Uveal Melanoma

About Uveal Melanoma

When faced with a possible diagnosis of uveal melanoma, you can turn to our experienced, board-certified ocular oncologist at Retina Consultants of Texas. Dr. Amy Schefler is our ocular oncologist and surgeon with many years of expertise in working with patients with cancer. Uveal melanoma is a rare malignant cancer of adults with less than 2,000 new cases diagnosed in the United States per year. These tumors of the eye often emerge from a nevi (a medical term for mole) within the pigmented layers of the eye — the choroid, the iris, or the ciliary body. Each tumor consists of a mass of colored cells, which can present as brown or yellow.

When the tumor is located in the middle or back of the eye, it cannot be seen with the naked eye. It is most commonly detected in a standard optometry or ophthalmology appointment, not by pain or symptoms. Some patients do report floaters or decreased vision, but uveal melanoma typically does not cause pain. With respect to size, choroidal and ciliary body melanomas are generally 2mm – 12mm tall and 5mm – 18mm wide at the time that they are diagnosed. The overall size of the tumor is small (often smaller than a jelly bean), but the impact on the eye is rather significant due to the delicate structures of the eye. In most cases, melanomas arise from pre-existing nevi (freckles or moles), which are benign in appearance and then transform into cancer. Once the tumor cells develop the ability to divide and grow quickly, the cancer should be treated as soon as possible.

Ask The Doctor: Uveal Melanoma: Dr. Schefler

Known Causes

Uveal melanoma is most common in patients who are Caucasian, have lightly colored blue or green eyes, and are over the age of 60. Findings have not been consistent in pinpointing something in the diet, genetics, or environmental risk factors to link uveal melanoma to the development of this cancer. There are rare cases in patients exposed to extremely high doses of radiation, such as arc welders. A small percentage of patients inherit a mutation in a gene that puts them at risk for developing this cancer. In these families, members who inherit this faulty gene can develop uveal melanoma and/or other unusual cancers such as mesothelioma (a cancer of the lining of the lung), renal cell carcinoma (cancer of the kidney), or certain skin cancers.

Diagnosis

Unlike many cancers, we are able to diagnose uveal melanoma 95% of the time without a biopsy. We have the ability to characterize the tumor in the office with many sophisticated diagnostic testing techniques, such as ultrasound, ophthalmic photos, ophthalmic fluorescein angiography (a testing with a special dye), and optical coherence topography (a magnified image of the retina)[1] (Medina et al. 2014) and optical coherence topography-angiography (a magnified image of the retinal blood vessels). At the time of treatment with permission from our patients, we do often take a sample of the tumor for confirmation of the diagnosis, genomic testing, and research.

Treatment and Prognosis

Each uveal melanoma patient at RCTX is managed by a large team of experts at Houston Methodist Hospital, including a radiation oncologist, melanoma medical oncologist, ophthalmic pathologist, geneticist, researchers, and others.

Plaque Surgery/Brachytherapy

Brachytherapy is the standard, accepted form of therapy for uveal melanoma in the U.S. It is a form of very focused radiation that involves the placement of a radioactive disc on the surface of the eye. The disc has radioactive seeds, or beads that are made of an Iodine-125, Ruthenium, or other more rarely used isotopes. We work with our radiation oncology team at Houston Methodist to customize each plaque for every patient, based on the size and configuration of the tumor and the anatomy of the surrounding structures, in order to maximize the death of the tumor cells and minimize the damage to the surrounding healthy retina. The radiation disc is surgically placed on the outer surface of the eye and stays there for several days while the radiation treats the tumor. The patch is removed from the surface of the eye in a second surgery. The radiation kills the tumor cells so that they stop growing. The advantage of this treatment is that the vast majority of patients keep their eyes intact and enjoy useful (and in many cases, excellent) vision. Because our team has extensive experience in placing radioactive plaques, we are able to treat many patients, even those with very small, or very large tumors, safely, including those who are told at other centers that the eye must be removed.

Enucleation

If the eye must be removed to treat this cancer, Dr. Schefler will perform a procedure called an enucleation. This is the oldest form of uveal melanoma treatment. The advantage of this surgery is that it is a fast, definitive treatment that requires limited ophthalmic follow-up. However, you will not be able to preserve any of your natural eye or vision. Our team will do all that is possible to save your eye and will only resort to enucleation if it is necessary. This surgery takes about an hour to remove the eye. The muscles that move the eye, the conjunctiva (the skin of the eye), and the eyelids will all remain intact. Your surgeon will insert a sphere-shaped implant into the orbital cavity to fill the space. Your eye muscles will be reattached to the implant to create movement. After approximately six weeks when the tissues have healed, we will refer you to an ocularist who will fit you for an ocular prosthesis with a special contact lens, which is painted to match the other eye. The prosthesis does not move perfectly like a normal eye, but it moves very well and it will look natural.

Nanoparticle Therapy (clinical trial)

Dr. Schefler and other leading researchers in the U.S. (such as Wills Eye Hospital, Massachusetts Eye and Ear, and others) are currently involved in a multi-center collaborative Phase 2 and 3 clinical trials of novel treatments for uveal melanoma. One such trial involves nanoparticle therapy. Read about the trial at this link for details.

Research in Uveal Melanoma

We have many research trials for patients with choroidal nevi and uveal melanoma at Retina Consultants of Texas. These include:

• Multiple studies of the genetics in the patients’ blood and tumor(s) to understand why some tumors will grow or not, and which ones will develop metastases (cancer cells that have spread to other parts of the body)
• In treatment studies, injections are given of medications to improve the patient's vision after plaque therapy and to minimize the radiation side effects
• Family history studies help us understand the relationship between patients’ family histories of cancer and their own risks for developing uveal melanoma

Comparing Treatments

There was a large multi-center study across the U.S. in the 1980s and 1990s called the Collaborative Ocular Melanoma Study (COMS), sponsored by the National Institute of Health in which enucleation was compared to plaque surgery. The study demonstrated that survival for patients was the same regardless of the treatment. Given the results of this study, most patients are able to choose between these treatments and most choose brachytherapy.

Dr. Schefler at Retina Consultants of Texas uses a minimally invasive technique using advanced retinal instruments to perform fine needle aspiration biopsies for confirmation of diagnosis and genetic testing[2] (Singh et al. 2016). The biopsy, performed prior to plaque surgery, does not result in vision loss and causes no discomfort. This procedure is safe even if very small or very large tumors. Our team has published multiple reports in the medical literature about our extensive experience with these techniques. Collected uveal melanoma cells provide results which are used to guide how often patients need to be monitored by our melanoma medical oncologists after their plaque surgery or enucleation[3, 4] (Plasseraud et al. 2016; Aaberg et al. 2020). At times, we also have patients enroll in experimental clinical trials.

Let's Take a Look

You deserve a compassionate, highly specialized ophthalmologist surgeon to help treat and manage your care when you are faced with uveal melanoma. At Retina Consultants of Texas, it is our mission to offer a full range of diagnostic and treatment techniques to serve your needs. Please call and schedule an appointment if you have been referred to by your primary optometrist or would like a second opinion. Your eye health is in your hands, and we would like to assist you with excellent retinal care.

Neil’s Experience

“I will never forget the day I met Dr. Amy Schefler. I was 54 years old, it was Memorial Day weekend, and she gave me the news that I had a uveal melanoma, a very serious and life-threatening form of cancer. Further genetic testing showed that my tumor was a more serious type, fatal in about 50% of cases.

As you can imagine this was life-altering news so I asked the normal questions: Are you sure? Should I get a second opinion? What are the chances you are wrong? Dr. Schefler answered with immense integrity. She outlined the treatment options and told me to take some time to think about them and let her know.

Naturally, the Memorial weekend I had planned ... enjoying time with family and friends, boating, and grilling outside ... did not materialize. Instead, I spent the weekend researching uveal melanomas and the top medical professionals in this field. Turns out that Dr. Schefler and her team are indeed some of the very elite and most qualified individuals in ocular oncology worldwide.

Little did I know that during my initial visit, Dr. Schefler and her team had taken precise measurements of my tumor and its location and made all the necessary preparations to start treatment immediately. After a depressing weekend, I called and said I was ready to move forward with treatment. The fact that she and her team had been so proactive on my initial visit meant that I could start treatment the following week.

On one of my follow-up visits, I told Dr. Schefler how fortunate I felt that I had ‘randomly’ stumbled upon her as my doctor, with a condition that was so precisely matched to her area of expertise. She said, “serendipitous isn’t it?” I replied “yes, very serendipitous.” (When she left the room I pulled out my phone to look up its meaning!)

It has been six years since my diagnosis, and I can honestly say that without the expertise, professionalism, dedication and follow up of Dr. Schefler and her team, I would not be here today. Not only did she save my life, but she positively impacted the lives of my family, friends, and colleagues.

It is hard to put into words what it is like to have someone take steps on your behalf so that your life is not cut short, and so that your wife and children are not devastated. I am eternally grateful to Dr. Schefler and her team for this incredible turn of events.

Very serendipitous!”

Sincerely,
Neil R.