Uveal melanoma in children and adolescents

Abstract

Five-year survival data were available in 78 cases originally classified as uveal melanomas in patients less than 20 years old. Forty-two were malignant melanomas arising from the choroid and/or ciliary body; 13 patients from this group died of metastatic disease. Factors that significantly correlated with fatality were a red, painful eye, extraocular extension at enucleation, basal tumor diameter greater than 10 mm, increased mitotic activity, and tumor necrosis. There were 36 iridic tumors; nine were reclassified as nevi. Of the 27 patients with iridic melanomas, only four died of metastases. The predominant factors relating to fatal outcome were glaucoma, extension of tumor into the ciliary body, diffuse growth, deep angle invasion, scleral invasion by tumor cells, and increased mitotic activity. Except for their relative rarity, uveal melanomas in children and adolescents did not differ significantly from their counter-parts in adults.