Usually found in the Caucasian population, Uveal Melanoma is a fairly rare disease. Having said that, it is actually the most common primary intraocular tumor found in adults with an average of 5.1 cases per million per year with this number being stable for a few years now.
What is Uveal Melanoma?
Melanoma forms when melanocytes, the cells that make the pigment (melanin) in skin, hair, and eyes, undergo a transformation and become malignant. If melanoma arises in the uvea or uveal tract, one of the three layers of the wall of the eye, it is called uveal melanoma but can also be referred to as intraocular melanoma.
The uvea is made up of the iris, the colored area at the front of the eye; the ciliary body, a ring of muscle tissue found behind the iris that changes the size of the pupil; and the choroid, a layer of blood vessels that bring oxygen and nutrients to the eye.
Tumours that arise in different parts of the uvea have different behaviors and outlooks. Melanomas in the iris are usually small, grow slowly, and rarely spread to other parts of the body. Melanomas in the ciliary body are often larger and more likely to spread to other parts of the body than those in the iris. The choroid is the most common site of uveal melanomas, and these tumors are also often larger and more likely to spread to other parts of the body than those in the iris.
Although uveal melanoma is rare, it is the most common form of eye cancer in adults. It is largely older adults who have an average of 70 years old who are diagnosed with this type of cancer. Although not always the case, people who are Caucasian, fair-skinned, and have light-coloured eyes are at a higher risk of getting this type of cancer. Tumors are located either in iris (4%), ciliary body (6%), or choroid (90%). Other susceptability factors for uveal melanoma include inability to tan, ocular or oculodermal melanocytosis, cutaneous or iris or choroidal nevus, use of sunbeds and BRCA1-associated protein 1 mutation.
Types of Intraocular / Uveal Cancer
This is the most common type of primary intraocular cancer in adults. It begins when cells called melanocytes grow uncontrollably. Intraocular melanoma is also called uveal melanoma.
Other, less common types of an intraocular tumor include:
This type of lymphoma begins in the eyeball. This condition is rare and can be difficult for doctors to diagnose. Many doctors consider intraocular lymphoma to be a type of central nervous system lymphoma. Most intraocular lymphomas are non-Hodgkin lymphoma.
Retinoblastoma is a rare form of childhood eye cancer.
Hemangioma is a benign tumor of the choroid and retina that starts in the blood vessels.
Other, rare cancers of the eye include:
Conjunctival melanoma is a tumor of the conjunctiva; a membrane that lines the eyelid and eyeball. If left untreated, it can go on to spread to the lymph nodes. Even with treatment, this type of cancer can be recurring with dark spots appearing on the eye’s surface. Doctors often perform a biopsy on a spot that appears to be conjunctival melanoma. A biopsy is the removal of a sample of the tissue for examination under a microscope.
Eyelid carcinoma (basal or squamous cell) is a variation of skin cancer. This tumor may be surgically removed and is usually not dangerous if it is treated early.
Lacrimal gland tumor is a benign or malignant tumor of the glands that produce tears.
Currently, the most widely used first-line treatment options for this malignancy are resection, radiation therapy, and enucleation.
The most common intraocular cancer in adults is uveal metastases, which is cancer that has spread to the uvea from another place in the body. This is called secondary cancer. This guide is about primary intraocular cancer, meaning that the tumor started in the eye, not somewhere else in the body.
Causes of Intraocular / Uveal cancer
The following factors can raise a person’s risk of developing eye cancer:
- Age - People over the age of 50 are most likely to be diagnosed with primary intraocular melanoma. The average age of diagnosis is 55.
- Race - Primary intraocular melanoma is diagnosed more in white people and is less commonly diagnosed in black people.
- Gender - Intraocular melanoma affects about equal numbers of men and women.
- Individual history - People with the following medical conditions have a higher risk of developing primary intraocular melanoma - Ocular melanocytosis or Dysplastic Nevus Syndrome.
- Family history - Intraocular melanoma can often run in families, although it is rare. Usually, it is due to a mutation or change in a gene called BAP1, which is mostly linked with metastatic uveal eye cancer. This gene change is also seen in other cancer types, such as kidney cancer and mesothelioma.
- Other Factors - Research has suggested that sunlight or certain chemicals may be a risk factor for intraocular cancer.
Main Symptoms of Intraocular / Uveal Cancer
As with many skin cancers, the main symptom for intraocular / uveal melanoma is small spots or growth seen within the eye. A small growth may not cause any symptoms however as it grows larger, your symptoms may include:
- Blurry vision or sudden loss of vision
- Soreness in an eye, or bulging of the eye (uncommon)
- Flashes or “floaters” in your vision
- Dark spot on your iris, the colored ring at the front of your eye
- Change in the shape of your pupil, the black circle in the center of your eye
- A change in the way your eye moves or looks
Many of these symptoms can be confused with other health problems such as migraines for example however they could also be an indication of a starting melanoma in the eye. If you are in doubt, it is best to seek medical advice so you can be diagnosed and treated before it develops further.
Diagnosis & Detection of Intraocular / Uveal Cancer
An ophthalmologist (a specially trained eye doctor) will usually be the person to make a diagnosis for uveal cancer. The specialist will go through your health history, symptoms, risk factors, and family history of disease, followed by an eye exam. During the examination, a special scope with a light (ophthalmoscope) will be used to look at the inside of your eye.
Your ophthalmologist may be able to recognise intraocular melanoma by just looking at your eye but in some cases further examinations may be required such as:
- Ultrasound. This test uses high-frequency sound waves to see the inside of the eye. It’s often used because melanomas in your eye have a certain look when being detected on an ultrasound. It can also show where the tumor is and how big it is; determining the stage and grade.
- MRI. MRIs use radio waves and strong magnets to make an image. This test is helpful in learning the size of the tumor. It’s often used to check if the cancer has spread beyond the eye.
- Angiography. During this procedure, your healthcare provider injects dye into a blood vessel in your arm. Then he or she takes pictures of your eye as the dye moves through it. The dye helps to show any changes more clearly.
- Biopsy. Your healthcare provider may need to take a small tissue sample from the growth. This is rarely done because other tests work well to diagnose intraocular melanoma.
After a diagnosis of intraocular melanoma, you will probably also need further tests to give you more information on the cancer which will also be helpful in figuring out the staging. The stage is how much and how far the cancer has spread (metastasized) in your body. It is also important to get a description of how thick and wide the tumor is. These are important things to know when deciding how to treat the cancer.
Stages of Uveal Cancer
Staging is a way of describing where the cancer is located, if or where it has spread, and whether it is affecting other parts of the body. Doctors use diagnostic tests to find out the cancer's stage, so staging may not be complete until all the tests are finished.
Knowing the stage helps your doctor decide what type of treatment will be most suitable and can help determine a patient’s prognosis (the chance of recovery). There are different stage descriptions for different types of cancer.
TNM staging system
One of the main tools to describe the stage of cancer is the TNM system. Medical oncologists use this system, but your ophthalmologist may not. Doctors use the results from diagnostic tests and scans to answer these questions:
Tumor (T): How large is the primary tumor? Where is it located?
Node (N): Has the tumor spread to the lymph nodes? If so, where and how many?
Metastasis (M): Has the cancer metastasized to other parts of the body? If so, where and how much?
The results are combined to determine the stage of cancer for each person. There are four stages: stages I through IV (one through four). The stage provides a common way of describing the cancer, so doctors can work together to plan the best treatments.
In eye cancer, T for an iris melanoma is described differently than T for choroidal and ciliary body melanomas. N and M are described the same for iris, choroidal, and ciliary body melanomas.
Some ophthalmologists may not use the TNM system to stage an intraocular tumor. However, they will still consider the size of the tumor and how it is affecting a person’s vision when deciding on a treatment plan.
Here are more details on each part of the TNM system for eye cancer:
Using the TNM system, the “T” plus a letter and/or number (0 to 4) is used to describe the size and location of the tumor. Some stages are also divided into smaller groups that help describe the tumor in even more detail. The following classifications are the same for any type of intraocular melanoma:
TX: The primary tumor cannot be evaluated.
T0: There is no tumor in the eye.
An iris tumor is classified as T1, T2, T3, or T4. Some stages are divided into smaller groups that help describe the tumor in even more detail.
T1: The tumor is limited to the iris.
T1a: The tumor is in one quadrant (one-fourth) or less of the iris.
T1b: The tumor is in more than one quadrant of the iris.
T1c: The tumor is only in the iris, but there is melanomalytic glaucoma. This means that a buildup of certain cells in the eye blocks the flow of fluid in the eye, causing pressure.
T2: The tumor has joined or grown into the ciliary body and/or choroid.
T2a: The tumor has joined or grown into the ciliary body and/or choroid with melanomalytic glaucoma.
T3: The tumor has joined or grown into the ciliary body and/or choroid and extends to the sclera (outer wall of the eyeball).
T3a: The tumor has joined or grown into the ciliary body and/or choroid and extends to the sclera in association with melanomalytic glaucoma.
T4: The tumor has spread to the outside of the eyeball, the optic nerve, or to the eye socket. This is called extraocular extension.
T4a: The tumor has spread less than 5 millimeters (mm) outside of the eye.
T4b: The tumor has spread more than 5 mm outside of the eye.
Ciliary body and choroid melanoma
A tumor in the ciliary body and choroid is also classified as T1, T2, T3, or T4 based on the size of the tumor, which is measured in optic disc diameters or mm. The tumor is measured for both width and height (also called thickness). A tumor is given a classification according to a table, based on its width and height.
Your doctors may use and refer to this classification, called a category, even more than the stage. This is because the size and thickness of the tumor (the T) is most important for finding out a patient’s prognosis.
Also known as urothelial cell bladder cancer, this particular type of bladder cancer has spread into the muscle layer of the bladder and is more likely to spread. This type requires more intensive treatment than non-muscle invasive bladder cancer in order to prevent or limit the possibility of it spreading to other areas of the body.
Coping with an Intraocular / Uveal Cancer Diagnosis
Like most types of cancer, a diagnosis of eye cancer can leave you and your loved ones feeling uncertain, anxious and overwhelmed. There are important treatment decisions to make, emotional concerns to manage, and maybe also insurance and financial paperwork to organise, among other practical concerns.
It is helpful to keep in mind that there are many sources of information and support for people coping with eye cancer. By learning about your diagnosis and the latest treatment options, communicating with your primary doctors & oncologists, and, if possible, finding a support network; you will be better able to manage your eye cancer and experience a better quality of life.
Second Opinions for Intraocular / Uveal Cancer Diagnosis
If you've been told you have intraocular / uveal melanoma, it's important to ask as many questions as you can. Educate yourself by reading up information about the latest developments on treatment, as well as treatment options that may be currently being researched. It is often recommended to gain as much information as possible from your doctor and other sources so you can make an informed decision. You may want to consider a second opinion that can help provide you with this additional information.
Oncolomed can help facilitate second opinions with eye cancer specialists from leading hospitals globally. Find out more about obtaining a second opinion for uveal cancer.
Treatment Options for Uveal Melanoma
The most suitable treatment for intraocular/uveal cancer will depend on your test results, where the tumour is and the stage of the cancer. Your ability to see in the unaffected eye, age, overall health, and personal choice will also be considered. The main aim of treatment for intraocular cancer will be to save the eye; maintaining your ability to see, as well as keeping the cancer from spreading. Ask your specialist about your treatment choices, the goals of treatment, and what the risks and side effects may be.
Treating this cancer can potentially cause eye damage. For this reason, you may be advised not to start treatment right away if you don’t have symptoms and the tumor isn’t growing. This is called watchful waiting. You should see your specialist regularly who will take pictures of the tumor to track it’s growth. If the tumor starts to grow, it is surely time to start treatment.
As there are several treatment options for uveal cancer, it’s best to talk to your ophthalmologist about the most suitable treatment for you.
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