Ocular melanoma, also known as intraocular melanoma, is a rare but aggressive eye cancer that originates from melanocytes, the pigment-producing cells of the eye. It is the most common primary eye cancer in adults, with an annual incidence of approximately 5–6 cases per million individuals. The condition is more prevalent in fair-skinned individuals and is most commonly reported in Europe and North America.¹ The most common subtype of ocular melanoma is Uveal melanoma (constitutes around 80% of cases). Like skin melanomas, ocular melanomas are malignant (spread to other body parts). Therefore, timely treatment is important. The mainstay of the treatment is surgery. However, radiotherapy may also be employed.

Types Of Ocular Melanoma

Based on the anatomy, there are different types of ocular melanoma:

Uveal Melanoma:

This type of melanoma accounts for about 85% of the cases and is the most common type of ocular melanoma. The uvea is composed of different structures, thus, further classification of melanoma is as follows:

Iris melanoma is a cancer of the colored part of the eye i.e., the iris.
Ciliary body melanoma is a cancer of the ring of muscle fibers that control the shape of the lens i.e., the ciliary body which is present behind the iris.
Choroid melanoma is a cancer of a thin sheet of tissue that contains blood vessels i.e., choroid. Choroidal melanoma is the most common.²

Since uveal melanoma is significantly more common than conjunctival melanoma, the terms ocular melanoma and uveal melanoma are often used interchangeably.

Conjunctival Melanoma:

Very rarely, cancer can affect melanocyte cells of the conjunctival membrane. Less than 10% of the malignant eye tumor cases present with conjunctival melanoma.

Ocular Melanoma Staging

Doctors usually use the TNM staging to stage ocular melanomas. In a cancer report, you will get the TNM stage mentioned. This determines the extent of treatment required.

Tumor:

Tx

No primary tumor assessed

T0

No evidence of a primary ocular tumor

T1

Tumor base ≤ 9mm and thickness is ≤ 6mm
The tumor base is 9.1-12mm with a thickness of ≤ 3mm
Based on slight differences, T1 is further subclassified into:

T1a: T1 without ciliary body involvement and extraocular extension
T1b: T1+ciliary body involvement
T1c: T1+extraocular extension (≤5mm in diameter) but without ciliary involvement
T1d: T1+extraocular extension (≤5mm in diameter) with ciliary body involvement

T2

The tumor base is ≤ 9mm and the thickness is 6.1-9mm
The base of the tumor is 9.1-12mm and the thickness is 3.1-9mm
The tumor base is 12.1-15mm and the thickness is ≤ 6mm
The base of the tumor is 15.1-18mm and the thickness is ≤ 3mm

T2a: T2 + without ciliary body involvement and extraocular extension
T2b: T2 + with ciliary body involvement
T2c: T2 + extraocular extension (≤5mm in diameter) but without ciliary involvement
T2d: T 2+ extraocular extension (≤5mm in diameter) with ciliary body involvement

T3

The tumor base is 3.1-9mm and the thickness is 9.1-12mm
The base of the tumor is 9.1-12mm and the thickness is 9.1-15mm
The tumor base is 12.1-15mm and the thickness is 6.1-15mm
The base of the tumor is 15.1-18mm and the thickness is 3.1-12mm

T3a: T3+without ciliary body involvement and extraocular extension
T3b: T3+with ciliary body involvement
T3c: T3+extraocular extension (≤5mm in diameter) but without ciliary involvement
T3d: T3+extraocular extension (≤5mm in diameter) with ciliary body involvement

T4

The tumor base is 12.1-15mm and the thickness is >15mm
The base of the tumor is 15.1-18mm and the thickness is >12mm
The tumor base is >18mm with any thickness

T4a: T4+without ciliary body involvement and extraocular extension
T4b: T4+with ciliary body involvement
T4c: T4+extraocular extension (≤5mm in diameter) but without ciliary involvement
T4d: T3+extraocular extension (≤5mm in diameter) with ciliary body involvement
T4e: Any tumor size with extraocular extension >5mm

Node:

Nx: Regional lymph nodes can not be assessed
N0: No regional lymph node is involved
N1: Regional lymph nodes are involved or there are discrete tumor deposits in the orbit

N1a: Metastasis is on one or more regional lymph nodes.
N1b: There is no involvement of regional lymph nodes but there is a discrete tumor presence in the orbit (which is not contagious to the eye)

Metastasis:

M0: No distant metastasis is present
M1: Distant metastasis

M1a: The largest diameter of the largest metastasis is ≤3 cm
M1b: The maximum diameter of the largest metastasis is 3.1-8.0cm
M1c: The largest diameter of the largest metastasis is ≥8.1cm

Ocular Melanoma Symptoms

In several cases, there are no symptoms or the symptoms are difficult to spot. When present, you can notice the following symptoms:

Blurred/Distorted Vision & Diplopia:

Numerous patients with melanoma report blurred vision. In a clinical case, the patient reported only blurred vision. Investigations by clinicians revealed primary ciliary melanoma with invasion of the iris. The study concluded that even very small ciliary melanomas can do fast local invasion and lead to blurred vision.³ In another report of 25 diffuse iris melanoma cases, 52% of participants had blurred vision.⁴ Several patients also complain of having dual vision (diplopia).

Eye Pain:

Cancers of the ciliary body and choroid can present with pain. Eye pain is not very common; as per a study, only 2% of melanoma patients experienced eye pain.⁵

However, another study concluded the percentage of eye pain to be 11.5% in patients with choroidal cancer.⁶

Dark Spot & Floaters:

One of the common symptoms of eye cancer is dark spots in vision. Patients report floating shadowy specks with dark colors. These may be shaped like threads, webs, or just like a small dot.⁷

Reduced Field Of Vision:

The growth of ocular melanoma in the middle layers of the eye can interfere with or obstruct peripheral vision. Occasionally, iris melanomas lead to secondary glaucoma due to the seeding of melanoma in the trabecular meshwork. This chronic glaucoma can cause peripheral visual field loss.⁸

Flashing Lights (Photopsia):

According to a survey conducted by the Ocular Melanoma Foundation, a lot of patients see flashing lights as a symptom of eye melanoma. The phenomenon is usually an initial presentation and continues to increase with time.⁹

Changes in Pupils:

Uveal melanoma is linked to changes in the color and the shape of pupils. Cancer of the melanocytes can bring about significant changes in eye color (due to abrupt production of pigments).

Eye Displacement (Within The Socket)

A few eye cancer patients also report displacement of the eye. Exophthalmos is the protrusion of the eyes from the orbit. It is an unusual symptom of uveal melanoma.¹⁰

Retinal Detachment

There have been reports of coexistence of retinal detachment and choroidal melanoma in patients which raises suspicion of melanoma being the cause of the detachment.¹¹ Retinal detachment is more significant in cases of large choroidal tumors.¹²

Complete Loss Of Vision

Complete vision loss only occurs in advanced cases of eye melanoma. This occurs only when the tumors grow significantly large and damage the critical structures of the eye (macula, etc,). This can potentially lead to significant visual impairment and even vision loss. A complete loss of vision is mostly seen in long-standing melanomas.

Ocular Melanoma Causes

The exact cause of eye melanoma is unknown, however, experts believe abnormalities in the DNA of melanocytes lead to abnormal (uncontrolled) multiplication and formation of tumors.

Risk Factors:

Certain groups of individuals are at a greater risk of developing ocular melanoma:

Fair/Pale Skin

The skin houses a significant number of melanocytes. Fair-skinned people are more likely to fall prey to cutaneous melanomas. Astonishingly, multiple studies have found a link between fair or pale skin and ocular melanoma too.¹³

Family History

Though rarely, a family history of melanomas (cutaneous or ocular) serves as a novel risk factor for uveal melanomas.¹⁴

Light-Colored Eyes

Individuals with light-colored eyes are prone to developing eye melanoma. Grey, green, and blue eyes fall in the highest risk group while brown eyes have a moderate risk of intraocular melanoma. Studies suggest there are greater chances of ocular melanoma in people with a fair complexion and blue or green eyes.¹⁵

Sunlight/UV Light Exposure

Some believe that exposure to sunlight (artificial sunlight tanning beds) can cause eye melanoma. There is a weak link between ultraviolet radiation (UVR) exposure and uveal melanoma.¹⁶ As children/infants have thinner eye layers, experts suspect that the high rate of pediatric uveal melanoma is attributed to UV exposure.¹⁷

Inherited Skin Conditions

Individuals suffering from inherited dermal disorders like dysplastic nevus syndrome and moles are more likely to develop uveal melanoma. A nevus is a dark-colored growth in the eye commonly known as the “eye freckle”. People with melanocytic nevi are at a greater risk of developing ocular melanoma.

Old Age

Senile individuals are more prone to developing uveal

Ocular Melanoma Diagnosis

As most ocular melanomas are rare and asymptomatic, diagnosis is usually made during a routine eye checkup. Ophthalmologists opt for different types of tests and scans to diagnose ocular melanoma.

Eye Examination:

For an eye exam, your doctor will dilate your eye via special eye drops. This way the doctor can look into the structures of the eye.

Ultrasound:

An ultrasound aids in measuring the size of the ocular structures. Any abnormality in the size can correlate with tumorous growth. Your doctor will gently place a small probe on the eye surface after numbing it with anesthetic drops. Ultrasounds provide details about the size of the melanoma.

Fluorescein Angiography:

It is a study of the ocular vessels done via a special camera. Doctors inject a dye into your arm which is taken up by the blood vessels. The camera then takes pictures of the eye indicating any vascular blockage or leakage.
Fundus autofluorescence: Carried out with a special camera, this ophthalmic test highlights the areas of damage by turning them into small points of light in the photograph.

Optical Coherence Tomography (OCT):

An OCT is an imaging test that provides an extensively detailed picture of the inside of the eye (including cancerous masses).

After a comprehensive diagnosis of the condition, doctors perform the staging of the cancer to determine the size and extent of the cancer’s spread to other parts of the body.

Ocular Melanoma Treatment

Like with most cancers, the ideal treatment plan for ocular melanoma depends on multiple factors. The aspects that determine the type of treatment include:

Size (and thickness)
Location
Spread of cancer (metastasis)
Age
Overall health (presence of co-morbidities, immune health, etc.)

Watchful Waiting:

If your tumor is asymptomatic and has a thickness of less than 2.5mm, you may not need immediate treatment. In such cases, eye doctors advise you to monitor the development of symptoms carefully. This period is called “watchful waiting”.

Radiation Therapy:

Powerful ionizing radiations are frequently used to kill the cancerous cells. Several patients prefer non-invasive procedures like radiation therapy. Based on the location and size of the carcinoma, your healthcare provider can opt for either internal or external radiation therapy:

Internal Radiation Therapy

In internal radiation therapy, the doctor implants a radiation source (a radioactive seed) inside the eye i.e., near the tumor. The most common type of radiation therapy is Plaque radiation therapy in which the doctor places a metal disk (plaque) directly on the wall of the eye. Made out of gold, the plaque resembles a bottle cap in shape. Professionals use temporary stitches to hold the seed in place for 4-5 days.
Research indicates that plaque brachytherapy is an effective treatment for uveal (especially choroidal) melanoma. It provides excellent control of localized/focal tumors and serves as a feasible alternative to enucleation (for medium-sized tumors). The treatment modality has good survival rates.¹⁸

External Radiation Therapy

As the name indicates, the source delivers radiation from outside the body. Therapies like Proton beam radiation effectively target the eye tumor while being minimally damaging to the neighboring structures (eye and brain).

Surgery:

Surgeons can perform different types of surgeries for uveal melanoma:

External Resection

This involves removing the tumor and some of the neighboring tissues (healthy). It is indicated for small-sized tumors.

Enucleation

Doctors opt for enucleation i.e., complete removal of the eye in cases of large, painful tumors or tumors involving the optic nerve. After the removal of the eye, ophthalmologists replace the space with an artificial (prosthetic) eye.

Endoresection

In localized choroidal melanomas, doctors scoop out the part/area of the choroid affected by the tumor via a small incision. In this technique, the tumor is removed from within the eye, and the treatment type is effective for posteriorly localized uveal melanomas.¹⁹

Thermal Therapy:

Transpupillary thermal therapy utilizes lasers to generate heat. This directed heat aims to destroy eye tumors. Doctors deliver electromagnetic radiation via an infrared laser in a dilated eye.

Ocular Melanoma Prognosis

The prognosis of eye uveal melanoma relies heavily on the stage of cancer at the time of diagnosis/treatment. Localized tumors have a good prognosis and survival rates. However, the 5-year survival rate drops significantly in cases with metastasis (cancer spread).

Conclusion

Ocular melanoma is a rare eye cancer affecting pigment-producing melanocytes, most commonly in the uvea (uveal melanoma). It is often asymptomatic, but symptoms like eye pain, blurred vision, dark spots, and vision loss may occur. Genetic mutations in melanocytes contribute to its development, with risk factors including fair skin, light-colored eyes, family history, age, male sex, and UV exposure. Diagnosis relies on imaging techniques like OCT, ultrasound, and angiography. Treatment options include radiation therapy for small tumors and surgical removal or enucleation for larger ones. Prognosis is good for non-metastatic cases but poor if metastasis occurs, particularly to the liver.

Refrences

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Ocular Melanoma: A Rare but Serious Eye Cancer

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