Uveal melanoma is the most common cancer that starts in the eyeball in adults. It accounts for about 5% of all melanoma cases. It may be called choroidal melanoma or ocular melanoma.
Like all melanomas, uveal melanoma develops from a melanocyte cell. These cells give skin and eyes their color. Melanoma occurs when a melanocyte mutates and begins to divide uncontrollably.
Melanocytes are found throughout the eye, including the uveal tract. This structure sits between the sclera (the white of the eye) and the retina. The uveal tract has three parts:
- the iris, the colored part of the eye
- the ciliary body, a ring of tissue with muscle fibers that change the size of the pupil and the shape of the lens
- the choroid, also known as the posterior uvea. It is the back part of the eye under the retina. This is the largest part of the uvea and the most common place for uveal melanoma to develop.
Uveal melanoma risk factors
A risk factor is anything that increases your chance of developing a disease. Risk factors for uveal melanoma include:
- Race: White people are more likely to develop uveal melanoma than people of other races.
- Age: The chance for developing uveal melanoma increases with age.
- Nevus of Ota: A large birthmark associated with a high number of melanocytes. This condition causes dark patches to form on the skin or in the eyes.
- Atypical mole syndrome: A condition in which people have a high number of moles, including several that are large, oval shaped or with different colors.
- Hereditary cancer syndromes: A small number of uveal melanoma cases are linked to a mutation in the BAP-1 gene. Learn more about hereditary cancer syndromes.
Uveal melanoma often has no symptoms, especially in its early stages. In these cases, it is often found during routine eye exams.
If there are symptoms, they may include:
- vision changes, including blurry vision
- flashing lights
- a dark spot on the iris
- a change in the shape of the pupil
- eye pain or redness
These symptoms do not always mean you have eye cancer. However, it is important to discuss any eye cancer symptoms with your doctor, since they may signal other health problems.
An accurate diagnosis is the first step toward successful treatment.
Uveal melanoma is usually diagnosed with a thorough exam of the eye, as well as an ultrasound or other diagnostic tests.
This diagnosis depends on the doctor’s expertise. Patients with suspected cases of uveal melanoma should work with an ophthalmologist (an M.D. specializing in the eye) who has received specialty training in diagnosing cancers of the eyeball.
Patients diagnosed with uveal melanoma may undergo a prognostic biopsy. This involves removing and studying cancer tissue. A prognostic biopsy helps identify cancers that have a higher chance of spreading to distant parts of the body, such as the liver. Doctors can use this information to develop a treatment and monitoring plan for each patient.
Prognostic biopsies can be performed with a needle at the time the patient receives radiation therapy. Tissue removed during surgery can also be used for a prognostic biopsy.
The following therapies may be used to treat uveal melanoma.
Radiation therapy uses powerful, focused beams of energy to kill cancer cells. There are several different radiation therapy techniques. Doctors can use these to accurately target a tumor while minimizing damage to healthy tissue.
Radiation treatments for uveal melanoma include:
- Brachytherapy: Brachytherapy delivers radiation therapy with small pieces of radioactive material that are placed on or inside the patient’s body as close to the tumor as possible. This allows doctors to deliver very high doses of radiation directly to the patient’s tumor while limiting radiation exposure to healthy tissue. Learn more about brachytherapy.
- Proton Therapy: Proton therapy is similar to traditional radiation therapy, but it uses a different type of energy that may allow doctors to target tumors with much more accuracy. This limits damage to nearby healthy tissue and allows for the delivery of a more powerful dose of radiation. MD Anderson does not offer proton therapy for uveal melanoma at this time. Read more about proton therapy.
- Stereotactic radiosurgery (SRS): Stereotactic radiosurgery is a non-invasive treatment that uses dozens of tiny radiation beams to accurately target tumors with a single high dose of radiation. Despite its name, SRS is not a surgical procedure. It does not require an incision or anesthesia. For uveal melanoma patients, it is used in limited cases. Read more about stereotactic radiosurgery.
One of the following surgeries may be used to treat uveal melanoma.
- Eye-preserving surgery: Eye-preserving surgery can be performed in select cases. Our highly specialized eye surgeons use the latest surgical techniques to remove the cancer while making every effort to preserve the function and aesthetic appearance of your eye, eyelid and facial area.
- Iridectomy: The iris (colored part of the eye) is removed.
- Enucleation: Enucleation is the removal of the entire eyeball. It is performed on some patients with advanced uveal melanoma.
- Orbital exenteration: This surgery entails the removal of the eye, eyelids, surrounding skin, orbital muscles, fat and nerves. Though it is rarely performed, it is an option for patients with very advanced disease.
- Laser therapy: In laser therapy, a surgeon uses an intense, focused beam of light to destroy eye cancer tissue.
- Eye reconstructive surgery: Following enucleation or exenteration, a specialized team will fit the patient with a prosthetic eye. Each prosthetic is made specifically for the patient, and patients often describe the appearance as excellent.
The immune system finds and defends the body from infection and disease. Cancer is a complex disease that can evade and outsmart the immune system. Immunotherapy improves the immune system’s ability to eliminate cancer.
At this point, immunotherapy is used to treat uveal melanoma that has spread, or metastasized to distant points in the body, such as the liver.
The following immunotherapies are used to treat for uveal melanoma:
- Bispecific T-cell engagers: These drugs help T cells, a type of immune system cell, fight disease by bringing them closer to cancer cells.
- Checkpoint inhibitors: Immune checkpoint inhibitors stop the immune system from turning off before cancer is completely eliminated. Learn more about immune checkpoint inhibitors.
Liver metastases treatment
Uveal melanoma can spread to the liver. Treatments for liver metastases include:
- Embolization: During this procedure, doctors insert a catheter into a large artery and steer it to the liver, near the metastatic tumor. Doctors then inject a substance or material into the artery to stop or slow the cancer’s growth.
- Cryoablation: During this procedure, a special probe is inserted into the tumor and cooled to temperatures well below freezing. A ball of ice forms at the tip of the probe, freezing and destroying cancerous tissue. Cryotherapy is not as invasive as surgery and can sometimes be performed as an outpatient procedure. Learn more about cryoablation.
- Microwave/RF ablation: These treatments use heat to kill cancer cells. During the procedure, a needle-thin probe delivers microwaves or radiofrequency waves directly to the tumor, heating the tissue until it is destroyed. These treatments are not as invasive as surgery and can sometimes be performed as an outpatient procedure. Learn more about microwave/RF ablation.
Uveal melanoma clinical trials
MD Anderson has one of the largest clinical trial programs for uveal melanoma in the world. These can include trials for metastatic uveal melanoma and trials for new types of treatments, including immunotherapy and targeted therapy. Learn more about clinical trials.
Why choose MD Anderson for your uveal melanoma care?
Uveal melanoma is a rare subtype of a rare cancer. It’s important to get care from doctors who have experience treating this disease.
MD Anderson’s work in uveal melanoma dates back decades. The first uveal melanoma research paper from MD Anderson was published in 1981, making our efforts to treat this disease among the oldest in the nation.
Since that time, our faculty have been leaders in uveal melanoma care and research. The have helped conduct some of the most important studies of uveal melanoma and have served as leaders of international healthcare organizations, including the International Society of Ocular Oncology, the American Association of Ophthalmic Oncologists and Pathologists, the American Society Clinical Oncology and the American Association of Cancer Researchers. In addition, public and private organizations often ask our faculty to serve as reviewers for uveal melanoma research grant applications.
Today, specialists in our Ophthalmology Clinic continue to advance care for patients with uveal melanoma. We are one of the few cancer centers in the United States to offer brachytherapy with ruthenium as the radiation source, which may offer improved vision outcomes after treatment compared to other forms of brachytherapy.
We’re also home to one of the largest clinical trial programs for metastatic uveal melanoma. As a rare cancer, there are few approved treatments for the disease. To help provide the best possible care, MD Anderson conducts trials for new uveal melanoma treatments including immunotherapy and targeted therapy. Trials also include efforts to reduce side effects from ocular radiation therapy. You may even be eligible for clinical trials to help monitor your cancer.
Uveal melanoma often spreads to other parts of the body, including the liver. We can offer a genomic prognostication of uveal melanoma tumors to identify cancers that are likely to spread. If you have uveal melanoma, we will put you on a tailored surveillance program to monitor the disease and catch any spread as early as possible.
And at MD Anderson you're surrounded by the strength of one of the nation's foremost comprehensive cancer centers. From support groups to rehabilitation services to integrative medicine care, we have all the services needed to treat the whole person – not just the eye.