Uveal melanoma in children and teenagers

PurposeTo review the features and prognosis of uveal melanoma in children.MethodsRetrospective case series.ResultsOf 122 children with uveal melanoma, there were 53 (43%) male and 69 (57%) female patients. In this group, the mean age at presentation was 15 years (median 16 years, range 3–20 years). Age at presentation was 0 to 5 years in 4 (3%), 5.1 to 10 years in 14 (11%), 10.1 to 15 years in 43 (35%), and 15.1 to ⩽20 in 61 (50%). Associated ocular melanocytosis was present in 4 (3%). The melanoma was primarily located in the iris (n = 30, 25%), ciliary body (n = 10, 8%), or choroid (n = 82, 67%). The mean tumor basal dimension was 9.8 mm and mean thickness was 5.0 mm. The tumor color was pigmented (brown) (n = 102, 84%), nonpigmented (yellow) (n = 19, 16%), or mixed (n = 25, 21%). Subretinal fluid (n = 66, 54%) and hemorrhage (n = 9, 7%) were noted. Primary treatment involved laser photocoagulation (n = 3, 2%), transpupillary thermotherapy (n = 17, 14%), local tumor resection (n = 26, 21%), plaque radiotherapy (n = 42, 34%), or enucleation (n = 54, 44%). Kaplan Meier 5, 10, and 20-year estimates for uveal melanoma-related metastasis were 9%, 9%, and 20%, respectively, for children compared to 15%, 25%, and 36% for all ages.ConclusionUveal melanoma in children tends to occur most often in the teenage years as a pigmented tumor involving the choroid or iris and with mean thickness of 5 mm. Prompt treatment is advised.