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Ocular Melanoma

Treatment

How is Ocular Melanoma Treated?

Once a diagnosis of ocular melanoma (OM) is made, choice of treatment depends on the location, site of origin within the eye, size of the tumor, as well as patient age, overall health, visual potential and status of the unaffected eye. Because the conventional systemic (full-body) therapies don’t tend to work in OM, early diagnosis and treatment is essential. If the melanoma has metastasized, or spread, it can be more difficult to treat. Find an OM specialist to help you with your treatment decision.

Primary Ocular Melanoma Treatment

Primary ocular melanoma means that the tumor originated in the eye. The goals of treating the primary tumor are to stop tumor growth, spare the eye, preserve vision and improve patient survival. Treatment most often includes a combination of radiation and surgery but depends on the size and location of the tumor, among other factors.

Radiation
For most small and medium-sized tumors, radiation is the recommended treatment. There is currently no evidence that one form of radiation is better than the other. The different types of radiation therapy include:

  • Plaque Brachytherapy (Radiotherapy) – A thin piece of metal, called a plaque, is sewn onto the outside wall of the eye. The radioactive seeds in the plaque give off radiation, which aims to kill the cancer. The treatment usually lasts a few days and the plaque is removed at the end of treatment. This is the most common therapy in the United States for posterior (choroidal and ciliary body) ocular melanoma and is considered the standard of care for most OM patients with small or medium-sized tumors. After this treatment, removal of the eye is not usually necessary, and most patients are able to retain some degree of visual function.
  • Proton Beam Radiotherapy – Clips are surgically placed onto the eye a the tumor base and an external beam of radiation is aimed at the tumor, most often through the front of the eye. Treatment is usually finished after 3-5 daily outpatient treatments.
Surgery
In some cases, the recommended treatment for ocular melanoma is surgical removal of the tumor. Surgery is often recommended for tumors of large size and for iris melanomas. Surgery may also be recommended after radiation. Types of surgery include:

  • Enucleation – Removal of the eye is sometimes recommended in cases involving large tumors. Following enucleation, an artificial eye may be placed in the socket and, with the help of an ocularist, made to look like a natural eye.
  • Iridectomy – Removal of part of the iris where the tumor is present.
  • Iridocyclectomy – Removal of part of the iris (iridectomy) as well as the ciliary body (cyclectomy) where the tumor is present.
  • Trans-Scleral Local Resection – Removal of the tumor through an opening in the wall, or the white part, of the eye. This is often used when the tumor is large. A radioactive plaque may be placed over the treated area to reduce the risk of recurrence.
Other Treatments
Transpupillary Thermotherapy – The temperature of the tumor is slowly raised, killing cancer cells and shrinking the tumor.  This treatment is most often used for small tumors in the retina and choroid.

Cryotherapy – The temperature of the tumor is lowered since melanocytes are susceptible to freezing. This treatment option is not frequently used in OM.

Gamma Knife – A focused, single dose of radiation is given to the tumor, sparing healthy tissue in and around the eye.

Intraocular Injections – Injections in the eye are used to administer medications to treat a variety of ocular conditions. These medications may include steroids for inflammation and or anti-angiogenic factors, which shrink blood vessels. Intraocular injections might be used if changes have occurred to the retina and optic nerve due to radiation.

Adjuvant Treatment
In skin melanoma, adjuvant treatment is treatment used after the primary treatment (most often, surgery), to prevent the spread of disease. It can also refer to treatment used in addition to the primary form of treatment. Currently, in ocular melanoma, all adjuvant treatments are in the clinical trial stage and nothing yet has been proven to show great results.

Systemic Ocular Melanoma Treatment

If OM has spread beyond the eye, it is considered metastatic. Approximately half of OM patients will develop metastatic disease, although each person’s individual risk is based on factors such as tumor genetics and other clinical characteristics. When OM metastasizes, it first spreads to the liver nearly 90% of the time.

Systemic Treatments

Currently, only one FDA approved systemic treatment for metastatic ocular melanoma is available. In January 2022, the FDA approved tebentafusp-tebn (Kimmtrak), a bispecific gp100 peptide-HLA-directed CD3 T cell engager, for HLA-A*02:01-positive adult patients with unresectable or metastatic uveal melanoma. Other clinical trials are ongoing for additional systemic treatment options.

Webinar: First FDA Approval for Uveal Melanoma

 

  • Immunotherapy – A type of systemic treatment given to activate a person’s immune system so that it will destroy melanoma cells within the body. Several immunotherapies are FDA-approved for cutaneous melanoma and some are being studied in ocular melanoma.
  • Targeted therapy – A form of treatment in which drugs are developed with the goal of destroying cancer cells while leaving normal cells intact. These drugs are designed to interfere with the specific molecules, genetic mutations in the tumor itself, that are driving the growth and spread of the tumor.
  • Chemotherapy – Overall, chemotherapy has not been shown to be effective for ocular melanoma. However, it may still be recommended in some cases.
Treatments Targeting the Liver

These treatments may be used in conjunction with systemic (full body, or through the bloodstream) treatments, so be sure to discuss them with your treatment team.

  • Resection – Surgical removal of the tumor. Resection is mainly used when a single tumor is present. It is often reserved for patients who are several years out from a primary diagnosis and repeated imaging studies show only one tumor. Since lever resection can sometimes remove some healthy tissue along with the tumor, it is reserved for select cases.
  • Ablation – Ablation involves inserting small probes into tumors and heating (i.e. radio frequency ablation, microwave ablation) or freezing (cryoablation) the tumors to kill them. This can be done through the skin or surgically. Like resection, this is typically not recommended if multiple tumors are present. it can be used in other areas of the body beyond the liver, such as the lung, kidney and soft tissue.
  • Radiation – Targeted radiation can be used to treat liver disease. This includes treatments such as stereotactic radiosurgery (Gamma Knife and Cyber Knife), that can be used to target specific tumors while sparing normal tissue. Radiation can be used to treat other areas of the body including lung, bone, and brain, and can be used to treat isolated metastases or to relieve symptoms caused by a specific tumor.

Transarterial Catheter-Directed Liver Therapies

Regional liver directed therapies that affect the entire liver include:

  • HEPZATO KIT (melphalan/Hepatic Delivery System [HDS]) – is a liver-directed treatment for adult patients with metastatic uveal melanoma (mUM) with unresectable hepatic metastases affecting less than 50% of the liver and no extrahepatic disease, or extrahepatic disease limited to the bone, lymph nodes, subcutaneous tissues, or lung that is amenable to resection or radiation. This is the first FDA approval of a liver-directed therapy for patients with metastatic uveal melanoma.
  • Immunoembolization – Immunotherapy drugs called cytokines are injected into the hepatic arteries (the arteries that supply the liver). This is combined with embolization of the hepatic (liver) artery. Embolization blocks off the blood supply to the tumors using injections into the arteries. The goal is to induce an inflammatory response in the tumor. This process may also stimulate the immune system outside the liver, which could help suppress tumor growth in other places of the body.
  • Chemoembolization (TACE) – A chemotherapy drug is injected into the hepatic arteries. The goal is to block off the blood supply to the tumors.
  • Radioembolization – Small beads, embedded with a radioactive material, are injected into the hepatic arteries. These microspheres emit high doses of radiation to the tumor cells to destroy them. This treatment is sometimes referred to as Y-90 radioembolization, Sirspheres or Theraspheres.
  • Hepatic Arterial Chemoinfusion (HAI) – Infusion of chemotherapy into the liver through a specialized infusion system in which a catheter is placed into the hepatic artery to directly and continuously deliver the chemotherapy to the liver. This direct infusion allows for fewer side effects of chemotherapy and allows high doses to be delivered.
  • Isolated Hepatic Perfusion (IHP) – A different type of procedure to deliver high doses of chemotherapy to the liver. In IHP, a catheter is placed into the hepatic artery and another is placed into the vein that takes blood away from the liver. This temporarily separates the liver’s blood supply from blood circulating through the rest of the body and allows high doses of chemotherapy to be directed only to the liver. When this is done percutaneously, or through the skin, it is referred to as Percutaneous Hepatic Perfusion (PHP).
Clinical Trials
There is some important information you should know about clinical trials in ocular melanoma:

  • Trials help physicians determine which patients should receive which drugs in which order (sequence).
  • Trials can give you access to therapies that are not yet approved by the FDA but that may be more effective in the treatment of ocular melanoma.
  • It is often free to participate in clinical trials and sometimes you may even have more diagnostic tests while participating than you otherwise would have during regular treatment. Be sure you understand your health insurance policy and the coverage of the clinical trial so you are fully aware of what is covered and what is not.
  • You can choose to stop participating in a trial at any time.

Visit the MRF’s Clinical Trial Finder to find an ocular melanoma trial.