Mortality of uveal melanoma in children and adolescents from 2004 to 2020.

PURPOSE: To analyze epidemiological factors, tumor characteristics, and mortality rates in a cohort of pediatric patients with uveal melanoma (UM) from the National Cancer Database (NCDB).

METHODS

In this retrospective cohort study, patients ≤20 years of age in the NCDB diagnosed with UM were included. Patients were identified using ICD-10 and ICD-O-3 codes. Main outcome measures were demographics, clinical features, and overall survival. Mortality was assessed using Kaplan-Meier survival analysis and Cox logistic regression. Significance was defined as P < 0.05.

RESULTS

Of 129 patients (68 females) included, mean age at diagnosis was 15.9 years. Most patients were White (91.5%), followed by Black (3.1%), and other races (5.4%). Tumors were primarily in the choroid (75.2%), followed by ciliary body (13.2%) or iris (7.0%). Kaplan Meier overall survival at 5-,10-, and 15-years was 93.6%, 80.3%, and 63.4%, respectively. Survival was worse with more advanced tumor T category (10-year survival 73.8% for cT1, 82.6% for cT2, 0% for cT4 [P = 0.022]) and American Joint Committee on Cancer stage (15-year survival 61.9% for stage I vs 45.5% for stage III [P < 0.001]). Cox logistic regression analysis identified older age at presentation (OR = 1.618, P = 0.036) and non-White race (OR = 21.39, P = 0.045) as significant predictors of mortality.

CONCLUSIONS

In our study cohort, older age, non-White race, and advanced tumor stage at diagnosis were associated with increased mortality in children and adolescents with UM. Adolescents have a similar prognosis to adults, whereas children have more favorable outcomes.