How Ocular Melanoma is Treated

Melanoma 101 Types of Melanoma How Ocular Melanoma is Treated

Who treats ocular melanoma?

Different types of doctors and other healthcare professionals often work together as a team to provide treatment for people with ocular melanoma. The treatment team may include doctors that specialize in treating cancer with radiation (called a radiation oncologist), medications (medical oncologist), and/or surgery (surgical oncologist), together with doctors who specialize in treating eye disorders (ophthalmologist) or ocular oncologists who are also eye surgeons.

What are the treatments for uveal melanoma?

Treatment for uveal melanoma depends on factors like its stage, location within the uveal tract, and whether the melanoma has spread to other parts of the body. Doctors also consider the age, overall health, and preferences of the patient in determining what treatments are most appropriate. The likelihood of saving vision in the affected eye is also considered. Treatment decisions require careful discussion between members of the healthcare team who are familiar with all aspects of the case, with input from the patient.

Uveal melanoma in the eye is usually treated with radiation therapy or surgery.

  • Radiation therapy is commonly used to treat small- or medium-sized uveal melanomas. Local radiation works by damaging the DNA in the cancer cells so that the cells die or lose their ability to divide to make more cancer cells, leading to tumor shrinkage.
    • Brachytherapy (sometimes called ‘plaque therapy’, endocurietherapy, or sealed source radiotherapy) is the most common type of radiotherapy used to treat uveal melanomas in the US. Brachytherapy involves implanting a small disk containing radioactive material into the eye socket near the base of the tumor and removing it after several days.
    • External-beam charged particle radiation (also called proton-beam radiation), which uses a machine outside of the body to deliver laser beams of charged particles that damage the tumor cells in the eye, is another common approach that may be used to treat large uveal tumors or those growing near the optic nerve.
    • External-beam radiation therapy may be used after surgery to prevent recurrence (called an adjuvant therapy) or to alleviate symptoms (sometimes called palliative treatment).
    • Stereotactic radiosurgery, where a machine is used to deliver precisely focused beams of high doses of radiation to a small, targeted area containing the tumor cells is also sometimes used to treat large uveal melanomas.
  • Surgery, sometimes called surgical resection or surgical excision, involves removing part or all of the affected eye. In most cases when surgery is considered, there has been the decision for surgical removal of the entire eye, called enucleation. Enucleation is recommended for large uveal melanomas that cannot be effectively treated with radiation, or that are causing symptoms like blindness or pain or tumors with significant extensions outside the eye.

Uveal melanoma that has spread to distant organs is sometimes treated with medications that affect the whole body. If the liver is affected, doctors may recommend treatment approaches that target the cancer cells growing in the liver, called liver directed or loco-regional therapy.

  • Tebentafusp-tebn (Kimmtrak) is the first and only medication approved by the US FDA to treat people with HLA-A*02:01-positive uveal melanoma that has spread to distant organs or that cannot be treated with surgery (unresectable uveal melanoma). Tebentafusp (often referred to as “tebe”) is a type of medication that targets the immune system, called an immunotherapy. It engages a certain type of immune cells, called T cells, to target and kill uveal melanoma cells that present a protein called gp100 at their surface. Tebentafusp was approved in 2022 based on the results of a Phase 3 clinical trial that was done in 378 people with unresectable or metastatic uveal melanoma. In that trial, overall survival was longer in patients who received tebentafusp compared with a control group of patients who received other medications (pembrolizumab, ipilimumab, or dacarbazine). At one year, overall survival was 73% in the tebentafusp group versus 59% in the control group.
  • There are no other approved medications to treat uveal melanoma that has spread to distant organs. Some medications, including chemotherapy drugs and drugs that target the immune systems (immunotherapies) have been used in small groups of patients but have not shown clear benefits in improving survival.
  • Clinical trials may be the best option for some people with uveal melanoma that has spread to distant parts of the body. If you are diagnosed with metastatic uveal melanoma, it is important to discuss the possible treatment options with your healthcare team, including what clinical trials may be open to you.
  • Different types of liver-directed therapies may be used to limit cancer growth and alleviate symptoms caused by the melanoma growing in the liver. Approaches may include injecting medicines or other substances into the liver (embolization), regional isolation-perfusion of the liver, procedures that use heat (thermal ablation) or freezing (cryotherapy) temperature to destroy the cancer cells (called ablation), surgical removal of the liver mass, or radiation of the cancer cells in the liver.

What is the best treatment for uveal melanoma?

While a number of treatment options are available (as described above), there is no ‘one-size-fits-all’ best treatment for uveal melanoma. Each treatment approach comes with its own set of risks and benefits. Doctors may recommend different treatment approaches for their individual patients depending on factors, including where the melanoma is located within the uveal tract, the stage, possible side effects, and the individual patient’s preference and overall health.

After being diagnosed with uveal melanoma, it is important to get an opinion at a center with experience in treatment this rare melanoma. It is important to discuss the different treatment options available to you with your care team. Asking questions about anything that is unclear is an important part of making an informed decision. For example, it is important to understand the treatment goals, what benefit is reasonable to expect, and the side effects you might encounter.

What are the treatments for conjunctival melanoma?

Conjunctival melanomas that have not spread outside of the eye are usually treated by surgically removing the tumor. Doctors may recommend radiation therapy (including brachytherapy or external beam radiation therapy) or topical medications (like interferon or Mitomycin-C) after surgery to help prevent recurrence and metastasis. In some cases, cryotherapy, which uses extreme cold to freeze and destroy abnormal tissue, may be used to treat conjunctival melanomas.

In patients with very aggressive conjunctival melanoma and advanced local disease, a surgical approach called orbital exenteration may be recommended. Orbital exenteration requires removal of the eye and the surrounding tissues.

There is no standard recommendation for the treatment of patients with advanced conjunctival melanoma primarily because of the rarity of the disease. Generally, these patients are treated similarly to patients with advanced cutaneous melanoma, using immunotherapy, and targeted therapy, if applicable. In patients with tumors over 2 mm or other adverse features, sometimes a procedure is done to determine whether the melanoma has spread to a nearby lymph node. This procedure is called sentinel lymph node mapping and biopsy.  Should the melanoma be found in the lymph node, patients may then be recommended for additional treatment to try to prevent further spread, which often includes immunotherapy. A biopsy for genetic information may also be done to help doctors get more information about the tumor and possible treatments. Conjunctival melanoma may have similar mutations to skin melanoma, such as BRAF, which can be a target for treatments.

Will I lose my eye?

Removing the eye, called ‘enucleation’, was common in the past, but has become less common in recent years. Radiation therapy (brachytherapy or external-beam radiation therapy) can be used in many cases to preserve the affected eye, even in larger tumors.

However, removing the eye is still sometimes the only choice to treat certain types of large tumors. Factors like the size of the tumor, where it is located in the eye, and whether it has spread to nearby structures or other parts of the body determine what treatment, including removing the eye, may be most appropriate for an individual patient with ocular melanoma to prevent it from spreading.

Patients can have difficulty with depth perception after an eye is removed, although most people eventually adjust to the difference.

Cosmetic surgery can be used with good results to insert a prosthetic artificial eye into the area where the eye was removed. Artificial eyes typically look very similar to the person’s remaining eye and can move very similarly to a natural eye.