Ocular Melanoma Patient and Caregiver Guide

Ocular Melanoma Patient and Caregiver Guide Support and Education When It Matters

CURE OM (the Community United for Research

and Education of Ocular Melanoma) is the MRF’s initiative to increase awareness, education and research funding for ocular melanoma, while improving the lives of people affected by this disease.

An ocular melanoma (OM) diagnosis can be a scary and overwhelming experience, and it’s important to learn all you can about your diagnosis. The truth is, informed and empowered patients live longer, better lives. By arming yourself with knowledge, you’ll better understand what you’re up against. Soon, you will likely know more about melanoma than you ever thought possible. You will become familiar with terms and language you never knew before. You will understand the importance of regular skin exams, eye exams and the difference between an optometrist and an ophthalmologist. Researchers are also exploring new therapies in clinical trials that aim to improve outcomes and, in some cases, preserve vision. As a result of all of this knowledge, you may even help spread awareness about melanoma and early detection. This is not a community anyone wants to join. However, it is important to know that You Are Not Alone. At the end of this section, you will find ideas on how to connect with others who have been diagnosed and, if you’d like, ways to get involved in the fight against this disease. Just Diagnosed With Ocular Melanoma… Now What?

In this guide, words in dark blue are defined in the glossary on page 26 .

This guide was developed by the Melanoma Research Foundation’s CURE OM initiative, which focuses on increasing awareness, education and research funding for ocular melanoma while supporting those affected. To learn more, go to melanoma.org/cure-om/

Melanoma is a type of cancer, most often of the skin. However, melanoma can also occur in the eye (ocular melanoma). Ocular Melanoma — The Basics

Ocular melanoma (OM), or melanoma of the eye, is the most common primary

Sclera

Anterior chamber aqueous body

eye tumor in adults. It is the second most common form of melanoma in the

Choroid

Cornea

Retina

Pupil

United States. Like all forms of melanoma, OM

Fovea

Lens

begins in melanocytes — the cells that color

Blood vessels

Iris

the skin and eyes, and form moles.

Ciliary body

Optic nerve

Suspensory ligament

Optic disc blind spot

Vitreous body

What you need to know

So what do you do if you have just been diagnosed with OM?

What to do next? First, take a breath, stay calm and keep reading.

Once you feel ready, it might help you to learn more about Ocular Melanoma. Research Educate Advocate

Types of Ocular Melanoma Different types of melanoma of the eye include:

UVEAL MELANOMA The uveal tract is made up of three main sections: the choroid, the iris and the ciliary body. Uveal melanoma (UM) can form in any of these layers and is named for where it forms. There are around 2,000–2,500 new cases diagnosed each year in the US.

CONJUNCTIVAL MELANOMA The conjunctiva is the clear tissue that covers the white part of the eye, as well as the inside of the eyelids. Conjunctival melanoma is very rare. About 500 people are diagnosed in the United States per year. It often appears as a raised tumor and may contain little or even no pigment. Conjunctival melanoma most commonly occurs in the bulbar conjunctiva — the mucous membrane that covers the outer surface of the eyeball. Unlike other forms of ocular melanoma that spread most often to the liver, when conjunctival melanoma spreads, it most often spreads to the lymph nodes and lungs.

Choroid

Iris

Conjunctival Melanoma

Ciliary body

Tumors

Iris

l Choroidal melanoma begins in the layer of blood vessels — the choroid — beneath the retina. l Iris melanoma occurs in the front, colored part of the eye. l Ciliary melanoma occurs in the back part of the eye — in the ciliary body. OCULAR MELANOMA VS. CUTANEOUS MELANOMA Cutaneous (skin) melanoma and ocular melanoma are distinct conditions. While conjunctival melanoma is more similar biologically and genetically to cutaneous, uveal melanoma is very different both biologically and genetically. Both forms of melanoma begin in melanocytes but, beyond that, there are many differences and only a few similarities.

Sclera

PROGNOSIS AND METASTASIS The size of the tumor and the degree

of invasion are major factors in determining the prognosis — or

outcome — in melanoma. When the disease spreads, however, it spreads differently than cutaneous melanoma. Uveal melanoma tends to spread through the blood, while cutaneous and conjunctival melanoma tend to spread through the lymphatic system. Uveal melanoma metastasizes in up to 50% of all cases and when it spreads, it spreads to the liver 85–90% of the time. Cutaneous and conjunctival melanoma are less predictable and can spread to the lymph nodes, liver, lungs, brain, bone and soft tissue.

OCULAR MELANOMA

UVEAL Melanoma

CONJUNCTIVAL Melanoma

IRIS Melanoma

CILIARY BODY Melanoma

CHOROIDAL Melanoma

Ocular melanoma is most often detected by an optometrist or an ophthalmologist during a dilated eye exam. Often, OM is asymptomatic until the tumor grows large enough to create visual disturbances. Iris and conjunctival melanoma may sometimes be diagnosed by external — from the outside — examination. Detection of choroidal or ciliary melanoma requires a thorough dilated eye exam. Diagnosing Ocular Melanoma

Unlike cutaneous melanoma, a biopsy is not usually taken to diagnose OM. Rather, OM tends to be a clinical diagnosis — meaning it is often made based on signs and symptoms. In some cases, a fine-needle aspiration biopsy may be performed to help guide prognosis or determine eligibility for certain clinical trials.

After an OM diagnosis, your doctor may order imaging tests — such as an MRI or CT scan of the liver, and in some cases a PET/CT — to check for signs of cancer beyond the eye. Liver imaging is particularly important, as this is the most common site of metastasis.

What you need to know l Educate yourself and loved ones about your diagnosis. l Find a support system. Family, friends, strangers, in-person, online, phone support — choose one (or more) that is best for you. l Ocular melanoma survival statistics describe a group of similar patients… but they may have nothing to do with your individual chance of survival. l Every patient is different. There is no “blanket” treatment plan. l It is important to be an active participant in your treatment. Seek out an OM specialist. Be your own advocate.

Primary UM

What do I need to know?

DEFINITION

Primary uveal melanoma means that the tumor originated in the eye.

What you need to know l In the US, there are roughly 2,000–2,500 new cases of ocular melanoma diagnosed each year (equivalent to approximately five cases per million people). l A dilated eye exam is the best way to detect the disease and catch it in its early stages, often before symptoms appear.

l Uveal melanoma is the most common primary tumor of the eye in adults. l Although uveal melanoma is more common in Caucasian men with light-colored eyes, it does not discriminate by

age, race or gender. Everyone is at risk.

Tumor and Blood Testing in Uveal Melanoma

Once uveal melanoma (UM) is diagnosed, several items should be discussed with your treatment team that will help everyone learn more about your specific diagnosis. While treating the primary eye tumor remains the most important clinical issue, determining a patient’s risk for developing metastatic disease is also important.

Common Genetic Tests in UM

Healthcare providers can determine a patient’s risk for metastatic disease based upon the size and location of the tumor. From a biopsy , they can also test the genes in the tumor itself to help determine the risk of cancer recurrence and metastasis . The results of these tests can help your treatment team develop

an appropriate and individualized surveillance plan and, if necessary, a treatment plan.

Timing is critical because:

These genetic tests must be performed on a biopsy sample of the tumor. The biopsy sample must be taken before the tumor is treated with radiation therapy (including plaque brachytherapy or proton beam therapy).

Two different types of genetic testing may be performed: 1 CHROMOSOME ANALYSIS (KARYOTYPING)

Abnormalities in chromosomes 1, 3, 6 and 8 may indicate an increased risk of uveal melanoma metastasis. About half of UM tumors will show an alteration of chromosome 3 and metastatic UM occurs almost exclusively in patients with a loss of chromosome 3 (monosomy 3). These changes can be detected by several laboratory methods, including karyotyping and other specialized genetic tests.

(continued)

Common Genetic Tests in UM (continued)

2 GENE EXPRESSION PROFILE (GEP) TESTING

Based on a 15-GEP test and the expression of the PRAME gene, tumors are grouped into low-, medium- or high-risk for metastasis. l Class 1/PRAME Negative: low risk l Class 1/PRAME Positive: intermediate risk l Class 2: high risk

HLA Testing

Your doctor may recommend a blood test called HLA typing. HLA status refers to a person’s specific human leukocyte antigen (HLA) type, which helps the immune system recognize cells in the body. In ocular melanoma, HLA status is most often used to determine eligibility for certain immunotherapies and clinical trials, particularly T-cell-based treatments that are designed to work only in people with specific HLA types. HLA status does not affect the diagnosis of ocular melanoma and does not change over time. There is currently one FDA-approved systemic treatment for metastatic uveal melanoma (UM): tebentafusp (Kimmtrak), approved in 2022 for patients who test positive for HLA-A*02:01. Clinical trials exploring additional treatment options are ongoing and remain an important consideration for many patients. Therefore, knowing your mutation status may be helpful.

Speak with your doctor about how long it will take to find out the results and whether or not insurance will cover the cost of the test(s). Speaking with a certified genetic counselor may also be helpful.

Genetic Mutations in UM

often associated with older patient age and high risk for metastasis. The BAP1 mutation is strongly associated with a Class 2 gene expression profile (GEP). SF3B1 SF3B1 mutations (seen in ~10–20% of cases) are often linked with intermediate risk of metastasis. EIF1AX EIF1AX mutations (seen in ~10%) are generally linked with a better prognosis and lower risk of metastasis.

A variety of genetic mutations have been found in UM. The following mutations are thought to “drive” the disease: GNAQ and GNA11 The GNAQ and GNA11 mutations are the most common mutations in uveal melanoma, appearing in more than 80% of all cases. These mutations do not seem to be associated with patient outcomes or risk of metastasis. BAP1 The BAP1 mutation is found in about half of uveal melanoma cases. It is most

WHAT DOES THIS MEAN FOR TREATMENT? Mutation status does not guide standard treatment for primary UM, but in metastatic disease it may qualify certain patients for FDA-approved tebentafusp and help determine clinical trial eligibility or surveillance plans.

Speak with your ocular or medical oncologist about what your mutation status could mean and when your tumor should be tested.

Treatment of the Primary Tumor The goals of treating the primary tumor are to stop tumor growth, spare the eye, preserve vision and improve patient survival. Treatment most often involves radiation therapy (such as plaque brachytherapy or proton beam), sometimes combined with surgery if necessary. The exact approach depends on the size and location of the tumor, the patient’s overall health and other individual factors.

Something to consider

A melanoma diagnosis of any kind is never easy .

Patients who have been diagnosed with UM often feel a variety of emotions ranging from denial to anger. A UM diagnosis can be especially difficult. You may find yourself continuously wondering whether or not your melanoma has spread, or will spread, to other places in your body. Know that these feelings and emotions are normal. Meeting others, joining support groups and learning about this disease may help you during this difficult time.

Radiation

For most small and medium-sized tumors, radiation is the recommended treatment. Both plaque brachytherapy and proton beam radiotherapy have high rates of tumor control; the choice often depends on the tumor’s size, location and the availability of specialized treatment centers.

The different types of radiation therapy include: 1 PLAQUE BRACHYTHERAPY (RADIOTHERAPY)

A thin piece of metal, called a plaque, is sewn onto the outside wall of the eye. The plaque contains radioactive seeds (often iodine-125 or ruthenium-106), which give off radiation that aims to kill the tumor. 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 UM patients with small or medium-sized tumors. After this treatment, removal of the eye is not usually necessary, and many patients, depending on the tumor location, are able to retain some degree of visual function. 2 PROTON BEAM RADIOTHERAPY

Clips are surgically placed onto the eye at the tumor base and an external beam of radiation is aimed at the tumor, most often through the front of the eye. This approach is sometimes preferred for tumors near the optic nerve or in locations where plaque placement is challenging. Treatment is usually finished after 3–5 daily outpatient treatments.

In some cases, the recommended treatment for uveal 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. It may also be recommended if the tumor grows after radiation or causes complications such as eye pain or severe vision loss. Surgery

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. It is generally performed at specialized centers and may be combined with a radioactive plaque placed over the treated area to reduce the risk of tumor recurrence .

Types of surgery include: Enucleation Removal of the eye is sometimes

recommended in cases involving large tumors. This procedure is less common today due to the effectiveness of radiation for most small and medium 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.

OTHER POSSIBLE TREATMENTS

Neoadjuvant Treatment

Transpupillary thermotherapy The temperature of the tumor is slowly raised, killing cancer cells and shrinking the tumor. This treatment is now less commonly used as a primary treatment and is more often used for very small tumors or as an adjunct to radiation. Gamma Knife A focused, single dose of radiation is given to the tumor, sparing healthy tissue in and around the eye. Gamma Knife is rarely used for primary ocular melanoma and is more often considered for melanoma metastases to the brain. Intraocular injections Injections in the eye are used to administer medications to treat a variety of ocular conditions. These may include steroids for inflammation and/or anti-VEGF (anti-angiogenic) agents, which shrink abnormal blood vessels. Intraocular injections are sometimes used if radiation has caused changes to the retina or optic nerve (radiation retinopathy).

Neoadjuvant treatment is a treatment used to reduce primary tumor size to reduce radiation or avoid enucleation. There are ongoing neoadjuvant clinical trials — speak to your doctor to see if neoadjuvant clinical trials are right for you. Adjuvant Treatment Adjuvant treatment is defined by treatment used after the primary treatment to prevent recurrence or spread of disease (plaque radiation, enucleation). Currently in UM, adjuvant treatments are in the clinical trial stage. In clinical trials, adjuvant treatments are often recommended for patients with high risk of recurrence. Speak to your doctor about adjuvant clinical trials to see if there is one that is right for you.

Clinical Trials There is some important information you should know about clinical trials in ocular melanoma: l Trials help physicians determine which patients should receive new treatment approaches, including different drugs, targeted therapies, immunotherapies or treatment combinations, and in what order (sequence). l Trials may provide you access to therapies not yet approved by the FDA but that may be more effective. l Trials are usually (but not always) free to participate in and you may 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. l Joining a clinical trial is voluntary, and you can leave a trial at any time.

Speak with a Clinical Trial Navigator about finding a clinical trial from the Melanoma Research Foundation’s Clinical Trial Finder. How can I join a clinical trial? If you’re interested in joining a clinical trial, talk with your doctor. You can also…

Learn about clinical trials in melanoma.

Scan this QR code with your phone or click here to watch a video.

Questions to ask your doctor l D o I need to have my eye removed? l W hat are the dimensions of the tumor? What does this mean for treatment options? l W ill you do a biopsy of the tumor? Why or why not? l W ill you test my tumor for genetic mutations? l D o mutations affect the risk for metastasis? l H ow will you determine if the cancer has spread?

l W hat are my treatment options and what are the differences? l S hould I get a second opinion or explore another treatment center with more experience with ocular melanoma? l W hat are the side effects of each treatment? l W ill my vision be affected? l A m I eligible for any clinical trials now or in the future?

Metastatic UM

What do I need to know?

Treatment of Metastatic UM If UM has spread beyond the eye, it is considered metastatic . Approximately half of UM patients will develop metastatic disease, although each person’s individual risk is based on factors such as tumor genetics and other clinical characteristics. When UM metastasizes, it first spreads to the liver 85–90% of the time. It is important to note that the treatment of uveal melanoma can be very different from that of cutaneous melanoma. While some treatments are used both in cutaneous melanoma and UM, the diseases are very different. It is important for your treatment team to understand the differences. There are currently two FDA-approved treatments for metastatic uveal melanoma (UM): tebentafusp (Kimmtrak), approved in 2022 for patients who test positive for HLA-A*02:01, and melphalan/Hepatic Delivery System (HEPZATO KIT), a treatment for unresectable hepatic (liver) metastases. Clinical trials exploring additional treatment options are ongoing and remain an important consideration for many patients.

Visit melanoma.org to find an ocular melanoma treatment center and a specialist who can discuss all possible treatment options with you, including clinical trials.

MRF

Questions to ask your doctor

l H ow will this diagnosis and the treatment affect my day-to-day life? l If I choose no treatment for my metastatic disease, what is my life expectancy? l D o I need scans on a regular basis? If so, what type of scans do you recommend? l W hat should my follow-up plan be? l W ill I be able to continue my normal daily life? l S hould I seek a second opinion? l A m I eligible for tebentafusp (Kimmtrak)? l W hat supportive care is available to help manage symptoms and side effects?

l W here has the UM spread? l D o I need additional scans to determine the extent of the disease? l H ave you tested for genetic mutations? How will this affect treatment? l D o you recommend liver-directed therapy or systemic therapy? l C an different treatments be combined? l S hould I consider clinical trials? If so, which ones? l W hat are the side effects of each treatment? l D o I have to travel for the treatment or can I receive it close to home?

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.

LOCALIZED TREATMENTS FOR INDIVIDUAL TUMORS IN THE LIVER AND OTHER ORGANS

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 eye tumor diagnosis and repeated imaging studies show only one tumor. Suitability also depends on overall health and liver function. Since liver 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) 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. Ablation can be used in other areas of the body beyond the liver, such as the lungs, kidneys and soft tissues.

Histotripsy Histotripsy is a high frequency ultrasound. It is a noninvasive way to treat liver tumors. The safety and efficacy of this treatment is still being determined in UM. Radiation Targeted radiation can be used to treat liver disease. This may include stereotactic body radiation therapy (SBRT) for the liver or stereotactic radiosurgery techniques such as Gamma Knife or CyberKnife for brain or spine lesions. Radiation can be used to treat other areas of the body including the lungs, bones and brain, and can be used to treat isolated metastases or to relieve symptoms caused by a specific lesion.

These treatments may be used in conjunction with other treatments.

Fact: Liver embolization is a treatment that blocks or reduces blood flow to tumors, depriving them of oxygen and nutrients. Embolization treatments can be safely performed because the liver gets its blood supply from both the hepatic artery and a separate vein, called the portal vein.

Liver-Directed Therapies

REGIONAL LIVER-DIRECTED THERAPIES THAT AFFECT THE ENTIRE LIVER INCLUDE:

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 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 areas of the body. Immunoembolization is typically performed at specialized centers and may be available only through clinical trials. Chemoembolization (TACE) A chemotherapy drug is injected into the hepatic arteries. In addition, an agent is used to block off blood supply to tumors. The chemotherapy drugs that are used can vary. 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, SIR-Spheres or TheraSpheres. Tumors preferentially get their blood supply from the artery, and the healthy liver from the vein. That is why the blood supply can be cut off through the hepatic artery and not cause significant damage to the healthy liver.

HEPZATO KIT (melphalan/Hepatic Delivery System (HDS) ) 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. Approved by the FDA in August 2023, HEPZATO KIT is the first liver-directed therapy specifically approved for metastatic uveal melanoma. HEPZATO KIT involves closed circuit perfusion of the liver with high doses of a chemotherapy called melphalan. The procedure is a bit more complex than the other liver-directed therapies and requires close monitoring especially of blood counts.

These treatments may be used in conjunction with other treatments.

Systemic Treatments There is currently one FDA-approved systemic treatment for metastatic uveal melanoma (UM): tebentafusp (Kimmtrak). Approved in January 2022, tebentafusp is indicated for adult patients with unresectable or metastatic UM who are HLA-A*02:01 positive and remains the only approved systemic therapy as of 2026. Tebentafusp is a targeted immunotherapy that engages T cells to recognize and attack melanoma cells. Some clinicians may also recommend therapies approved for cutaneous melanoma, although their effectiveness in UM is more limited. For patients who are HLA-A*02:01 negative, and whenever appropriate, participation in clinical trials is strongly encouraged, as trials may offer access to investigational systemic therapies before approval.

Targeted Therapy A form of treatment in which drugs are developed to target specific mutations in the tumor 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. Common genetic mutations in melanoma (such as BRAF) are not often found in UM. The most common mutations in uveal melanoma are the GNAQ, GNA11 and BAP1 genes. Clinical trials are currently underway to develop drugs to target these mutations. Chemotherapy Overall, chemotherapy has not been shown to be effective for uveal melanoma and is rarely used. 16

Immunotherapy A type of systemic treatment given to activate a person’s immune system so that it will destroy melanoma cells within the body. Tebentafusp (Kimmtrak) is a type of immunotherapy specifically approved for metastatic UM. Additionally, there are several that are approved for cutaneous melanoma that have some activity in UM. Some of these treatments may include immune checkpoint inhibitors: ipilimumab (Yervoy), nivolumab (Opdivo), pembrolizumab (Keytruda), nivolumab/ relatlimab (Opdualag). Clinical trials are currently underway to better understand immunotherapies in UM.

Clinical Trials There is some important information you should know about clinical trials in uveal melanoma: l Trials help physicians determine which patients should receive new treatment approaches — including different drugs, targeted therapies, immunotherapies or treatment combinations — and in what sequence. l Trials may provide you access to therapies not yet approved by the FDA but that may be more effective. l Trials are usually (but not always) free to participate in and you may 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. l Joining a clinical trial is voluntary, and you can leave a trial at any time.

Visit melanoma.org to find an ocular melanoma treatment center and a specialist who can discuss all possible treatment options with you, including clinical trials. How can I join a clinical trial? If you’re interested in joining a clinical trial, talk with your doctor. You can also…

Learn about clinical trials in melanoma.

Scan this QR code with your phone or click here to watch a video.

Tips to remember l N ever hesitate to mention your side effects to your treatment team. Keeping your treatment team informed of all side effects as soon as they occur is of the utmost importance. l Experiencing few or no side effects does not mean the treatment isn’t working. l It’s impossible to know how you will react to any given treatment. l T here is no one-size-fits-

Managing Side Effects Unfortunately, side effects are a reality of every treatment option. Side effects vary by treatment and by individual. Some patients experience every possible side effect while others experience very few, and sometimes no side effects from their treatment. How side effects are managed is different too . Be sure to talk to your doctor about possible side effects before starting treatment so you know what to expect.

all treatment for ocular melanoma — everyone’s case is different.

Conjunctival Melanoma

What do I need to know?

What Is Conjunctival Melanoma? The conjunctiva is the clear tissue that covers the white part of the eye, as well as the inside of the eyelids. Conjunctival melanoma often appears as a raised tumor and may contain little or no pigment. Conjunctival melanoma most commonly occurs in the bulbar conjunctiva — the mucous membrane that covers the outer surface of the eyeball. Conjunctival melanoma is very rare, making up only 2% of all eye tumors and 0.25% of all melanomas. According to registry data from five countries, overall incidence is between 0.24 and 0.8 cases per million. However, incidence appears to be increasing, just as incidence rates are increasing in melanoma of the skin, suggesting a possible association between conjunctival melanoma and ultraviolet (UV) exposure. Diagnosing Conjunctival Melanoma A conjunctival melanoma diagnosis usually begins with a thorough examination of the eye and all conjunctival surfaces, including the inside of the eyelids. It is recommended that photographs be taken before a biopsy is taken. This allows the doctor to properly document the extent of the melanoma and may assist in planning for treatment and follow-up. A biopsy of the tumor and examination of the tissue under a microscope will rule out or diagnose conjunctival melanoma. If conjunctival melanoma is diagnosed, the extent of disease and treatment plan should be discussed with a trained ocular oncologist .

OCULAR MELANOMA

Reminder

UVEAL Melanoma

CONJUNCTIVAL Melanoma

IRIS Melanoma

CILIARY BODY Melanoma

CHOROIDAL Melanoma

Primary Management and Treatment Surgery is the most common type of treatment for conjunctival melanoma. Most cases of conjunctival melanoma only require removal of the tumor seen in the eye, however for more extensive or recurrent CM, a surgery called orbital exenteration may be required. Similar to cutaneous melanoma, sentinel lymph node mapping and biopsy may be recommended for staging. Excision is often followed by one or more types of adjuvant therapy in an effort to prevent the melanoma from spreading, or metastasizing. Types of adjuvant therapies could include cryotherapy (using freezing or near-freezing temperatures), topical chemotherapy or radiation therapy.

Long-term follow-up of patients with conjunctival melanoma is recommended in order to detect recurrences or metastatic disease. Patients should be monitored for recurrence by a trained ocular oncologist.

Living with Conjunctival Melanoma Please take a moment to review the final tab of this booklet — Living with Ocular Melanoma — for helpful information regarding follow-up care, coping with vision issues and living with monocular vision.

Prognosis and Metastases Conjunctival, uveal and cutaneous melanoma are distinct from one another. Therefore, they each require different treatment strategies. While there is no standardized treatment for uveal or conjunctival melanoma, significant advances have been made in our understanding of these rare melanoma subtypes. This has led to novel targeted therapy and immunotherapy approaches.

Because conjunctival melanoma usually spreads through the lymph system, sentinel lymph node biopsy (SLNB) may be considered and discussed with your treatment team. Risk factors for metastasis include tumor thickness, ulceration and mitotic rate (how many cells are dividing under the microscope). These are known risk and prognostic factors for cutaneous melanoma and were incorporated into staging criteria.

When conjunctival melanoma spreads, it often behaves more closely to cutaneous melanoma in that it usually spreads through the lymph nodes. From there, it has the ability to spread to the lungs, liver, soft tissues, bone and brain. When uveal melanoma spreads, it most often spreads to the liver and doesn’t usually spread through the lymph nodes.

Metastatic Treatment of Conjunctival Melanoma Although there are currently no FDA-approved systemic treatments specifically for conjunctival melanoma that has metastasized, some clinicians recommend treatment with therapies that have been FDA-approved for cutaneous melanoma. In addition, ongoing clinical trials may give patients access to systemic agents before they are approved.

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 conjunctival 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 conjunctival melanoma. However, it still may be recommended in some cases. For example, the BRAF mutation, which is found in about 50% of cutaneous melanomas, is also present in about 30% of conjunctival melanomas. Therefore, some clinicians may recommend targeted therapy for the treatment of conjunctival melanoma in which the BRAF mutation has been found.

Clinical Trials Clinical trials should be explored as a treatment option for anyone diagnosed with conjunctival melanoma. Here’s why: l Trials help physicians determine which patients should receive which drugs in which order (sequence). l Trials may provide access to therapies not yet approved by the FDA but that may be more effective. l Trials are usually (but not always) free to participate in and you may 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. l Joining a clinical trial is voluntary, and you can leave a trial at any time.

Questions to ask your doctor l W hat is my risk of the melanoma spreading? l H ow will you determine if the cancer has spread? l What is adjuvant therapy? l S hould I consider adjuvant therapy or explore clinical trials? l D o you recommend immunotherapy or targeted therapy? l D o I need scans on a regular basis? If so, what type of scans do you recommend? l H as my melanoma been tested for genetic mutations, such as the BRAF mutation? l What should my follow-up plan be? l W ill I be able to continue my normal, daily life? l Should I seek a second opinion?

Learn about clinical trials in melanoma.

Scan this QR code with your phone or click here to watch a video.

Living with OM

Surveillance & Survivorship

Follow-Up Care Follow-up care is different for every person. Factors to consider can be anything from the results of the tumor biopsy to the location of the tumor and even the medical provider. Follow-up care will consist of ongoing monitoring and surveillance, including: Scans (MRI/CT/PET scan/X-ray/ultrasound) are a way to monitor the spread of the disease. Scans are likely to be scheduled on a recurring basis, usually every three to twelve months, but it depends on your melanoma subtype and risk of recurrence. This is an important piece to discuss with your doctor.

Coping With Vision Issues

Depending on the treatment received, vision loss or monocular vision may become your new normal. Adjusting to monocular vision or vision loss will take time, so try not to get discouraged. Support services from social workers, therapists and loved ones may be helpful when adjusting to the loss of vision. It is normal to experience a period of grief due to these new changes and challenges. Always remember that there are people trained to assist you during this time. Resources for vision loss include adaptive services for reading, installing track lighting in areas that might need more light, services that perform home assessments and transportation plans if driving is affected. Employers may be able to help with lighting, reading assistance, screen shields or lens filters for more sensitive eyes. In addition, occupational therapists may be able to assist with visual perception issues.

It is important to keep up with routine visits with your ophthalmologist and medical oncologist.

Living With Monocular Vision Losing vision in one of your eyes takes some adjustment, both physically and emotionally. It takes time, so be gentle on yourself during this adjustment period. Some patients who require more extensive surgery, like orbital enucleation, may be offered a prosthetic eye. This is something to be discussed with your ocular oncologist and they may refer you to an ocularist.

Depth perception might be affected in some of the following ways:

l Judging distances while walking and using the stairs l Catching objects in the air

l Judging the heights of steps and the widths of entrances l Pouring liquids into containers

l Judging how close people are as they move in and out of your field of vision

Double vision may also become a challenge and is a known side effect of radiation therapy. Your brain will eventually start to assist you in compensating for the change in vision. Most day-to-day tasks and activities should fall back into place after an adjustment period. Be sure to work with your optometrist if you use corrective lenses to ensure your remaining eye is receiving the assistance it needs. Driving is still an option but be patient as you adjust to your new condition. Take the time to use your mirrors, become comfortable with the size of your vehicle and the blind spots that may occur while driving. Stopping, turning and changing lanes could be more challenging than they were before.

Questions to ask your doctor l W hat is my risk of the melanoma spreading? l H ow will you determine if the cancer has spread? l A re there any symptoms I should

be aware of that could signal that the cancer has spread?

l A re there changes to my diet or lifestyle that will make a difference in my cancer journey? l S hould I consider adjuvant therapy or explore clinical trials? l D o I need scans on a regular basis? If so, what type of scans do you recommend? l What should my follow-up plan be? l W ill I be able to continue my normal, daily life? l Should I seek a second opinion? l H ow often should I see my ophthalmologist and medical oncologist for follow-up?

Caregiver Support A guide to caring for a loved one with ocular melanoma Caring for someone with ocular melanoma is a team effort and caregivers are an essential part of that team, playing a vital role in treatment, daily life and emotional support. Your involvement can help your loved one navigate appointments, understand treatment options, manage side effects and feel supported through every stage of their journey.

Who Is on Your Loved One’s Care Team? Your loved one’s care team may include: l Ocular oncologist Specializes in diagnosing and treating eye cancers. l Medical oncologist Oversees systemic treatments and coordinates cancer care. l Radiation oncologist Plans and delivers radiation therapy if needed. l Ophthalmologist/optometrist Monitors eye health and vision changes. l Nurses and nurse navigators Provide education, coordinate care and answer questions. l Social workers and counselors Offer emotional support, resources and help with practical needs. l Primary care provider Manages overall health and coordinates with specialists. 24

Questions to ask your loved one’s doctor

l H ow often will follow-up appointments and scans be needed? l W ho should we contact if new symptoms appear? l W hat supportive care services are available for both patient and caregiver?

l W hat is the goal of this treatment? l W hat side effects should we expect and how can they be managed? l H ow will we know if the treatment is working? l A re there clinical trials available for this stage of disease?

Who Is on Your Team as a Caregiver? Being a caregiver can be emotionally and physically demanding. It’s important to know who you can lean on when you need help, such as:

Friends and family To provide emotional support, help with meals, transportation or household tasks. Your own healthcare providers To help you maintain your physical and mental health. Support groups Both in-person and online groups for caregivers can help you connect with others who

Caregiving Tips l Ask questions early and often so you can help your loved one make informed decisions. l W rite things down during appointments so nothing gets missed. l Share responsibilities when possible, to avoid burnout. l Take breaks and allow yourself time to rest and recharge. l Stay connected with your own support system — friends, family and professionals. l Look after your own health , including regular checkups, exercise and healthy eating.

understand what you’re going through. Community and faith-based resources

Local organizations, faith communities and nonprofits often provide respite care, counseling or financial assistance.

Important Note to Remember

You don’t have to do this alone. Being part of your loved one’s care team is important but so is taking care of yourself. Lean on your own support system, ask for help when you need it and remember that your well-being matters too. The Melanoma Research Foundation (MRF) offers programs and resources for both patients and caregivers, including virtual ocular melanoma support groups, the MRF Patient Forum and free educational materials, such as quarterly newsletters and educational webinars, at melanoma.org.

For more information on our CURE OM initiative email cureom@melanoma.org . For additional emotional or practical support, organizations like CancerCare ( cancercare.org ) and the Cancer Support Community ( cancersupportcommunity.org ) provide counseling, online groups and caregiver-focused programs.

melanoma.org melanoma.org/cure-om cureom@melanoma.org cancercare.org cancersupportcommunity.org forum.melanoma.org

MRF

Glossary and Resources

Common Terms

Ciliary melanoma A type of uveal melanoma that occurs in the ciliary body, a structure in the eye that helps control the lens and produces fluid inside the eye. Computed tomography (CT) scan A series of detailed images of the inside of the body taken from different angles. In ocular melanoma, CT is most often used to evaluate the liver or other organs for possible spread. Extension The melanoma has spread outside the eye by extending through the wall of the eye.

HLA status HLA status refers to a person’s specific human leukocyte antigen (HLA) type, which helps the immune system recognize cells in the body. In ocular melanoma, HLA status is most often used to determine eligibility for certain immunotherapies and clinical trials, particularly T-cell-based treatments that are designed to work only in people with specific HLA types. HLA status does not affect the diagnosis of ocular melanoma and does not change over time. Iris melanoma A type of uveal melanoma that occurs in the iris, the colored part of the front of the eye. Malignant A term often used in melanoma, meaning invasive, cancerous or capable of metastasis.

Adjuvant therapy Used after the primary treatment, such as surgery or radiation, to decrease the chance of the ocular melanoma returning or spreading. Biopsy The removal of cells or tissues for examination under a microscope. Bone scan An imaging test that uses a small amount of radioactive material to check for cancer in the bones. Rarely used in ocular melanoma unless symptoms or other findings suggest bone involvement. Choroidal melanoma A type of uveal melanoma that begins in the choroid, the layer of blood vessels beneath the retina that supplies oxygen and nutrients to the eye.

Fine-needle aspiration biopsy (FNAB)

A procedure that uses a very thin needle to remove a small sample of tumor cells from the eye. The sample is tested to help determine the risk of the melanoma spreading.

Surgical oncologist A doctor who performs biopsies and other surgical procedures in cancer patients. Systemic treatment Treatments that travel through the bloodstream, affecting cells throughout the body. Examples include immunotherapy and chemotherapy. Ultrasound A procedure that uses high-energy sound waves to look at tissues and organs inside the body. May also be used to evaluate a tumor. X-ray An imaging test that uses radiation to produce pictures of structures inside the body. In ocular melanoma, chest X-rays are used far less often now; liver imaging with MRI or CT is preferred for metastasis surveillance.

Pathologist A specialist in pathology who looks at tumor biopsies under a microscope to determine diagnoses. PET scan An imaging test that detects areas of increased metabolic activity in the body. Not routinely used for uveal melanoma staging, but may be ordered if there is concern about spread beyond the liver or to other organs. Commonly used in conjunctival melanoma. Plaque brachytherapy A type of radiation treatment for ocular melanoma. A small metal “plaque” containing radioactive seeds is placed on the outside wall of the eye, directly over the tumor, to deliver targeted radiation.

Medical oncologist A doctor who specializes in

diagnosing and treating cancer. A medical oncologist often is the main healthcare provider for someone who has cancer. They may also give supportive care and coordinate treatment given by other specialists. Metastatic The spread of the melanoma from the original site to other places in the body. Magnetic resonance imaging (MRI) An imaging test that uses radio waves and magnets to create detailed pictures of the body. In uveal melanoma, MRI is most often used for liver surveillance to monitor for possible metastasis. Brain MRI may also be used to evaluate for spread to the brain when clinically indicated. Neoadjuvant therapy A treatment or therapy used to shrink the primary tumor prior to radiation or to help avoid enucleation. Ocular oncologist An eye cancer physician who provides comprehensive care for patients with eye tumors. Ocular oncologists are trained in ophthalmology and have completed specialized training in eye cancers. Ocular oncologists diagnose, treat and research a variety of malignancies that include the eye, eyelid and surrounding tissue. Ophthalmologist A medical or osteopathic doctor who specializes in eye and vision care. An ophthalmologist diagnoses and treats eye diseases and is licensed to practice medicine and surgery. Many ophthalmologists are also involved in scientific research in eye diseases and disorders. Optometrist A health professional who provides primary vision care, sight testing and management of vision changes. An optometrist can perform eye exams, vision tests and detect certain eye abnormalities. An optometrist does not perform surgery.

Recurrence The return of the melanoma.

Sentinel lymph node biopsy (SLNB) A surgical procedure to find the first lymph node(s) (sentinel nodes) where cancer cells are likely to spread from a primary tumor.

Patient Resources CURE OM

Cure OM Support Groups

We invite you to participate in our upcoming CURE OM online support groups, which take place monthly: Space for Connection (Monthly Meeting) First Wednesday of each month, 1:00–2:00 PM ET 10:00–11:00 AM PT. This group is offered in partnership with the Sidney Kimmel Comprehensive Cancer Center at Jefferson and is facilitated by Molly Vocino, MSW, LCSW, a licensed clinical oncology social worker. The group’s discussion centers on the metastatic ocular melanoma experience. If you’ve attended previously, we warmly encourage you to continue joining. For more information and to register, please email cureom@melanoma.org . Space for Connection (Monthly Evening Meeting) Fourth Thursday of each month, 7:00–8:00 PM ET 4:00–5:00 PM PT This evening group is facilitated by the MRF’s Education Program Manager, Caroline Glavin, MSW. The group is open to all ocular melanoma patients and caregivers. For more information and to register, please email cureom@melanoma.org . These groups are designed to foster emotional support, shared understanding and connection. Whether you’re newly navigating an ocular melanoma diagnosis or have been living with it for years, this is a space to reflect, express feelings, explore coping strategies and find comfort in community.

CURE OM (Community United for Research and Education of Ocular Melanoma) is an initiative by the Melanoma Research Foundation (MRF) aimed at increasing awareness, education and research funding for ocular melanoma. Founded in 2011, CURE OM focuses on improving the lives of those affected by this disease by providing resources, support and funding for research. The initiative includes annual symposiums, support groups and educational materials to help patients and caregivers navigate their journey with ocular melanoma.

melanoma.org/cure-om

Eyes on a Cure: Ocular Melanoma Patient & Caregiver Symposium Eyes on a Cure is the Melanoma Research Foundation’s annual meeting for the ocular melanoma community, bringing together patients, caregivers and leading experts for a weekend of education, connection and support. The program features research updates, treatment information and opportunities to connect with others navigating a similar experience.

melanoma.org/eyes on a cure symposium You may also scan this QR code with your phone to connect with the Eyes on a Cure symposium.

MRF Patient Forum Provides a space for patients, caregivers, family and friends to discuss the melanoma journey and find friends and resources to make that journey more bearable.

forum.melanoma.org

Ask a Nurse The MRF’s nurse provides free, personalized answers to your melanoma questions.

askanurse@melanoma.org

Melanoma Treatment Center Finder An interactive map listing melanoma centers of excellence and treatment centers that have experience treating melanoma.

melanoma.org/treatment-center-finder

Melanoma Research Foundation Clinical Trial Finder

connect.careboxhealth.com

You may also scan this QR code with your phone to connect with the clinical trial finder.

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