Uveal melanoma is a rare, aggressive ocular malignancy that can be difficult to recognize and diagnose. Primary ocular treatment may prevent local recurrence, yet many patients remain at high risk for metastatic spread and with poor long-term prognosis.1-5

~2,500–3,500

patients in the United States are diagnosed with uveal melanoma each year, and it is the most common type of primary intraocular malignancy in adults.6-8

The symptoms of uveal melanoma can include pain and vision changes including9:

Blurred vision
Photopsia
Floaters
Visual field loss
Metamorphopsia
30%

Up to 30% of patients are asymptomatic.4

Diagnosis is primarily based on clinical assessment and multimodal imaging; however, it may be challenging due to asymptomatic disease and the tumor’s intraocular location. Diagnosis often requires coordinated input across specialties.9-11

graph-horizontal image

Primary treatment approaches for uveal melanoma can be challenging12

Standard treatment approaches include radiation therapy and/or enucleation, selected according to tumor size and/or location.13

Most of these interventions or treatments result in vision loss, either immediate (enucleation) or progressive (radiation), and typically require care at specialized centers, adding to patient burden of coordination and travel.12,14

doctor
~50%

Up to half of patients with uveal melanoma will eventually progress to metastatic disease (~1,500 annually in the US)7,15-18

Metastatic risk is driven by molecular alterations (GEP class, PRAME), and certain clinical factors (tumor size, ciliary body involvement, extraocular extension), which determine surveillance frequency.19

graph-vertical image

Risk of Metastasis and Guidance for Surveillance as per NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®)16,19,20

Risk Category Risk Definitions 5-Year Metastatic Risk by GEP Class Surveillance Considerations

High
  • AJCC stage: T4 disease
  • GEP: Class 2
  • Genomic profile:
    Monosomy 3, gain of
    chromosome 8q, BAP1
    mutation

Class 2: ~72%
  • Imaging every 3–6
    months for 5 years
  • Imaging every 6–12
    months for years 6–10,
    then as clinically
    indicated

Medium
  • AJCC stage: T2–T3
  • GEP: Class 1B
  • Genomic profile: SF3B1
    mutation, PRAME
    expression

Class 1B: ~21%
  • Imaging every 6–12
    months for 10 years, then as clinically indicated

Low
  • AJCC stage: T1 disease
  • GEP: Class 1A
  • Genomic profile: Disomy
    3, gain of chromosome
    6p, EIF1AX mutation

 

Class 1A: ~2%
  • Imaging every 12 months
    for 5 years or as clinically
    indicated

NCCN makes no warranties of any kind whatsoever regarding their content, use or application and disclaims any responsibility for their application or use in any way.

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[Dr. Smith] provides insights into uveal melanoma, exploring its unique biology, clinical 
challenges, and current approaches to diagnosis and management.

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AJCC, American Joint Committee on Cancer; BAP1, BRCA1-associated protein 1; EIF1AX, eukaryotic translation initiation factor 1A, X-chromosomal; GEP, gene expression profiling; NCCN, National Comprehensive Cancer Network® (NCCN®); PRAME, preferentially expressed antigen in melanoma; SF3B1, splicing factor 3b subunit 1.

1. Kaliki S, Shields CL. Uveal melanoma: relatively rare but deadly cancer. Eye (Lond). 2017;31(2):241-257. doi:10.1038/eye.2016.275

2. Ziogas DC, Foteinou D, Theocharopoulos C, et al. State-of-the-art in metastatic uveal melanoma treatment: a 2025 update. Curr Oncol Rep. 2025;27(7):803-821. doi:10.1007/s11912-025-01684-0

3. Hanratty K, Finegan G, Rochfort KD, Kennedy S. Current treatment of uveal melanoma. Cancers (Basel). 2025;17(9):1403. doi:10.3390/cancers17091403

4. Rodriguez A, Dueñas-Gonzalez A, Delgado-Pelayo S. Clinical presentation and management of uveal melanoma: review. Mol Clin Oncol. 2016;5(6):675-677. doi:10.3892/mco.2016.1037

5. Mallone F, Sacchetti M, Lambiase A, Moramarco A. Molecular insights and emerging strategies for treatment of metastatic uveal melanoma. Cancers (Basel). 2020;12(10):2761. doi:10.3390/cancers12102761

6. Wespiser M, Neidhardt E, Negrier S. Uveal melanoma: in the era of new treatments. Cancer Treat Rev. 2023;119:102599. doi:10.1016/j.ctrv.2023.102599

7. Key statistics for eye cancer. American Cancer Society. Updated May 5, 2025. Accessed December 22, 2025. https://www.cancer.org/cancer/types/eye-cancer/about/key-statistics.html

8. Ocular melanoma. AIM at Melanoma Foundation. Accessed December 22, 2025. https://www.aimatmelanoma.org/melanoma-101/types-of-melanoma/ocular-melanoma/

9. Damato EM, Damato BE. Detection and time to treatment of uveal melanoma in the United Kingdom: an evaluation of 2384 patients. Ophthalmology. 2012;119(8):1582-1589. doi:10.1016/j.ophtha.2012.01.048

10. Foti PV, Travali M, Farina R, et al. Diagnostic methods and therapeutic options of uveal melanoma with emphasis on MR imaging-part II: treatment indications and complications. Insights Imaging. 2021;12:67. doi:10.1186/s13244-021-01001-w

11. Solnik M, Paduszyńska N, Czarnecka AM, et al. Imaging of Uveal Melanoma—Current Standard and Methods in Development. Cancers (Basel). 2022;14(13):3147. doi:10.3390/cancers14133147

12. Zemba M, Dumitrescu OM, Gheorghe AG, et al. Ocular complications of radiotherapy in uveal melanoma. Cancers (Basel). 2023;15(2):333. doi:10.3390/cancers15020333

13. Intraocular (Uveal) Melanoma Treatment (PDQ®)–Health Professional Version. National Cancer Institute. Updated May 16, 2025. Accessed January 15, 2026. https://www.cancer.gov/types/eye/hp/intraocular-melanoma-treatment-pdq

14. Ng CA, Luckett T, Mulhern B, Kee D, Lai-Kwon J, Joshua AM. What matters most to people with metastatic uveal melanoma? a qualitative study to inform future measurement of health-related quality of life. Melanoma Res. 2024;34(3):248-257. doi:10.1097/CMR.0000000000000961

15. Shields CL, Samuelson AG, Oh GJ, et al. Conditional metastasis of uveal melanoma in 8091 patients over half-century (51 years) by age group: assessing the entire population and the extremes of age. Invest Ophthalmol Vis Sci. 2023;64(10):7. doi:10.1167/iovs.64.10.7

16. Carvajal RD, Sacco JJ, Jager MJ, et al. Advances in the clinical management of uveal melanoma. Nat Rev Clin Oncol. 2023;20(2):99-115. doi:10.1038/s41571-022-00714-1

17. Kujala E, Mäkitie T, Kivelä T. Very long-term prognosis of patients with malignant uveal melanoma. Invest Ophthalmol Vis Sci. 2003;44(11):4651-4659. doi:10.1167/iovs.03-0538

18. Diener-West M, Reynolds SM, Agugliaro DJ, et al. Development of metastatic disease after enrollment in the COMS trials for treatment of choroidal melanoma: collaborative Ocular Melanoma Study Group Report No. 26. Arch Ophthalmol. 2005;123(12):1639-1643. doi:10.1001/archopht.123.12.1639

19. Referenced with permission from the NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®) for Melanoma: Uveal V.1.2026. © National Comprehensive Cancer Network, Inc. 2026. All rights reserved. Accessed February 17, 2026. To view the most recent and complete version of the guideline, go online to NCCN.org.

20. Field MG, Harbour WJ. Recent developments in prognostic and predictive testing in uveal melanoma. Curr Opin Ophthalmol. 2014;25(3):234-239. doi:10.1097/ICU.0000000000000051