Eye cancer develops in the eye and is a rare disease. The diagnosis is difficult and is usually made during a routine eye examination. Several eye cancer types exist, depending on the structure and the cells involved. The stages of eye tumors range from stage 1 to stage 4.
The eye is a complex organ comprising tissues with significant structural and functional variations. These structures include the conjunctiva, retina, choroid, ciliary body, iris, and lacrimal gland. When the cells of one or more of these structures undergo mutation and start dividing and proliferating uncontrollably, the condition is known as eye cancer. An eye tumor is both life-threatening and vision-threatening and has the potential to metastasize to other organs.
Eye cancer is a rare disease. Most cases of eye cancer are secondary, i.e., the cancer starts in other parts of the body and spreads to the eyes (metastasis). In the United States, the estimated prevalence rate is about 12/100,000 people, while the incidence rate is about 1/100,000 people. In India, the incidence rate of eye tumors among all cancers is about <0.5%. Studies have reported a higher burden of eye tumors in India, and the cases are increasing. The increasing trend may partially be due to better cancer detection techniques, epidemiological transition, and more effective cancer data collection.
Depending on the type of cell it originates from, eye cancer is classified into different types. The following are the different types of eye cancer:
It is one of the most common primary eye cancer types and affects the uvea. The condition may develop in the choroid, ciliary body, or iris. The condition is also subdivided into classes 1 (having low metastatic risk) and 2 (having high metastatic risk). Patients with uveal melanoma have higher metastatic disease-related mortality and visual morbidity.
This condition occurs in the iris and constitutes about 5% of all uveal melanomas. Most iris melanoma cases are asymptomatic and generally diagnosed during routine eye examinations.
It is a rare subtype of uveal melanoma and generally occurs with iris and choroidal melanoma. The condition may present without symptoms for a considerable period and is generally diagnosed in advanced stages.
It is one of the most common primary intraocular cancers. It is the second most common location for developing melanoma. The condition is diagnosed through various techniques, such as indirect ophthalmoscopy, fundus fluorescein angiography, A- and B-ultrasonography scans, and transillumination.
Intraocular lymphoma is an ocular cancer generally considered a part of central nervous system lymphoma. The disease includes primary and secondary intraocular lymphoma. The condition may develop in the optic nerve, Bruch's membrane, vitreous, uvea, and retina. Intraocular lymphoma primarily arises from the B-cell; however, some intraocular lymphoma may also arise from T-cells. Intraocular cancers are tumors inside the eye.
Orbital tumors are benign or malignant lesions that develop in the tissues surrounding the eyes. They may be primary or secondary. This type of eye tumor may be characterized by vascular lesions, cysts, neurogenic tumors, lymphomas, and secondary tumors. Eyelid tumors develop in the eyelid.
It is the most common type of eyelid cancer (90%) and usually develops in the lower eyelid. The risk factors include a history of ionizing radiation exposure, systemic and local immune dysfunction, and ultraviolet radiation.
Squamous cell carcinoma of the eyelid constitutes about 5% of all eyelid tumors. Risk factors for this condition include chronic skin lesions, albinism, and genetic skin disorders, such as epidermodysplasia verruciformis and xeroderma pigmentosum.
The condition commonly affects children and is characterized by the presence of tumor lesions around the eye. Almost 90% of cases of orbital rhabdomyosarcoma occur before the age of 16, and the mean age of onset is around 5 to 7 years.
Conjunctival tumors occur in the conjunctiva and are divided into carcinoma in situ, dysplasia, and squamous cell carcinoma. The risk factors for these tumors include immunodeficiency, human papillomavirus (HPV), and exposure to ultraviolet radiation.
Lacrimal gland tumors occur in the lacrimal glands of the eye and constitute about 10% of all orbital tumors. Lacrimal gland tumors are of different types, such as epithelial tumors and lacrimal gland lymphomas.
It is one of the most common cancers in childhood and constitutes about 3% of all childhood malignancies. The condition is characterized by strabismus, leukocoria, red eyes, pain, and reduced vision.
Basal cell carcinoma may occur in various parts of the eye, including the conjunctiva. However, BCC in conjunctiva is rare.
Conjunctival squamous cell carcinoma is a subset of ocular surface squamous neoplasia (OSSN). The disease is characterized by vision loss. The advanced stage of the disease may cause death.
Different stages of intraocular melanoma involving the iris are:
The lesion is limited only to the iris and is less than one-fourth of the size of the iris.
It is limited to the iris and greater than one-fourth of the iris size, OR is limited to the iris and results in glaucoma, OR extends to the ciliary body.
The tumor extends into the choroid and does not cause glaucoma, OR the tumor grows into the choroid, ciliary body, or both with or without involving the sclera.
The tumor extends into the choroid and causes glaucoma, OR the tumor extends out of the sclera, but its size is limited to less than 5 mm in diameter.
The tumor extends out of the sclera with a diameter greater than 5 mm.
Cancer grows into the surrounding lymph nodes or distant organs, including the liver, lungs, or bone.
Eye cancer symptoms may not always be obvious. Most eye cancer symptoms overlap with those of other ocular diseases. It is important to consult the doctor if the symptoms are persistent and affect vision. There are several reasons for eye cancer.
Following are the various sites at which eye cancer commonly occurs:
The eyelid comprises a thin skin that covers and protects the eye. Eyelid cancer may occur in or on the eyelid. Several types of eyelid cancer include basal cell carcinoma, sebaceous carcinoma (cancer of the glands in the eyelid), squamous cell carcinoma, and melanoma.
The colored section of the eye visible from the outside is known as the iris. Iris melanoma may occur in the iris. Patients may not experience iris melanoma symptoms in the early stages. Ophthalmologists may notice it during routine eye examinations. Evidence indicative of iris melanoma includes blood vessels in the tumor, a deformed pupil, secondary glaucoma, and cataracts below the tumor.
Choroid melanoma is the most common primary intraocular cancer of the eye in adults. It has a high risk of metastasis. Patients with choroidal melanoma experience reduced vision or vision loss, visual field defects, and photopsia.
It is a rare type of melanoma, a subset of uveal melanoma. It is difficult to diagnose the disease early, as the symptoms of ciliary melanoma, such as blurred vision, overlap with the other less serious eye diseases.
The following are the different eye cancer symptoms:
The presence of eye flashes is a symptom of various ocular conditions, including eye cancer. The symptom indicates vision-threatening ocular conditions and should be immediately reported to the ophthalmologist.
Patients with eye cancer may also have blurred vision. It is the growth of lesions or tissue mass around or in the eye that potentially reduces the overall visual field and leads to blurred vision.
Not all eye cancer results in complete or partial vision loss. The severity of vision loss depends upon several factors, such as the size, location, and extent of the cancer. This may be a sign of a tumor behind the eye.
Eye bulging is a common symptom in patients with orbital tumors. The growth of the tumor in the orbit disturbs the original position of the eyeball, leading to a bulging appearance. The bulging eye may be an eyeball cancer symptom.
When the eyelid cells divide and grow uncontrollably, the patients have a tumor mass or lump in the eyelid.
Eye cancer causes irritation in various ways. First, it disturbs the anatomy of the eye structures and pushes the healthy tissues. Second, it affects the lubrication and overall tear film of the eye. It may be an early-stage eye cancer symptom.
Various eye cancers, such as retinoblastoma, orbital tumors, and intraocular melanoma, cause eye pain. The severity of the pain depends on the affected structure.
Signs and symptoms of eye cancer present in children are:
Children with retinoblastoma are presented with a white or cloudy pupil appearance. The presence of a white or cloudy pupil is known as leukocoria.
Eye muscles and nerves control eye movements. When eye cancer affects the muscles, nerves, or both, the patient may have crossed eyes or strabismus.
Eye redness and swelling are some of the most non-specific eye cancer symptoms, as these are frequently encountered with several other common eye conditions. Eye redness and swelling may be due to intraocular cancer, conjunctival tumors, and eyelid tumors.
Excessive and prolonged irritation due to cancerous tissues in the eyes may lead to epiphora or excessive tearing. Although various less serious conditions may also cause excessive tearing, patients should consult ophthalmologists for appropriate diagnosis and management.
Although we do not know what causes eye cancer exactly, we do know that certain factors increase its risk. Some of the important eye cancer causes are:
Eye color may not have a direct association with eye cancer. However, studies have reported that people with light-colored eyes are more likely to develop ocular melanoma.
Eye cancer may occur at any age. However, certain eye cancers are more common in people of particular age groups. For instance, retinoblastoma usually occurs in children under the age of 5, while ocular melanoma commonly develops in people between 50 and 60 years of age. Men are more vulnerable to eye cancer than women.
Certain inherited genetic disorders can increase the risk of eye cancer and, therefore, are considered possible eye cancer causes. These include ocular melanoma and retinoblastoma.
Moles present on the white portion of the eye have the potential to develop into conjunctival melanoma. This makes moles one of the possible eye cancer causes.
Eyes are complex and delicate organs, and any eye disease symptoms should be immediately reported to an ophthalmologist. Unfortunately, several eye conditions are irreversible, and early diagnosis and management are key to preventing permanent damage. Individuals with eye pain, irritation, swelling, redness, blurred vision, floaters, flashes, and crossed eyes should consult oncologists.
An eye cancer diagnosis is done using multiple test procedures. The earlier it is diagnosed, the better. Patients should consult the doctor if they have eye cancer symptoms. Several eye cancer treatments are available that improve the survival and quality of life of eye cancer patients.
People ask about how to detect eye cancer. Some of the tests for eye cancer diagnosis include:
A detailed eye examination is one of the most preliminary diagnostic tests for all eye conditions, including eye cancer. The ophthalmologists may ask the patients about the symptoms, the onset of the symptoms, their progression, and their severity. The doctor may also examine the external and internal structures of the eyes through the following methods:
An ophthalmoscope is a device used to determine the health status of various ocular structures, such as the retina, blood vessels, and optic nerves. The doctor may observe an alteration in the optic nerve or the presence of a retinal tumor during an ophthalmoscope examination. If there is suspicion of some serious condition, the doctor may advise imaging tests for a definitive diagnosis.
Although a slit lamp in itself is not a diagnostic tool for eye cancer, it provides a detailed illuminated view of the internal and external structures of the eye, such as the conjunctiva, cornea, lens, iris, retina, and vitreous. Slit lamps assist ophthalmologists in detecting various abnormalities of the eye, including eye cancer. However, confirmatory diagnosis is achieved through ocular biopsy and imaging techniques.
Various imaging tests are performed for eye cancer diagnosis. Some of these imaging eye cancer tests are:
Ocular ultrasound uses high-frequency sound waves to provide images of the eye structures. The technique is also known as ocular echography. Ocular ultrasound may be an A-scan or a B-scan ultrasound. It helps detect the presence of a tumor, the type of tumor (cystic or solid), the internal components of the tumor, and the precise tumor location.
A CT scan uses X-rays to provide detailed cross-sectional images of the eyes. CT offers detailed and valuable information about ocular lesions. CT scans in patients suspected of eye cancer serve purposes such as detection and localization of the tumor, evaluation of tumor size, assessment of tumor extension, determining metastasis, and analyzing periorbital and orbital structures.
It uses radio waves and powerful magnetic fields to develop clear and detailed images of the eye structures. MRI helps evaluate the eyes' soft tissues, such as the optic nerve, retina, and surrounding tissues. It also assists in determining the involvement of brain and orbital structures. Doctors may also plan the treatment based on information received from the MRI images.
These are not the first-line imaging techniques for eye cancer. A PET scan is usually implemented to gather extra information about eye cancer once the other imaging modalities strongly suspect the disease. A PET scan provides detailed and accurate information about the site and extent of cancer spreading to other organs. It helps in planning the treatment and evaluating the treatment response.
It is one of the imaging modalities for eye cancer diagnosis. It helps detect blood flow to the choroid and retina of the eye. The technique is used to identify various types of eye cancers, including choroidal melanoma and retinal tumors. Apart from these, the technique also detects vascular abnormalities. During this procedure, the doctor injects a fluorescent dye into the vein and tracks it with the help of a special camera.
A biopsy is the technique of obtaining samples of abnormal tissues and sending them to the laboratory to detect any abnormalities. Several techniques of biopsy are used for eye cancer diagnosis.
An incisional biopsy involves making an incision to remove the tissue samples. This technique is used when large amounts of tissue from a specific portion of the tumor are required or when it is impossible to remove the complete tumor. Incisional biopsy may sometimes be used to obtain samples of intraocular tumors, such as retinoblastoma.
It involves the removal of the entire abnormal tissue from the tumor along with the surrounding margin of healthy tissues for evaluation. This technique is used to determine the type of cancer and tumor's location and size, preserve the eye's function, confirm diagnosis, and as a treatment modality.
It uses a fine needle to obtain a tissue sample for further evaluation. It is indicated when the tumor is located in or surrounding the eye. It is useful for taking tumor samples located in the soft tissues (around the eye) or the orbit.
Several eye cancer treatment methods are available. The choice and efficacy of treatment for cancer of the eye depend on several factors, such as stage of disease diagnosis, site of cancer, response to treatment, and the patient's overall health to withstand the eye tumor treatment. Some of the eye cancer treatment options are:
Surgery is one of the most common eye cancer treatment modalities to remove the tumor, preserve ocular function, and manage the surrounding affected tissues. Several types of surgeries are available for eye cancer treatment, including:
Enucleation is the method that involves the complete removal of the affected eye. It is recommended for patients with extensive ocular tumors that cannot be effectively treated with other treatment methods. It is one of the most common treatments for retinoblastoma. The doctor may perform cosmetic surgery to place a prosthetic eye to improve its appearance.
It is a complex ocular surgery that removes all the components of the eye socket, including the eyeball, muscles, and surrounding structures. It is usually a treatment modality in which there is extensive involvement of the orbital tissues.
This procedure involves removing a part of the iris (the colored part of the eye). It is not the commonly recommended eye cancer treatment approach. However, oncologists may implement this technique in certain cases, such as malignant or benign tumors of the iris. This technique is also used for managing glaucoma and in iris repair.
This eye cancer treatment involves the removal of the ciliary body and iris in patients with tumors of the ciliary body and iris. The surgery aims to remove the most malignant tissues and preserve ocular function. Other treatment options, such as systemic therapies or radiation therapies, may be used with this procedure.
Transscleral resection is a procedure that involves the sclera, which is the outer layer of the eye. It is indicated for the treatment of ciliary body or choroidal tumors. The surgery aims to locally control the tumor, minimize functional damage, and prevent metastasis.
Eye radiation therapy is one of the most common modalities for eye cancer treatment. It uses high-energy radiation directed at the cancer cells to destroy them. Radiation therapy may be used along with surgery or chemotherapy to enhance their efficacy.
This technique involves the delivery of high-energy radiation from a machine outside the body to treat ocular cancer. EBRT is generally used when the tumor is large or is present in the back of the eye. It is usually indicated in choroidal melanoma and retinoblastoma.
Brachytherapy is the technique of delivering radiation by placing the radiation source near or inside the tumor. It is usually indicated in cases where the tumor is in the anterior eye segment. However, in some cases, this technique is also used as an eye cancer treatment for retinoblastoma and choroidal melanoma.
Laser therapy uses intense light beams to destroy cancerous tissues and abnormal blood vessels. The methods used in laser therapy include photocoagulation and photodynamic therapy. Photodynamic therapy uses light-activated drugs to improve its efficacy.
Chemotherapy is an eye cancer treatment modality that uses drugs to slow the growth of or kill cancer cells. It is usually used when the eye cancer metastasizes to other body organs. Chemotherapy may be delivered systemically or directly into the artery supplying blood to the eye (intra-arterial chemotherapy).
Immunotherapy for eye cancer works through two mechanisms. First, it interferes with the procedure in the cancer cells, which allows them to hide from the immune system. Second, they improve the immune system and strengthen it to recognize and kill cancer cells.
Targeted therapy for eye cancer treatment involves the delivery of drugs that interfere with processes or substances, such as proteins or enzymes, vital for the growth and proliferation of cancer cells. Unlike chemotherapy, targeted therapy attacks the pathways specific to the cancer cells and thus has relatively fewer side effects.
HCG is one of India's best institutes, specializing in comprehensive cancer care. The center is equipped with advanced diagnostic and treatment techniques for the effective management of eye cancer. The multidisciplinary team comprising medical oncologists, surgical oncologists, ophthalmologists, and paramedical staff is extensively experienced in managing simple to complex eye cancer cases.
There are several eye cancer risk factors. Some factors are specific to a particular age, while others are specific to a particular race and ethnicity. It is important to identify these eye cancer risk factors and follow eye cancer prevention measures to lower the risk.
Some of the common eye cancer risk factors are:
Although eye cancer may occur at any age, it is an eye cancer risk factor for several cancers. The risk of eye cancer usually increases with age, such as in uveal melanoma. However, certain eye cancers, such as retinoblastoma, generally occur at an early age.
Certain types of cancer, such as uveal melanoma and conjunctival melanoma, are more common in men than in women. However, the gender bias in eye cancer may vary widely and additionally depend on several other risk factors.
Certain types of eye cancer involve hereditary components. People with a family history of uveal melanoma are at greater risk of developing this condition than those with no family history of uveal melanoma. However, eye cancer running in families is rare, and the genetic causes behind it are under investigation.
People with a specific skin color or tone may have an increased risk for eye cancer. People with fair skin or freckles are at increased risk of developing eye cancer.
Eye color is another eye cancer risk factor. People with light-colored eyes are at higher risk for eye cancer. Further, gray, blue, or green eyes are also risk factors for ocular melanoma. People with abnormal spots on the uvea or iris have an enhanced risk for ocular melanoma.
The presence of certain inherited conditions is also an eye cancer risk factor. Dysplastic nevus syndrome, characterized by the presence of abnormal skin moles, increases the risk of eye cancer. Oculodermal melanocytosis increases the risk of uveal melanoma. Another rare inherited syndrome, BAP1 cancer syndrome, increases the risk for uveal melanoma along with various other cancers, such as malignant mesothelioma, skin melanoma, and kidney cancer.
Excessive exposure to solar radiation is also an eye cancer risk factor. Further, UV light from the tanning bed also increases the risk of ocular cancer.
The presence of unusual moles in the skin or the eyes increases the risk of uveal melanoma. The moles occur in the choroid and iris. Primary acquired melanosis, a condition characterized by the excessive growth of melanocytes in the eye, may increase the risk of conjunctival melanoma.
There are several other eye cancer risk factors. These include specific occupations such as welding, the presence of skin melanoma, HIV infection, HPV infection, use of immunosuppressant drugs, and white people (compared with Hispanics, African Americans, and Asian Americans).
Several people ask about how to prevent eye cancer. There is no method for complete eye cancer prevention. Certain measures may lower the risk of eye cancer. Following are some of the eye cancer prevention measures:
Appropriate doses of vitamin C and zinc not only reduce the risk of eye cancer but also help manage the symptoms of eye cancer and reduce the long-term complications and damage to the eyes. Vitamin C lowers the risk of age-related macular degeneration and cataracts. Both vitamin C and zinc improve the overall immunity that assists in the destruction of abnormal cells.
It is important to limit prolonged exposure to solar radiation. The steps that can lower sunlight exposure include tightly woven fabric without holes to cover the skin. A hat also helps protect the face and eyes from UV radiation.
There is a recommendation from the American Cancer Society to wear UV-protected sunglasses when going outside in strong sunlight. Wrap-around sunglasses provide 99% to 100% protection against ultraviolet radiation for the skin and eyes. These sunglasses may protect against eyelid cancer. Certain specialists believe that these sunglasses also reduce the risk of ocular melanoma.
People generally believe that smoking increases the risk of lung cancer. However, studies have reported that smoking also affects the cells of the eyes. It has been found that smoking increases the risk of dry eye syndrome, cataracts, age-related macular degeneration, and cataracts. Further, smoking reduces the flow of blood to the eyes, disrupts retinal function, and increases the risk of eye cancer. Thus, avoiding smoking lowers the risk of eye cancer and improves overall eye health.
Limiting alcohol consumption lowers the risk of eye cancer. Alcohol may be responsible for various ocular conditions, such as retinopathy, cataracts, and glaucoma. Consuming a significant amount of alcohol may alter the genetic makeup of eye cells and increase the risk of eye cancer.
It is important to avoid injuries to the eye, as certain eye injuries increase the risk of eye cancer.
Regular eye examinations are among the most important eye cancer prevention measures that lower the risk of eye cancer. Regular eye examination, especially in people with high eye cancer risk, assists in early diagnosis and appropriate management of those conditions that significantly enhance eye cancer risk.
Cancer of the eye occurs in any part of the eye. There are multiple eye cancer types. The most common eye cancer types include uveal melanoma, conjunctival cancer, eyelid cancer, and rhabdomyosarcoma. While stage 1 is the early stage or first stage of eye cancer, stage 4 is the last stage of eye cancer.
