Uveal Melanoma Guide: An Overview From Diagnosis to Treatment and Beyond

Uveal melanoma is a rare type of cancer that develops in the uvea, the middle layer of the eye. While it is the most common primary eye cancer in adults, it affects only about five in every one million people.

The uvea consists of three parts: the iris (the colored part), the ciliary body and the choroid. Most cases occur in the choroid, which is the vascular layer providing oxygen and nutrients to the retina. Unlike skin melanoma, uveal melanoma is not strongly linked to UV exposure, though it is more common in individuals with fair skin or light-colored eyes.

“Because metastatic uveal melanoma is so rare, it’s very important that patients seek a provider who’s familiar with the treatment landscape,” Dr. Marlana M. Orloff of Thomas Jefferson University told CURE in 2023.

What Does a Uveal Melanoma Diagnosis Look Like?

Because the tumor is often hidden behind the pupil, it is frequently discovered during a routine eye exam. To confirm a diagnosis, your ocular oncologist may use the following tools:

• Ultrasound (B-scan): A non-invasive test using high-frequency sound waves to measure the tumor’s size and thickness.
• Fluorescein Angiography: A procedure where dye is injected into the arm to highlight the blood vessels in the back of the eye.
• Optical Coherence Tomography (OCT): High-resolution imaging that captures cross-sections of the retina.
• Biopsy: In some cases, a small needle is used to take a tissue sample to analyze the genetic profile of the tumor (often called "Gene Expression Profiling"), which helps predict the risk of the cancer spreading elsewhere.

Treatment Options for Uveal Melanoma

The primary goal of treatment is to preserve the eye and vision whenever possible while ensuring the local tumor is destroyed.

Radiation Therapy

• Plaque Brachytherapy: This is the most common treatment. A small gold disc containing radioactive seeds is temporarily sewn onto the outside of the eye, directly over the tumor. It stays in place for several days before being removed.
• Proton Beam Therapy: A form of external radiation that uses precise proton particles to target the tumor with minimal damage to surrounding healthy tissue.

Surgery

• Enucleation: If the tumor is very large or involves the optic nerve, the eye may need to be removed. A prosthetic eye is later fitted to match the remaining eye.

Systemic Therapy

If the cancer spreads (metastasizes), typically to the liver, treatments like Kimmtrak (tebentafusp), a specialized immunotherapy, or localized liver treatments may be used.

Adverse Effects of Uveal Melanoma

Treatment side effects vary based on the size and location of the tumor:

• Vision Loss: Depending on the radiation’s proximity to the macula or optic nerve, vision may blur or decrease over time.
• Radiation Retinopathy: Damage to the retinal blood vessels from radiation, which can sometimes be managed with injections.
• Cataracts: Clouding of the eye’s lens is a common long-term side effect of radiation.
• Dry Eye or Discomfort: Usually temporary following plaque surgery.

A diagnosis of uveal melanoma marks the beginning of a long-term relationship with your medical team. While the focus starts with the eye, long-term care also includes regular monitoring of the liver and lungs, as this cancer has a tendency to spread through the bloodstream rather than the lymph nodes.

Early intervention and genetic testing of the tumor have significantly improved how oncologists personalize treatment plans today.

Reference

1. “Advice for Patients Diagnosed with Metastatic Uveal Melanoma,” CURE; https://www.curetoday.com/view/advice-for-patients-diagnosed-with-metastatic-uveal-melanoma

Editor's note: This article is for informational purposes only and is not a substitute for professional medical advice, as your own experience will be unique. Use this article to guide discussions with your oncologist. Content was generated with AI, reviewed by a human editor, but not independently verified by a medical professional.

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