Explore symptoms, treatments, and more about eye cancer on Meri Sehat. Empowering you with information on ocular health

Eye Cancer

A. JABBAR DENTAL CLINIC KARACHI

Sadia Khan

Overview

Eye cancer, also known as ocular cancer, refers to the abnormal growth of cells in the eye that can lead to the formation of tumors. 
&nbsp;

According to recent research, the incidence and mortality rates of eye cancer vary across different regions. The American Cancer Society states that the death rate for eye cancer has increased worldwide over the past decade.<a href="#accordion">[7]</a>

However, it is important to note that eye cancer is still considered a rare form of cancer compared to other types, such as lung, breast, and colorectal cancer. Around 850 new cases are diagnosed annually in the UK.<a href="#accordion">[9]</a>&nbsp;Further, only 3,500 new cases of the estimated 1.7 million cancer cases diagnosed in the US are of eye cancer.<a href="#accordion">[10]</a>

The 5-year survival rate for localized eye melanoma is 85%, while it plummets to 16% in the case of distant or metastasized cancer, indicating that early detection is important for a good outcome.<a href="#accordion">[11]</a> 
&nbsp;

It&rsquo;s important to mention here that not all eye tumors are cancerous. Some may be benign, while some may be malignant.

<ul>
	<li>Primary Eye Cancer &ndash; This describes cancer that starts in the eye.</li>
	<li>Secondary Eye Cancer &ndash; This describes cancer that has spread to another part of the body. It is more likely to occur in men with lung cancer and women with breast cancer.<a href="#accordion">[14]</a> 
	&nbsp;</li>
</ul>

<ol>
	<li>
	<h5>Intraocular Cancers</h5>
	</li>
</ol>

These include:

<ul>
	<li>Melanoma</li>
</ul>

Melanoma develops in pigment-producing cells called melanocytes in the eyeball, eyelid, or conjunctiva.

Uveal or choroidal melanoma is the most common type of eye melanoma in adults. The majority of them develop in the choroid part, while the rest develop in the iris or ciliary body. Also, they grow slowly and rarely metastasize.

Further, melanomas of the eyelid, or conjunctiva, are a rare form of eye cancer. Given they are extraocular cancer, their treatment differs from uveal melanoma. 
&nbsp;

<ul>
	<li>Squamous Cell Cancer Of The Eye</li>
</ul>

Another low-grade cancer, this is the most common cancer of the conjunctiva. Even though it typically grows on the surface of the conjunctiva, it can also grow into or around the eye.

A biopsy may also reveal conjunctival intraepithelial neoplasia, a pre-cancerous stage that may turn into invasive squamous cell cancer and metastasize. 
&nbsp;

<ul>
	<li>Lymphoma</li>
</ul>

Lymphoma is a cancer that originates in the lymphatic system. Primary intraocular lymphoma is a rare type of eye cancer that develops inside the eyes. The elderly, those who have AIDs, or those who have had organ transplants have a weak immune system that makes them highly susceptible to this type of eye cancer.

Ocular adnexal lymphoma develops in the tissue surrounding the eyes. It is treated similarly to primary intraocular lymphoma. 
&nbsp;

<ul>
	<li>Cancer Of The Eyeball In Children</li>
</ul>

Two types affect children, including retinoblastoma and medulloepithelioma. Retinoblastoma originates in the retina and primarily affects children under 5. Medulloepithelioma can develop in children aged 10 years and under. 
&nbsp;

<ol start="2">
	<li>
	<h5>Cancers Around The Eyeball</h5>
	</li>
</ol>

These include:

<ul>
	<li>Rhabdomyosarcoma</li>
</ul>

Rhabdomyosarcoma is a type of cancer that arises from soft muscle cells. Orbital rhabdomyosarcoma is the most common type of eye rhabdomyosarcoma and typically affects children. However, it can also affect babies and the elderly. 
&nbsp;

<ul>
	<li>Basal Cell Carcinoma</li>
</ul>

Also referred to as &ldquo;rodent ulcer,&rdquo; this is cancer of the skin cells. As such, it can also affect the eyelids. 
&nbsp;

<ul>
	<li>Lacrimal Gland Cancer</li>
</ul>

This begins in the tear gland of the eye. It may be adenoid cystic carcinoma or lymphoma, with treatment depending on the size and type of cancer. 
&nbsp;

Sign and Symptoms

The signs and symptoms of eye cancer can vary depending on the type and location of the tumor. Some common signs and symptoms include:<a href="#accordion">[9]</a>

<ul>
	<li>Blurred vision, loss of vision, or a dark spot in the field of vision</li>
	<li>Pain in or around the eye</li>
	<li>Persistent irritation or inflammation of the eye</li>
	<li>Bulging or swelling of the eye, eyelid, or surrounding tissues</li>
	<li>Floaters or flashes of light</li>
	<li>Lump on the eyelid</li>
</ul>

Note: These symptoms are not specific to cancer. Other eye conditions can also present with these symptoms and, therefore, should be properly evaluated by a healthcare professional. 
&nbsp;

Causes

To date, there is no clarity as to what causes eye cancer. Generally, DNA changes cause healthy eye cells to multiply out of control and accumulate in the eye. It may be inherited or acquired during the individual&rsquo;s lifetime. 
&nbsp;

Currently, there are no regular screening tests for eye cancer.

However, eye exams should be a part of routine checkups, especially if the individual presents with dysplastic nevus syndrome (a lot of irregular moles on their body) or eye nevus (mole on the iris) that needs to be monitored closely. 
&nbsp;

Doctors should also check for any signs of tumors in high-risk individuals. For instance, those with a family history of retinoblastoma are likely to be born with a mutation in the RB1 gene. Nearly 40% of people who have this mutation develop eye cancer, which typically affects both eyes.

Screening for them involves regular eye examinations from birth to the age of 3 years. In some cases, a blood test may help diagnose the condition and provide information about the risk of developing cancer in other family members.<a href="#accordion">[14]</a> 
&nbsp;

Diagnosis

The diagnosis of eye cancer involves a combination of clinical evaluation, imaging tests, and pathological examination of tissue samples. The specific methods and procedures used for diagnosing eye cancer may vary depending on the suspected type and location of the tumor.<a href="#accordion">[12]</a> 
&nbsp;

<ol>
	<li>
	<h5>Clinical Evaluation</h5>
	</li>
</ol>

A thorough clinical evaluation, including a detailed medical history and physical examination, is essential for the initial assessment of a suspected eye tumor. The healthcare provider may ask about symptoms, risk factors, and a family history of eye cancer. 
&nbsp;

<ol start="2">
	<li>
	<h5>Imaging Tests</h5>
	</li>
</ol>

Imaging tests, such as ultrasound and fluorescein angiography, may be used to visualize the tumor and determine its size and location. Further, chest X-rays, CT scans, PET scans, and MRIs may be needed to assess the extent of the spread. 
&nbsp;

<ol start="3">
	<li>
	<h5>Biopsy</h5>
	</li>
</ol>

A biopsy involves the removal of a small sample of tissue from the tumor to determine the type of cancer and its characteristics. A biopsy can be performed using various techniques, including fine-needle aspiration biopsy, incisional biopsy, or excisional biopsy, to find out how aggressive it is and which certain treatments can help. 
&nbsp;

<h5>Cancer Staging</h5>

Staging determines the location of the cancer, whether it has spread to other parts of the body, and where it has spread. It can be pathological (based on what&rsquo;s found during surgery) or clinical (based on tests before surgery). 
&nbsp;

The most common systems for eye melanomas are TNM staging and COMS tumor classifications.<a href="#accordion">[15]</a>

<ol>
	<li>TNM Staging</li>
</ol>

This is a very detailed staging system that classifies the disease according to Tumor, Node, and Metastasis (TNM).

<ul>
	<li>T &ndash; Describes the size of the tumor, where it is located in the eye, and whether it has spread to the inside or outside of the eye</li>
	<li>N &ndash; Describes cancer that has spread to nearby lymph nodes around the neck or ear</li>
	<li>M &ndash; Describes cancer that has spread to distant parts of the body (most commonly the liver) 
	&nbsp;</li>
</ul>

Tumor

<ul>
	<li>TX &ndash; The primary tumor cannot be evaluated</li>
	<li>T0 &ndash; There is no tumor 
	&nbsp;</li>
</ul>

For Iris Melanoma

<ul>
	<li>T1 &ndash; The tumor is limited to the iris</li>
	<li>T1a &ndash; The tumor is 1/4 or less of the iris</li>
	<li>T1b &ndash; The tumor is more than 1/4 of the iris</li>
	<li>T1c &ndash; The tumor is present in the iris only but accompanied by melanomalytic glaucoma 
	&nbsp;</li>
	<li>T2 &ndash; The tumor has spread to the ciliary body and/or choroid</li>
	<li>T2a &ndash; The tumor has spread to the ciliary body</li>
	<li>T2b &ndash; The tumor has spread to the ciliary body and choroid</li>
	<li>T2c &ndash; The tumor has spread to the ciliary body and/or choroid, causing melanomalytic glaucoma 
	&nbsp;</li>
	<li>T3 &ndash; The tumor has spread to the ciliary body and/or choroid and is spreading to sclera</li>
	<li>T3a &ndash; The tumor has spread to the ciliary body and/or choroid and is spreading to the sclera in association with melanomalytic glaucoma 
	&nbsp;</li>
	<li>T4 &ndash; The tumor has spread to the optic nerve, the outside of the eyeball, or the eye socket</li>
	<li>T4a &ndash; The tumor has spread less than 5 mm outside of the eye</li>
	<li>T4b &ndash; The tumor has spread more than 5 mm outside of the eye 
	&nbsp;</li>
</ul>

For Ciliary Body And Choroid Melanoma

<ul>
	<li>T1 &ndash; The tumor is size category 1</li>
	<li>T1a &ndash; The tumor does not involve the ciliary body or other parts of the eye</li>
	<li>T1b &ndash; The tumor involves the ciliary body</li>
	<li>T1c &ndash; The tumor does not involve the ciliary body but a small area (5 mm or less in diameter) has spread beyond the eyeball; this is called extraocular spread</li>
	<li>T1d &ndash; The tumor involves the ciliary body with extraocular spread of less than 5 mm 
	&nbsp;</li>
	<li>T2 &ndash; The tumor is size category 2</li>
	<li>T2a &ndash; The tumor does not involve the ciliary body or other parts of the eye</li>
	<li>T2b &ndash; The tumor involves the ciliary body</li>
	<li>T2c &ndash; The tumor does not involve the ciliary body but has an extraocular spread of 5 mm or less in diameter</li>
	<li>T2d &ndash; The tumor involves the ciliary body with extraocular spread of less than 5 mm 
	&nbsp;</li>
	<li>T3 &ndash; The tumor is size category 3</li>
	<li>T3a &ndash; The tumor does not involve the ciliary body or other parts of the eye</li>
	<li>T3b &ndash; The tumor involves the ciliary body</li>
	<li>T3c &ndash; The tumor does not involve the ciliary body but has an extraocular spread of 5 mm or less in diameter</li>
	<li>T3d &ndash; The tumor involves the ciliary body with extraocular spread of less than 5 mm 
	&nbsp;</li>
	<li>T4 &ndash; The tumor is size category 4</li>
	<li>T4a &ndash; The tumor does not involve the ciliary body or other parts of the eye</li>
	<li>T4b &ndash; The tumor involves the ciliary body</li>
	<li>T4c &ndash; The tumor does not involve the ciliary body but has an extraocular spread of 5 mm or less in diameter</li>
	<li>T4d &ndash; The tumor involves the ciliary body with extraocular spread of less than 5 mm</li>
	<li>T4e &ndash; The tumor is any size category with extraocular spread greater than 5 mm in diameter 
	&nbsp;</li>
</ul>

For Conjunctival Melanoma

<ul>
	<li>T1 &ndash; The tumor is present in the bulbar conjunctiva</li>
	<li>T1a &ndash; The tumor is less than 1/4 of the bulbar conjunctiva</li>
	<li>T1b &ndash; The tumor is in 1-2 quadrants of the bulbar conjunctiva</li>
	<li>T1c &ndash; The tumor is in 2-3 quadrants of the bulbar conjunctiva</li>
	<li>T1d &ndash; The tumor is in 3-4 quadrants of the bulbar conjunctiva 
	&nbsp;</li>
	<li>T2 &ndash; The tumor is in the nonbulbar conjunctiva and will probably spread to the caruncle</li>
	<li>T2a &ndash; The tumor is at most 1/4 of the nonbulbar conjunctiva and not spread to the caruncle</li>
	<li>T2b &ndash; The tumor is in more than 1 quadrant of the nonbulbar conjunctiva and not spread to the caruncle</li>
	<li>T2c &ndash; The tumor is in at most 1/4 of the nonbulbar conjunctiva and has spread to the caruncle</li>
	<li>T2d &ndash; The tumor is in more than 1 quadrant of the nonbulbar conjunctiva and not spread to the caruncle 
	&nbsp;</li>
	<li>T3 &ndash; The tumor is any size and spread to nearby structures of the eye</li>
	<li>T3a &ndash; The tumor has spread to the globe</li>
	<li>T3b &ndash; The tumor has spread to the eyelid</li>
	<li>T3c &ndash; The tumor has spread to the orbit</li>
	<li>T3d &ndash; The tumor has spread to the nasolacrimal duct, paranasal sinuses, and/or lacrimal sac 
	&nbsp;</li>
	<li>T4 &ndash; The tumor is any size and spread to the central nervous system 
	&nbsp;</li>
</ul>

Node

<ul>
	<li>NX &ndash; The regional lymph nodes cannot be evaluated</li>
	<li>N0 &ndash; The cancer has not spread to the regional lymph nodes</li>
	<li>N1 &ndash; The cancer has spread to the regional lymph nodes or elsewhere</li>
	<li>N1a &ndash; The cancer has spread to the regional lymph nodes</li>
	<li>N1b &ndash; The cancer has not spread to the regional lymph nodes but to other parts of the eye 
	&nbsp;</li>
</ul>

Metastasis

<ul>
	<li>MX &ndash; The distant metastasis cannot be evaluated</li>
	<li>M0 &ndash; There is no distant metastasis</li>
	<li>M1 &ndash; Cancer has spread to other parts of the body</li>
	<li>M1a &ndash; Cancer has spread and the biggest metastasis is 3 cm or less in diameter</li>
	<li>M1b &ndash; Cancer has spread and the biggest metastasis is 3.1-8 cm in diameter</li>
	<li>M1c &ndash; Cancer has spread and the biggest metastasis is larger than 8.1 cm in diameter 
	&nbsp;</li>
</ul>

Important!

<ul>
	<li>Letters or numbers following the TNM stages are to provide greater detail about each factor. The more advanced the cancer is, the higher these numbers or letters will be.</li>
	<li>In the case of uveal melanoma, T for conjunctival melanomas and ciliary body and choroidal melanomas is different than T for iris melanoma.</li>
	<li>N and M staging is the same for iris, ciliary body, and choroidal melanomas, but different for conjunctival melanoma.</li>
	<li>M1a, M1b, and M1c do not apply to conjunctival melanoma. 
	&nbsp;</li>
</ul>

The TNM information is then combined to stage the cancer:

<ul>
	<li>Stage I &ndash; T1a, N0, M0</li>
	<li>Stage IIA &ndash; T1b, T1c, T1d, T2a, N0, M0</li>
	<li>Stage IIB &ndash; T2b, T3a, N0, M0</li>
	<li>Stage IIIA &ndash; It can be any of the following conditions:</li>
</ul>

<ol style="margin-left:40px">
	<li>T2c, T2d, N0, M0</li>
	<li>T3b, T3c, N0, M0</li>
	<li>T4a, N0, M0</li>
</ol>

<ul>
	<li>Stage IIIB &ndash; It can be any of the following conditions:</li>
</ul>

<ol style="margin-left:40px">
	<li>T3d, N0, M0</li>
	<li>T4b, T4c, N0, M0</li>
</ol>

<ul>
	<li>Stage IIIC &ndash; T4d, T4e, N0, M0</li>
	<li>Stage IV &ndash; It can be any of the following conditions:</li>
</ul>

<ol style="margin-left:40px">
	<li>Any T, N1, M0</li>
	<li>Any T, any N, M1</li>
</ol>

<ul>
	<li>Recurrent &ndash; Cancer returns after treatment 
	&nbsp;</li>
</ul>

<ol start="2">
	<li>
	<h5>COMS Staging</h5>
	</li>
</ol>

This is devised by the Collaborative Ocular Melanoma Study (COMS) group and is relatively simpler than TNM. According to this, eye melanomas can be divided into:

<ul>
	<li>Small &ndash; 1-3 mm in height and 5-16 mm in diameter</li>
	<li>Medium &ndash; 3.1-8 mm in height and not more than 16 mm in diameter</li>
	<li>Large &ndash; Greater than 8 mm in height and greater than 16 mm in diameter 
	&nbsp;</li>
</ul>

Treatment

The treatment of eye cancer depends on several factors, including the type and stage of the tumor, the location of the tumor, and the individual&#39;s overall health.<a href="#accordion">[11]</a>

<ul>
	<li>Surgery is a common treatment option for small, localized eye tumors. The type of surgery performed depends on the location and size of the tumor. Surgical interventions may include transscleral resection, iridectomy, enucleation, iridocyclectomy, or orbital exenteration. 
	&nbsp;</li>
	<li>Radiation Therapy, such as brachytherapy or external beam radiation therapy, is more common than surgery to treat eye cancer. It uses high-energy radiation to kill cancer cells or shrink tumors. It is the most common treatment for eye cancer. Sometimes, laser therapy may follow brachytherapy to prevent the cancer from recurring. 
	&nbsp;</li>
	<li>Chemotherapy involves the use of drugs to kill cancer cells or stop their growth. It is not a primary treatment for eye cancer but may be recommended if the tumor is metastatic or not responding to any other treatment. The drugs are taken intravenously, orally, or injected into a certain part of the body, such as the liver. 
	&nbsp;</li>
	<li>Targeted Therapy is a type of treatment that targets specific parts of cancer cells to destroy them. For example, targeted therapy drugs may be used to target the BRAF gene mutation present. 
	&nbsp;</li>
	<li>Immunotherapy involves the use of drugs that stimulate the body&rsquo;s immune system to recognize and destroy cancer cells. Drugs include immune checkpoint inhibitors and bispecific T-cell engagers. 
	&nbsp;</li>
	<li>Laser Therapy is considered in cases where radiation or surgery is not possible. It includes transpupillary thermotherapy and laser photocoagulation. 
	&nbsp;</li>
</ul>

Eye cancer and its treatment can lead to various complications, including<a href="#accordion">[13]</a>:

<ul>
	<li>Vision loss or blindness, particularly if the tumor affects the retina or optic nerve</li>
	<li>Metastasis, with cancer spreading to other parts of the body, such as the liver, lungs, or bones</li>
	<li>Glaucoma 
	&nbsp;</li>
</ul>

These include:

<ul>
	<li>Ethnicity, e.g., people with lighter eye color and fair skin</li>
	<li>Prolonged Exposure to UV radiation from sunlight or tanning beds</li>
	<li>Family History</li>
	<li>Underlying Medical Conditions, such as skin melanoma</li>
	<li>Age; eye cancer can occur at any age but mostly affects adults in their 50s</li>
	<li>Gender; primary eye cancers affect men more than women 
	&nbsp;</li>
</ul>

Some common preventive measures for eye cancer include: 
&nbsp;

<ul>
	<li>
	<h5>UV Protection</h5>
	</li>
</ul>

Protecting the eyes from prolonged exposure to UV radiation may help reduce the risk of eye cancer. This can be achieved by wearing sunglasses with 99-100% UVA and UVB absorption, wide-brimmed hats, and protective clothing. Also, avoid direct sunlight during peak hours and wear sunscreen with an SPF of at least 30 all year. 
&nbsp;

<ul>
	<li>
	<h5>Regular Eye Examinations</h5>
	</li>
</ul>

Regular eye examinations, particularly in individuals at high risk, may help detect eye tumors at an early stage when treatment is most effective. Individuals with a family history of eye cancer or certain genetic conditions may benefit from genetic counseling. 
&nbsp;

<ul>
	<li>
	<h5>Occupational Safety</h5>
	</li>
</ul>

Certain studies suggest welders are at increased risk of uveal cancer.<a href="#accordion">[10]</a>&nbsp;Individuals working in occupations that involve exposure to hazardous substances or UV radiation should follow appropriate safety measures, such as wearing protective eyewear and following workplace safety guidelines. 
&nbsp;

<ul>
	<li>Healthy Lifestyle</li>
</ul>

Maintaining an overall healthy lifestyle, including a balanced diet, regular exercise, and avoiding smoking, may help reduce the risk of developing eye cancer.

Note: These are not yet proven to prevent eye cancer from progressing or recurring. More research is needed to verify these preventive measures.

[1] Arepalli, S., Kaliki, S., & Shields, C. (2015). Choroidal metastases: origin, features, and therapy. Indian Journal of Ophthalmology, 63(2), 122.

[2] Leclerc, R. and Olin, J. (2019). An overview of retinoblastoma and enucleation in pediatric patients. Aorn Journal, 111(1), 69-79.

[3] Goto, S., Takeda, H., Sasahara, Y., Takanashi, I., & Yamashita, H. (2019). Metastasis of advanced gastric cancer to the extraocular muscle: a case report. Journal of Medical Case Reports, 13(1).

[4] Loda, A., Turati, M., Semeraro, F., Rezzola, S., & Ronca, R. (2022). Exploring the fgf/fgfr system in ocular tumors: new insights and perspectives. International Journal of Molecular Sciences, 23(7), 3835.

[5] Smith, J., Stempel, A., Bharadwaj, A., & Appukuttan, B. (2016). Involvement of b cells in non-infectious uveitis. Clinical & Translational Immunology, 5(2), e63.

[6] Li, Y., Wang, H., Lu, Z., Sun, J., Zhang, J., Jiang, F., … & Xu, A. (2021). Incidence and mortality of eye cancer in shandong, china: a population-based study during 2013-2017..

[7] Siegel, R. L., Miller, K. D., & Jemal, A. (2020). Cancer statistics, 2020. CA: A Cancer Journal for Clinicians, 70(1), 7-30.

[8] Keay, L., Ren, K., Nguyen, H. H., Vajdic, C. M., Odutola, M., Gyawali, R., … & Jalbert, I. (2022). Risk factors common to leading eye health conditions and major non-communicable diseases: a rapid review and commentary. F1000Research, 11, 1289.