary body adenoma. Electron microscopy revealed characteristic neuroepithelial melanosomes, distinct from those of choroidal melanocytes, and occasional annulate lamellae. A fibrovascular membrane extended over the tumor surface and was adherent to lens capsule. The association of this adenoma with a persistent stalk of primary vitreous indicates a congenital origin of this tumor. Both adenoma and adenocarcinoma of the pigmented and nonpigmented ciliary epithelium tend to be disorders of adults. The authors report the youngest presentation of a pigment epithelium adenoma, the only well-documented case associated with persistent hyperplastic primary vitreous, and the only documentation of annulate lamellae in a ciliary body tumor.
D000236:Adenoma / Q000150:complications* / Q000648:ultrastructure; D002924:Ciliary Body; D005260:Female; D005315:Fetal Diseases / Q000150:complications*; D006801:Humans; D006965:Hyperplasia / Q000150:complications; D007223:Infant; D008854:Microscopy, Electron; D011247:Pregnancy; D014604:Uveal Neoplasms / Q000150:complications* / Q000648:ultrastructure; D014822:Vitreous Body / Q000473:pathology*