Uveal & Retinal Melanoma (Intraocular Malignancy)
The most common primary intraocular malignancy in adults, arising from melanocytes of the uveal tract (choroid, ciliary body, and iris).
Article Table of Contents
1. Comprehensive Overview
Uveal Melanoma is the most common primary intraocular malignancy in adults, with an incidence of ~5 per million per year, arising from neuroectodermal melanocytes of the uveal tract.
Anatomical Distribution: - Choroid (85–90%): Typically presents as a pigmented, dome-shaped or collar-stud/mushroom-shaped mass breaking through Bruch's membrane with secondary exudative retinal detachment and orange lipofuscin pigment clumping. - Ciliary Body (5–8%): Clinically silent until advanced; presents with sentinel episcleral vessels, lens subluxation, or localized sector cataract. - Iris (3–5%): Lowest malignant potential; manifests as an enlarging vascularized hyperpigmented iris nodule.
Diagnostic Modalities: High-frequency B-scan ultrasonography (pathognomonic low-to-medium internal acoustic reflectivity, acoustic shadowing, and internal vascular pulsations), Optical Coherence Tomography (OCT), and Fundus Autofluorescence (FAF).
Therapeutic Revolution: Eye-Sparing Plaque Brachytherapy: Iodine-125 or Ruthenium-106 radioactive episcleral plaque brachytherapy has largely replaced enucleation for small-to-medium melanomas, achieving >95% local tumor control while preserving the physical eye and functional vision.
2. Symptoms & Warning Signs
If you observe any of the following symptoms repeatedly, an eye exam is strongly recommended:
- Asymptomatic in early stages (often detected as an incidental elevated pigmented choroidal lesion on routine dilated exam)
- Photopsia (flashing lights) and visual floaters provoked by vitreoretinal traction over the tumor apex
- Progressive peripheral visual field defect or expanding dark shadow corresponding to secondary exudative retinal detachment
- Decreased visual acuity from macular subretinal fluid or direct foveal invasion
- Visible enlarging dark pigmented iris spot, pupillary distortion (corectopia), or spontaneous hyphema
- Absence of pain until late secondary glaucoma occurs
3. Causes & Risk Factors
- 1Somatic oncogenic driver mutations in G-protein alpha subunits (GNAQ, GNA11, PLCB4, CYSLTR2) and prognostic BAP1 / SF3B1 / EIF1AX mutations
- 2Fair skin phototype, light iris color (blue/green eyes), and blonde/red hair
- 3Pre-existing atypical choroidal nevi (transformed by risk factors: TFSOM-DIM mnemonic)
- 4Congenital Ocular / Oculodermal Melanocytosis (Nevus of Ota)
- 5Cumulative lifetime UV radiation exposure in susceptible genetic backgrounds
4. Treatments & Medical Management
5. Prevention & Daily Eye Care Advice
- Annual dilated fundus examination and baseline photographic/OCT documentation for all documented choroidal nevi
- Consistent lifelong wear of 100% UV-blocking sunglasses
- Surveillance of high-risk choroidal nevi exhibiting risk factors (Thickness >2mm, Fluid, Symptoms, Orange pigment, Margin near disc)
6. When to Seek Emergency Eye Care
Visit an immediate eye emergency center if you experience any of the following critical indicators:
- ⚠Persistent photopsia (light flashes) or a progressive peripheral visual shadow
- ⚠Growing or elevating dark spot noticed on the colored iris
- ⚠Gradual monocular visual decline without pain or external inflammation
- ⚠Follow-up evaluation for a previously diagnosed choroidal nevus
7. Frequently Asked Questions
Q:Is whole eye removal (enucleation) always necessary for ocular melanoma?
No. Due to advances in customized plaque brachytherapy (I-125) and proton beam radiotherapy, over 90% of ocular melanoma cases are cured while preserving the anatomical globe and functional sight. Enucleation is strictly reserved for massive, end-stage tumors.