Uveal melanoma in children and teenagers

ABSTRACT

To review the features and prognosis of uveal melanoma in children. Methods: Retrospective case series. Of 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 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. Conclusion: Uveal 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