Eye Cancer Guide

There are several types of ocular cancer that can develop in the portions of the eye. This is when healthy cells mutate and grow faster than usual, resulting in a tumor. 

Primary ocular cancer is a cancer that forms in the eye. Secondary ocular cancer is a cancer that forms somewhere else in the body, metastasizes, and then spreads to the eye. 

It is much more common for secondary cancers, such as breast or lung cancer, to metastasize and spread to the eye. Primary eye cancer is relatively rare, but certain types such as uveal melanoma can be serious, especially when spread to other parts of the body. 

Ocular cancer can occur in all parts of the eye. It can lead to partial vision loss, permanent blindness, and even death in rare cases.

Uveal Melanoma

Uveal melanoma is a rare cancer that forms in the middle layer of the eye’s wall (the uvea) and can spread to the other regions of the eye. Although rare, uveal melanoma is the most common primary ocular cancer. Melanoma that occurs on the ocular surface is conjunctival melanoma, which shares many similarities with uveal melanoma, but is much more rare. 

Symptoms

Early cases of uveal melanoma typically don’t cause any visible symptoms. When left untreated, the melanoma can spread and cause symptoms that include the following:

• Decreased or blurry vision
• Floaters that appear as tiny specks in the field of vision
• A dark spot on the iris (the colored tissue in the center of the eye) or the conjunctiva (the tissue covering the white portion of the eye) that keeps growing
• Flashing sensations
• Complete loss of peripheral vision
• Distorted or irregularly shaped pupil

Causes

Exact causes of uveal melanoma are still unknown, but there are several risk factors associated with it. Having blue or green eyes, being Caucasian, and being 70 or older are all common among people with uveal melanoma. Other risk factors include the following:

• Too much exposure to ultraviolet light
• An inherited predisposition for uveal melanoma
• A genetic mutation in the CDKN2A or the CDK4 genes

Diagnosis

Detectable symptoms may not occur for years, so diagnosing uveal melanoma is usually done by an optometrist or ophthalmologist during an examination. A magnetic resonance imaging (MRI) test is used to confirm the diagnosis. 

Treatment

The most common treatment for uveal melanoma is surgery. Depending on the severity of the disease, a surgeon can remove just the tumor or the entire eye and eyelid.

Other treatments that break down and help get rid of the cancer include the following:

• Radiation therapy: using x-rays or similar forms of radiation
• Photocoagulation: using laser beams or other intense light
• Thermotherapy: using heat from a laser

Prognosis & Complications

If uveal melanoma is successfully treated, chances of the disease reoccurring are low. However, uveal melanoma metastasizes about 50 percent of the time and typically spreads to the liver before ever being diagnosed. This scenario is almost always fatal and reaffirms the importance of routine eye exams.

An ophthalmologist is the best type of doctor to detect and treat uveal melanoma. An ophthalmologist may refer a patient to a surgeon if surgery is needed.

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Squamous Cell Carcinoma

Squamous cell carcinoma in the eye grows on the surface of the mucous membrane that covers the front of the eye (conjunctiva). The disease is rare and usually does not spread to other parts of the body. However, it can lead to vision loss and can even be fatal if left untreated.

Symptoms

Symptoms of squamous cell carcinoma of the conjunctiva include the following:

• Red or watery eyes
• Sensitivity to bright lights
• Irritation
• A foreign body sensation around the affected area
• Growth on the surface of the eye

Causes

Squamous cell carcinoma in the eye is typically caused by too much exposure to ultraviolet light. Other risk factors include being diagnosed with HIV/AIDS, contracting the human papilloma virus (HPV), and allergic conjunctivitis, which is an inflammatory response of the conjunctiva to an allergen.

Diagnosis

Diagnosing squamous cell carcinoma can sometimes be difficult due to variable symptoms. However, ophthalmologists are able to detect the cancer by the grayish color of the abnormal squamous cells.

Treatment

Surgery is the most common method of treating squamous cell carcinoma of the conjunctiva. After the initial surgery, adjuvant therapies are typically performed to ensure more tumors do not grow. These therapies include cryotherapy and the application of topical antibiotic steroid eye drops.

Prognosis & Complications

Around half of the people who undergo surgery to remove tumors from squamous cell carcinoma in the eye report having recurring tumors. To prevent the rapid growth and invasiveness of the disease, aggressive medical, surgery, or combination therapy is necessary. 

Retinoblastoma

Exclusive to young children and infants, retinoblastoma is a rare cancer that forms in the retina of the eye. The cancer can spread from the very back part to other portions of the eye. The disease usually only forms in one eye (unilateral retinoblastoma), but one in three children with the disease can develop it in both eyes (bilateral retinoblastoma).

Symptoms

An early sign of retinoblastoma is white pupillary reflex, or leukocoria. This is when an abnormal white reflection from the eye’s retina shows up in the pupil after being exposed to a bright light instead of a normal red reflection.

Other symptoms of retinoblastoma include the following:

• Pain or discomfort
• Redness or bleeding
• Strabismus (lazy eye)
• Swelling or bulging
• Different colored eyes

Causes

Most cases of retinoblastoma are caused by genetic mutations that begin during early fetal development. It is currently unknown what causes the genetic mutations. However, around 40 percent of retinoblastoma cases are inherited, while the rest are due to spontaneous genetic mutations.

Diagnosis

Diagnosing retinoblastoma is easiest if the disease is present in a child’s family history. That way, the child can be specifically tested for it within their first two years of life. 

Other ways of diagnosing retinoblastoma include the following:

• Ophthalmic examination: moving the retina into view to check for tumors
• MRI scan: using radio waves to create detailed images that may contain tumors
• Ultrasound: emitting sound waves to detect if tumors are present
• Electroretinogram: measuring the electrical activity of the retina in response to light

Treatment

The most common treatment for retinoblastoma is chemotherapy, which uses powerful chemicals to kill the cancerous cells. Systemic chemotherapy usually takes around six months to shrink the tumor, which makes other treatments more effective and can prevent secondary cancers. 

Other treatments that help break down the cancerous cells of retinoblastoma include the following: 

• Radiation therapy: using x-rays or similar forms of radiation
• Photocoagulation: using laser beams or other intense light
• Cryotherapy: using extreme cold
• Surgery: removing the entire eye and part of the optic nerve

Surgery is a rarer form of treatment for retinoblastoma and is only needed for severe cases with larger tumors.

Prognosis & Complications

Retinoblastoma has one of the highest survival rates of all pediatric cancers in developed countries. If left untreated, retinoblastoma can lead to retinal detachment, retinal necrosis, and orbital invasion.

An ophthalmologist checks for retinoblastoma during routine dilated eye exams in early childhood. If retinoblastoma is found, the ophthalmologist may refer the child to another specialist or a surgeon if surgery is required. 

Eye Lymphoma

Primary intraocular lymphoma (PIOL) is a rare type of eye cancer that forms in the retina and in the gel that fills the retina, the vitreous. Lymphoma can also develop near the eye (ocular adnexal lymphoma) and in the uvea (uveal lymphoma), but PIOL is the most common. 

Symptoms

Eye lymphoma typically affects both eyes, but symptoms may be more prevalent in one eye over the other. Symptoms of eye lymphoma include the following:

• Blurred field of vision 
• Impaired or complete loss of vision
• Increased sensitivity to light
• Floaters
• Redness or inflammation 

Causes

Eye lymphoma is caused by white blood cells (lymphocytes) developing a genetic mutation that allows them to multiply at a rapid rate. Even though mutated DNA causes eye lymphoma, the disease is not inherited like other types of cancers caused by genetic anomalies. Sex does not seem to be a factor either.

Diagnosis

An ophthalmologist typically uses a special lens that magnifies the retina in order to detect the presence of a tumor. If eye lymphoma is present, image testing is usually done in ensuing visits to monitor the spread of the irregular cell growth. Samples of the vitreous can also be tested to determine if tumors in the retina are related to eye lymphoma. 

Treatment

Similar to other types of cancer, chemotherapy is the most commonly used treatment for eye lymphoma. Other common treatments that help break down the tumors associated with eye lymphoma include the following:

• Radiation therapy: using x-rays or similar forms of radiation
• Targeted therapy: injecting drugs to target specific genes and proteins
• Immunotherapy: stimulating the immune response by substances made in the body

Prognosis & Complications

The prognosis for eye lymphoma depends on how far the cancer has spread and how it has been treated. Typically, the disease can be temporarily treated, but the prognosis remains poor with a survival rate of around 60 percent. 

Eye lymphoma can advance and damage the eye if left untreated or not detected soon enough, resulting in visual impairment or permanent blindness.

An ophthalmologist is the best type of doctor to diagnose and treat people with eye lymphoma.

Lacrimal Gland Cancer

Lacrimal gland cancer occurs within the orbit above the lateral end of the eye where tears are released. The disease is extremely rare, but it is often aggressive and can be fatal if left untreated or if it recurs.

Symptoms

Lacrimal gland tumors usually only form in one eye. Symptoms can include the following:

• Watery eyes
• A lump in the eyelid
• Blurry vision
• Swelling and bulging
• Impaired or complete loss of vision

Lacrimal gland cancer can also cause pain around the affected area, especially if left untreated.

Causes

Currently, there is not much known about what causes lacrimal gland cancer. However, research has shown that malignant tumors tend to grow faster than benign tumors.

Diagnosis

Lacrimal gland cancer can be found in people at any age, but it typically appears after the age of 60. Diagnosing the disease can be done by via these methods:

• Ophthalmic examination: moving the tear duct into view to check for tumors
• MRI scan: using radio waves to create detailed images that may contain tumors
• CT scan: combines a series of x-ray images taken from different angles to detect tumors

Biopsies can also be performed on samples of the lacrimal gland tumor to determine if it is cancerous or not.

Treatment

The most common treatment for lacrimal gland cancer is surgery, which usually involves the removal of the entire eyeball and eyelid. Other treatment options are available if the disease is caught early enough, including radiotherapy and chemotherapy. However, the best treatment of lacrimal gland cancer remains unclear.

Prognosis & Complications

The prognosis for lacrimal gland cancer depends on which stage the cancer is when it is found. The average survival rate 10 years after the disease is treated is approximately 20 to 30 percent. Since lacrimal gland cancer is so aggressive, it typically recurs after treatment.

References

1. Chemotherapy in Retinoblastoma: Current Approaches. (December 2015). Turkish Journal of Ophthalmology.

2. Clinical Management of Uveal Melanoma: A Comprehensive Review With a Treatment Algorithm. (September 2020). Radiation Oncology Journal.

3. Differential Diagnosis of Leukocoria and Strabismus, First Presenting Signs of Retinoblastoma. (December 2007). Clinical Ophthalmology.

4. History and Genetics of Retinoblastoma. (May 2019). Retinoblastoma – Past, Present and Future.

5. Intraocular Lymphoma. (August 2017). International Journal of Ophthalmology.

6. Iris Melanoma: Management and Prognosis. (October 2020). MDPI Applied Sciences.

7. Ocular Surface Squamous Neoplasia. (September 2022). American Academy of Ophthalmology.

8. Outcomes in Patients With Lacrimal Gland Carcinoma Treated With Definitive Radiotherapy or Eye-Sparing Surgery Followed by Adjuvant Radiotherapy. (June 2020). Radiation Oncology.

9. Orbital and Eyelid B-Cell Lymphoma: A Multicenter Retrospective Study. (September 2020). Cancers.

10. Primary Intraocular Lymphoma. (September 2014). Survey of Ophthalmology.

11. A Prognostic Test to Predict the Risk of Metastasis in Uveal Melanoma Based on a 15-Gene Expression Profile. (October 2013). Methods in Molecular Biology.

12. Squamous Cell Carcinoma of the Conjunctiva. (February 2017). Community Eye Health Journal.

13. Treatment of Retinoblastoma: What Is the Latest and What Is the Future. (April 2022). Frontiers in Oncology.

14. Uveal Melanoma: Relatively Rare but Deadly Cancer. (December 2016). Eye.