Uveal melanoma in children and teenagers.
OBJECTIVE
To review the features and prognosis of uveal melanoma in children.


METHODS
Retrospective case series.


RESULTS
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 ⩽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.


CONCLUSIONS
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.
Ocular Oncology Service, Wills Eye Hospital, Thomas Jefferson University, Philadelphia, PA, United States.
Carol L
CL
Shields
Swathi
S
Kaliki
Sruthi
S
Arepalli
Hatice Tuba
HT
Atalay
Fairooz P
FP
Manjandavida
Guilia
G
Pieretti
Jerry A
JA
Shields
Children; Choroid; Ciliary body; Eye; Iris; Melanoma; Pediatric; Uvea