Adults, median around 60; light iris colour is a risk factor
Approximately 5-6 per million per year
Last audited: 2026-08-13 against NCCN, ESMO, and WHO 5th ed. guidelines.
Clinical Presentation & Hallmark Symptoms
Presenting signs most frequently observed across clinical case series
Genomic Profiling & Defining Molecular Lesions
Critical diagnostic fusions, somatic mutations, and therapeutic targets
Rare malignancies frequently depend on distinct oncogenic drivers rather than conventional environmental carcinogens. Comprehensive Next-Generation Sequencing (NGS comprehensive panel) and FISH/IHC are mandatory to establish the true diagnosis and screen for basket trial agents.
Standard-of-Care Treatment Protocol
Frontline and multimodal strategies established under international consensus guidelines
Plaque brachytherapy - the commonest eye-preserving treatment
Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.
Proton beam radiotherapy for larger or posteriorly located tumours
Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.
Enucleation for large tumours or when vision is already lost
Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.
Tebentafusp for HLA-A*02:01-positive metastatic disease
Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.
Liver-directed therapy - hepatic perfusion, embolisation - for liver-limited spread
Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.
Prognostic biopsy with gene expression profiling to guide surveillance intensity
Administered in specialized high-volume oncology programs with subspecialty pathology and organ-preservation protocols.
Prognosis & Disease Trajectory
Objective clinical outlook without false reassurance
Note: Statistics reflect cohort averages. Individual outcomes depend heavily on performance status, resectability, biomarker expression, and timely access to specialized tertiary care.
Active Research, Biomarkers & Clinical Trials
Novel investigational agents, phase I/II trials, and international rare disease consortia
Tebentafusp in earlier lines, HLA-agnostic ImmTAC agents for the HLA-A*02:01-negative majority, and liver-directed combinations are the principal areas of development.
Need Help Matching to an Active Rare Cancer Trial?
ByOnco scans ClinicalTrials.gov, NCI trial networks, and institutional registries for open patient cohorts.
Verified Specialists for Uveal Melanoma
Dr. Takami Sato
Medical Oncologist
Jefferson Hospital
Philadelphia, USA
Dr. Adrian Tan
Senior Consultant Ophthalmologist
SNEC
Singapore, Singapore
Prof. Sarah Coupland
Ocular Pathologist/Oncologist
University of Liverpool
Liverpool, United Kingdom
Dr. Santosh Honavar
Ocular Oncologist
LV Prasad Eye Institute
Hyderabad, India
Designated Cancer Centers with Dedicated Programs
NCI-Designated Comprehensive Cancer Center
World-leading rare tumor board & pediatric solid tumor protocols
NCI-Designated Comprehensive Cancer Center
Largest specialized rare cancer and sarcoma multidisciplinary program
NCI-Designated Comprehensive Cancer Center
Pioneering genomic molecular tumor boards & rare histologies
NCI-Designated Comprehensive Cancer Center
High-volume surgical oncology & rare endocrine/neuroendocrine expertise
Frequently Asked Clinical Questions
Authoritative guidance on diagnosis, tumor boards, genomic markers, and care options
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Facing a Diagnosis of Uveal Melanoma?
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