Ocular Cancer

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Ocular cancer or cancer of the eye is a malignancy that starts and grows in the eye. A malignancy is a group of cells that are cancerous and can spread to other parts of the body. Ocular cancer is a rare disease, and only a small number of doctors in the United States have the appropriate training to treat it.

The most common primary intraocular tumor in adults is uveal melanoma. Texas Retina Associates has been caring for uveal (ocular) melanoma patients for over 40 years, treating approximately 80 cases each year. We currently have two physicians who sub-specialize in treating this rare eye cancer:

Timothy S. Fuller, MD — Trained under international ocular cancer research and treatment leaders Jerry A. Shields, MD, and Carol Shields, MD, at Wills Eye Hospital in Philadelphia, Pennsylvania

Rajiv Anand, MD — Trained in ocular oncology at Wills Eye Hospital and authored Eye Melanoma: A Guidebook for Patients

In addition, because ocular cancer can be an overwhelming diagnosis, Sophia Sotero, our experienced and dedicated Oncology Services Coordinator, serves as a personal resource to our patients and families, helping them navigate their medical journey. She has worked in the field of eye cancer for over 10 years and helps patients navigate through their evaluation, diagnosis, treatment and follow-up plans.

What is Ocular Melanoma/Uveal Melanoma?

There are a variety of tumors that can exist in the eye. The eye cancer we treat most at Texas Retina Associates is uveal melanoma (also called ocular melanoma). It involves the iris, ciliary body, and/or choroid, which are collectively referred to as the uvea. These tumors develop in the pigmented cells within the uvea. These are the cells that give the eye its color. Uveal melanoma is a very rare disease, with only 2,000 – 3,000 cases per year in the United States.

Although rare, uveal melanoma (ocular melanoma) can be aggressive. It is a malignant tumor that can grow and spread to other parts of the body.

To address patients’ concerns about their ocular cancer metastasizing (spreading to other parts of the body), Texas Retina Associates provides the most advanced genetic analysis of ocular melanomas by performing fine needle aspiration biopsy at the time of treatment. This allows for more customized metastatic screening and a greater peace of mind.

Texas Retina Associates is also continuing to follow over 100 uveal melanoma patients who were enrolled at our site in the Collaborative Ocular Oncology Group 2 study led by William Harbour, MD, at Bascom Palmer Eye Institute. This study is ushering in the next level of genetic analysis and prognostication for ocular cancer.

Symptoms

Most uveal melanomas are identified in a dilated eye exam, and many patients do not have any symptoms. Some patients report experiencing:

Blurred or decreased vision
Floaters
Light flashes

Risk Factors

In most uveal/ocular melanoma, there is no family history. And unlike skin melanomas, it is not related to sun exposure. Uveal/ocular melanoma does occur more commonly in people with the following characteristics:

Caucasian (although it can occur in any race)
Light-colored eyes — blue or green
Age 55+

If uveal melanoma is caught early, the prognosis is better and the potential loss of vision is reduced. That is why it is important for everyone to get a dilated eye exam once a year. Dilation is critical because “no dilation” eye exams can miss detection of conditions like uveal/ocular melanoma.

Diagnosis

Uveal/ocular melanoma is diagnosed through a dilated eye exam. Your Texas Retina physician may perform additional tests to gather information about your specific condition. These can include:

Ultrasound

A small probe is placed on the surface of the eye and bounces sound waves through the eye. The pattern made by the reflection of the sound waves can help differentiate a benign tumor from an ocular melanoma.

Fluorescein Angiography

In this test, a small amount of vegetable-based dye is injected through a patient’s peripheral vein — usually in the arm or hand. The injected dye lights up the retina’s intricate vascular network, and a certified ophthalmic angiographer takes a series of detailed retinal photographs.

Treatment

The two primary treatment options for uveal melanoma are radiation therapy and enucleation (surgical removal of the eye).

1 | Radiation Therapy

Radiation therapy is the most common treatment of uveal melanoma today. This is provided through a long-time partnership between Texas Retina Associates and Texas Health Presbyterian Hospital Dallas. Every radiation therapy provided by this physician team is custom-designed to minimize the patient’s vision loss. The goal is to halt growth of the cancerous tumor cells with the least negative effects on the normal tissue surrounding them.

The standard-of-care radiation therapy used to treat uveal/ocular melanoma is called brachytherapy or plaque therapy. A small disc-shaped shield known as a plaque encases radioactive seeds on one side and is covered with a thin sheet of gold on the other. It is surgically attached to the outside surface of the eye, over the tumor. The gold protects the surrounding areas from radiation damage. The plaque is left in place for several days, allowing the radiation to treat the tumor. Once complete, it is then removed, and no radioactive material remains on or within the patient.

Proton beam therapy is another type of radiation sometimes used to treat uveal/ocular melanoma.

2 | Enucleation

Enucleation is surgical removal of the eye and is another option to treat uveal/ocular melanoma. In this procedure, the eye is replaced with a ball implant which is sewn into position and attached to the patient’s eye muscles to allow movement. After healing for about six weeks, a specialist called an ocularist can fit the patient with an ocular prosthesis. This is a plastic contact lens painted to look like the patient’s other eye, and it rests on the ball implant. While the prosthetic eye improves cosmetic appearance, vision in that eye is lost and cannot be replaced. Enucleation is usually reserved for very large tumors that are not amenable to radiation therapy or tumors involving the optic nerve.

Emerging Research on New Ocular Melanoma Treatment Options

Texas Retina has been involved in ocular melanoma research for over four decades. We serve as active members of the Collaborative Ocular Oncology Group, a network of most major ocular oncology physicians in North America who are pursuing the latest research into ocular (uveal) melanoma.

Texas Retina is also currently participating in two clinical trials, testing potential new treatments for ocular melanoma.

AU-011

In the 1980s, brachytherapy revolutionized how ocular melanoma was treated. However, it unfortunately almost always comes with vision loss.

Together with Aura Biosciences, we are investigating the safety and efficacy of belzupacap sarotalocan (AU-011), a light-activated viral nanoparticle. Texas Retina previously participated in the Phase II trial where Dr. Timothy Fuller was the first in the nation to treat a patient with AU-011 delivered via suprachoroidal injection. Following injection, the tumor is treated with a special ophthalmic laser to activate the viral nanoparticles to selectively destroy the tumor. All of this is done in an outpatient, office-based setting.

In the currently enrolling Phase III trial, patients are randomized to receive a high-dose regimen of AU-011, a low-dose regimen, or sham control. The study’s primary endpoint is time to tumor progression.

With AU-011, we are investigating the potential to treat this deadly disease with less harmful effects on vision. AU-011 has been shown in prior studies to selectively destroy cancer membrane cells while sparing the overlying retina, a tissue essential for good vision.

IDE196-010

Under the leadership of principal investigator Dr. Timothy Fuller, Texas Retina is also enrolling patients in a randomized, Phase III, open-label study of neoadjuvant darovasertib in subjects with primary non-metastatic uveal melanoma (neoadjuvant means therapy before definitive treatment like plaque placement or eye removal).

The clinical trial will determine if this oral agent leads to less vision loss from plaque therapy. In addition, for patients with tumors too large for radiation, this therapy could possibly provide an option to maintain their eye rather than have it enucleated. The hope is that darovasertib can shrink the tumor enough to allow for plaque treatment.

Additional Resource

In addition to the information presented on this page, Texas Retina’s Rajiv Anand, MD, has published a great reference guide for uveal/ocular melanoma patients that is available on Amazon.

Care for Other Eye Cancers

At Texas Retina Associates, we also evaluate and treat a number of other ocular cancers and benign conditions which can convert to cancer (thus requiring lifelong monitoring), including:

Retinoblastoma
Metastatic cancer to the choroid/retina
Choroidal nevus (benign)
Iris nevus (benign)
Congental hypertrophy of the retinal pigment epithelium (benign)
Melanocytoma (benign)
Choroidal hemangioma (benign)

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