Abstract
A 5-year-old white boy developed a gray-tan discoloration with nodular thickening of the iris stroma in his left eye six weeks earlier. Ophthalmologic examination demonstrated keratic precipitates, peripheral anterior synechiae, and several pale-tan choroidal infiltrates in the involved eye. Cytologic examination of the aqueous humor and results of iris biopsy were interpreted as malignant melanoma of the iris, predominantly of the epithelioid cell type. The globe was enucleated one week later. Histopathologic examination disclosed a diffuse malignant melanoma of the uvea, mixed cell type, with multiple cysts of the ciliary epithelium. Study by electron microscopy indicated bizarre neoplastic melanocytes containing numerous immature melanosomes in various stages of melanogenesis, as well as fully melanized mature melanosomes. Eight months after enucleation the patient developed metastasis to the nasopharynx and base of the skull.
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