Ocular/Uveal Melanoma: Staging and Treatment

What is staging for cancer?

Staging is the process of learning how much cancer is in your body and where it is. For ocular/uveal melanoma, an ultrasound, MRI, CT scan, biopsy, and optical coherence tomography (OCT) may be used to stage your cancer. OCT is a test that takes images of the back of the eye. Your providers need to know about your cancer and your health so that they can plan the best treatment for you.

How is ocular/uveal melanoma staged?

Staging helps guide your treatment options. Staging looks at the size of the tumor, where it is, and if it has spread to other organs.

Ocular/uveal melanoma is staged using either the TNM system or the Collaborative Ocular Melanoma Study (COMS).

TNM Staging System

A staging system called the “TNM system,” as described by the American Joint Committee on Cancer, helps guide the staging of ocular/uveal melanoma. It has three parts:

• T- Describes where the tumor is.
• N- Describes if the cancer has spread to the lymph nodes.
• M- Describes if the cancer has spread to other organs (metastases).

Your healthcare provider will use the results of the tests you had to figure out your TNM result and combine these to get a stage from I (one) to IV (four).

The staging systems are very complex. Below is a summary of the TNM staging for choroidal and ciliary body melanomas, which are part of the uvea. Talk to your provider about the stage of your cancer.

Stage I (T1a, N0, M0): The tumor is 12 mm (millimeters) wide or less and 3 mm tall or less, OR it is 9 mm wide or less and between 3.1 and 6 mm tall. It is not growing in the ciliary body or outside the eye (T1a). The tumor has not spread to the lymph nodes (N0), or to any other parts of the body (M0).

Stage IIA (T1b or T2a, N0, M0): The tumor is 12 mm (millimeters) wide or less and 3 mm tall or less, OR it is 9 mm wide or less and between 3.1 and 6 mm tall. It is growing in the ciliary body (T1b).

OR, the tumor is between 12.1 mm and 18 mm wide and 3 mm tall or less. OR, the tumor is between 9.1 mm and 15 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is 12 mm wide or less and between 6.1 mm and 9 mm tall. It is not growing in the ciliary body or outside the eye (T2a).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

Stage IIB (T2b or T3a, N0, M0): The tumor is between 12.1 mm and 18 mm wide and 3 mm tall or less. OR, the tumor is between 9.1 mm and 15 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is 12 mm wide or less and between 6.1 mm and 9 mm tall. It is growing in the ciliary body (T2b).

OR, the tumor is between 15.1 mm and 18 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is between 12.1 mm and 18 mm wide and 6.1 mm to 9 mm tall. OR, the tumor is 18 mm wide or less and between 9.1 mm and 12 mm tall. OR, the tumor is 15 mm wide or less and between 12.1 and 15 mm tall. It is not growing in the ciliary body or outside the eye (T3a).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

Stage IIIA

(T2c or T2d, N0, M0): The tumor is between 12.1 mm and 18 mm wide and 3 mm tall or less. OR, the tumor is between 9.1 mm and 15 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is 12 mm wide or less and between 6.1 mm and 9 mm tall. It is not growing in the ciliary body, but is growing outside the eye. The growth is 5 mm or less across (T2c).

OR, the tumor is between 12.1 mm and 18 mm wide and 3 mm tall or less. OR, the tumor is between 9.1 mm and 15 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is 12 mm wide or less and between 6.1 mm and 9 mm tall. It is growing in the ciliary body and outside the eye. The growth is 5 mm or less across (T2d).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

(T3b or T3c, N0, M0): The tumor is between 15.1 mm and 18 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is between 12.1 mm and 18 mm wide and 6.1 mm to 9 mm tall. OR, the tumor is 18 mm wide or less and between 9.1 mm and 12 mm tall. OR, the tumor is 15 mm wide or less and between 12.1 and 15 mm tall. It is growing in the ciliary body (T3b).

OR, the tumor is between 15.1 mm and 18 mm wide and between 3.1 mm and 6 mm tall. OR, the tumor is between 12.1 mm and 18 mm wide and 6.1 mm to 9 mm tall. OR, the tumor is 18 mm wide or less and between 9.1 mm and 12 mm tall. OR, the tumor is 15 mm wide or less and between 12.1 and 15 mm tall. It is not growing in the ciliary body, but it is growing outside the eye. The growth is 5 mm or less across (T3c).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

 (T4a, N0, M0): The tumor is bigger than 18 mm wide and can be any height. OR, the tumor is between 15.1 mm and 18 mm wide and more than 12 mm tall. OR, the tumor is less than 15 mm wide and more than 15 mm tall. It is not growing in the ciliary body or outside the eye (T4a).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

Stage IIIB (T3d, N0, M0): The tumor is between 15.1 mm and 18 mm wide and 3.1 mm and 6 mm tall. OR, the tumor is between 12.1 mm and 18 mm wide and 6.1 mm to 9 mm tall. OR, the tumor is 18 mm wide or less and between 9.1 mm and 12 mm tall. OR, the tumor is 15 mm wide or less and between 12.1 and 15 mm tall. It is growing in the ciliary body and outside the eye. The growth is 5 mm or less across (T3d).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

(T4b, N0, M0): The tumor is bigger than 18 mm wide and can be any height. OR, the tumor is between 15.1 mm and 18 mm wide and more than 12 mm tall. OR, the tumor is less than 15 mm wide and more than 15 mm tall. It is growing in the ciliary body (T4b).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

(T4c, N0, M0): The tumor is bigger than 18 mm wide and can be any height. OR, the tumor is between 15.1 mm and 18 mm wide and more than 12 mm tall. OR, the tumor is less than 15 mm wide and more than 15 mm tall. It is not growing in the ciliary body, but is growing outside the eye. The growth is 5 mm or less across (T4c)

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

Stage IIIC (T4d or T4e, N0, M0): The tumor is bigger than 18 mm wide and can be any height. OR, the tumor is between 15.1 mm and 18 mm wide and more than 12 mm tall. OR, the tumor is less than 15 mm wide and more than 15 mm tall. It is growing in the ciliary body and outside the eye. The growth is 5 mm or less (T4d).

OR, the tumor is any size and it is growing outside the eye. The growth is 5 mm or bigger (T4e).

The tumor has not spread to any lymph nodes (N0), or to any other parts of the body (M0).

Stage IV (Any T, N1 or any N, M0-M1): The cancer is any size and may or may not have spread to the lymph nodes or other body parts.

Collaborative Ocular Melanoma Study (COMS) Staging

Your provider may also use Collaborative Ocular Melanoma Study (COMS) staging for iris melanoma. The iris is also part of the uvea. COMS staging is based on tumor size and is organized in 3 categories:

• Small: The tumor is between 1 mm and 3 mm tall and between 5 mm and 16 mm across.
• Medium: The tumor is between 3.1 mm and 8 mm tall and 16 mm or less across.
• Large: The tumor is bigger than 8 mm tall and more than 16 mm across.

How is ocular/uveal melanoma treated?

Treatment for ocular/uveal melanoma depends on many factors, like your cancer stage, age, overall health, and testing results. Your treatment may include:

• Surgery.
• Immunotherapy.
• Targeted therapy.
• Chemotherapy.
• Radiation Therapy.
• Clinical Trials.

Surgery

Surgery is the most common treatment for ocular/uveal melanoma. The goal of surgery is to remove as much of the tumor as possible. Some types of surgery may include getting an artificial (fake) eye. Your healthcare provider will try to match it to the size and color of your other eye. These are the types of surgery that may be used:

• Resection: The tumor and part of the area around it are removed.
• Enucleation: If the tumor has spread to the optic nerve, this type of surgery may be used. Often, you will need an artificial eye after this surgery.
• Exenteration: This type of surgery is used when your melanoma is in an advanced stage. It often involves removing the eyelid, eye socket muscles, fat, and nerves. Often, you will need an artificial eye after this surgery.

Immunotherapy

Immunotherapy is the use of medication to help your immune system fight cancer. An immunotherapy medication used to treat ocular/uveal melanoma is Tebentafusp-tebn (Kimmtrak®).

Targeted Therapy

Targeted therapy medications target something specific to the cancer cells, stopping them from growing and dividing. Some types of skin and eye melanomas have a mutation (change) in the BRAF gene. If this gene is found, BRAF inhibitor medications such as vemurafenib (Velboraf®), dabrafenib (Tafinlar®), and encorafenib (Braftovi®) may be used.

Chemotherapy

Chemotherapy is used to kill or damage cancer cells. Examples of chemotherapy medications that may be used to treat ocular/uveal melanoma are dacarbazine (DTIC, DTIC-Dome®), temozolomide (Temodar®) IV formulation, paclitaxel (Taxol®), paclitaxel protein-bound (Abraxane®), cisplatin (Platinol®), and carboplatin (Paraplatin®).

Radiation Therapy

Radiation therapy is the use of high-energy x-rays to kill cancer cells. Radiation is not used often to treat ocular/uveal melanoma, but can be used:

• If surgery is not an option for some reason. Radiation can be used to treat very early-stage melanoma.
• After surgery to prevent the cancer from coming back (recurrence) or to treat cancer that has already come back.
• To help with symptoms of the cancer. This is called palliative radiation.

Clinical Trials

You may be offered a clinical trial as part of your treatment plan. To find out more about current clinical trials, visit the OncoLink Clinical Trials Matching Service.

Making Treatment Decisions

Your care team will make sure you are a part of choosing your treatment plan. This can be overwhelming as you may be given a few options to choose from. Take the time to meet with different providers and think about your options and what is best for you. This is a personal decision. Friends and family can help you talk through the options and the pros and cons of each, but they cannot make the decision for you. You need to be comfortable with your decision – this will help you move on to the next steps. If you ever have any questions or concerns, be sure to call your team.