Longitudinal nationwide analysis of uveal melanoma in the United States1995-2018.

Abstract

Purpose: Uveal melanoma (UM) is the most common primary intraocular malignancy in adults, yet comprehensive nationwide epidemiologic data remain limited. This study aimed to provide an updated analysis of UM incidence, survival, and disparities in the United States (US) using near-complete population coverage.

Methods: We analyzed data from the North American Association of Central Cancer Registries (NAACCR) and US Cancer Statistics (USCS) program between 1995 and 2018, covering 98-100% of the US population. Age-adjusted incidence rates (AAIRs) and relative survival were calculated using SEER*Stat and Joinpoint regression.

Results: A total of 37,917 UM cases were identified, with an overall AAIR of 5.54 per million (95% CI: 5.48-5.59), showing a stable but slightly increasing trend (AAPC = 0.06). Incidence was highest in non-Hispanic whites (6.87 ppm), men (6.25 ppm), and those aged 75-79 years. Geographic variation was notable, with peak incidence in Oregon and Iowa. Most tumors (81.3%) were localized at diagnosis, and 27.4% of patients had another primary malignancy. 5- and 10-year relative survival rates were 83.3% and 73.9%, respectively, with worse outcomes in metastatic disease. A shift toward radiotherapy, especially brachytherapy, was observed, although survival gains remained limited.

Conclusions: This nationwide study refines US UM incidence estimates and highlights persistent disparities by sex, race, age, and geography. The high prevalence of secondary malignancies underscores the need for genetic counseling and extended surveillance. Despite advances in imaging, local therapies (e.g., brachytherapy, proton therapy), and emerging systemic approaches including immunotherapy, survival improvements in advanced UM remain limited.