The blood vessels of retinoblastomas.
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A malignant teratoid medulloepithelioma (ME) first became symptomatic in a 41-year-old woman. Her eye was enucleated for advanced glaucoma when the patient was aged 43 years, making her the oldest patient known to have had this tumor. The tumor showed little epitheliomatous differentiation, but a large rhabdomyoblastic component and nodules of cartilage. It had extended over the retina, creating a change in macular reflex as an early clinical sign, and eventually produced large retinal contraction folds. The lens was invaded by tumor, and an active phakoanaphylactic uveitis was in progress. Three of the four MEs recognized in adults have been malignant.
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.
An 18-year-old woman with malignant teratoid medulloepithelioma had an epibulbar, brown-black mass associated with expulsive hemorrhage and a limboscleral perforation. Histopathologically, a prominent pigmented neuroepithelial component was present and correlated to the black color of the mass. The massive hemorrhage is apparently related to rupture of necrotic and abnormal vascular channels within the angiomatous component of the neoplasm. Other mesenchymal elements included spindle-shaped cells, rhabdomyoblasts, and islands of hyaline cartilage.
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