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The different structures that make up the eye and its surrounding area include:
Eyeball: The structures inside the eye where cancer can develop include the choroid, ciliary body, iris and retina.
Uvea is the middle layer of the eyeball. It contains several structures, including:
- Iris: The colored part of the eye. The pupil, a small opening that lets light come into the eyeball, is in the middle of the iris
- Choroid: Thin layer around the eyeball that provides the eye with blood
- Ciliary body: Made up of muscles inside eye that help it focus and cells where aqueous humor (clear liquid in front of eye) begins
- Retina: The layer of cells in the back of the eye. The retina is connected by the optic nerve to the brain. Its cells are sensitive to light and help us see images. When light comes into the eye, it passes through the lens. This makes an image on the retina, which is then sent by the optic nerve to the brain.
Orbit: The space around and behind the eye has bony walls and contains important nerves, including the optic nerve, and muscles that move the eye.
Eyelid: Tumors that affect eyelids can be on the skin of the eyelid or on the inside layer of the eyelid (tarsus and conjunctiva).
Conjunctiva: Surface covering of the eye that also covers the inside of the eyelid
Lacrimal gland: The gland, which makes tears, is in the upper outer quadrant of the orbit.
Lacrimal sac/duct: This structure, which drains the tears, is in the inner lower quadrant of the orbit near the nose.
Types of eye cancer
Cancers involving the eyeball
Uveal melanoma: This is the most common primary intraocular eye cancer in adults. It accounts for about 5% of all melanoma cases. The uvea is behind the sclera (the white of the eye) and the cornea (the window at the front of the eye). It has three parts: the iris (the colored part of the eye); the ciliary body (a ring of tissue with muscle fibers that change the size of the pupil and the shape of the lens); and the choroid, also known as the posterior uvea (the back part of the eye under the retina).
Uveal melanoma may have no symptoms, and some cases are found during routine eye exams. If there are symptoms, they may include vision changes, flashing lights, a dark spot on the iris, a change in the shape of the pupil, glaucoma and, rarely, eye pain or redness.
Retinoblastoma: Retinoblastoma is a cancer of the retina, a light-sensitive layer of tissue in the eye. Among children, it is the most common malignant tumor that starts in eye. It usually occurs before age five, and most of these cases occur in children under two. The disease accounts for 3% of cancers in children. Visit our retinoblastoma disease page to learn more.
Cancers of the eyelid
Basal cell carcinoma: More than 90% of eyelid cancers are basal cell carcinomas, a type of skin cancer. The lower eyelids are involved in more than 70% of cases, followed by the inner corner of the eye, upper eyelid and the outside corner of the eye. This cancer is usually not aggressive and does not spread to lymph nodes or distant organs.
The disease is treated with surgery to remove the tumor. The eyelid tissue will also be reconstructed to preserve the patient’s vision, maintain comfort and restore the eye’s appearance. In advanced cases, targeted therapy and adjuvant radiation therapy may be appropriate.
Squamous cell carcinoma: This skin cancer occurs less often on the eyelid than basal cell carcinoma, but it is more aggressive. It can spread to nearby lymph nodes and other parts of the body.
The main treatment for this type of eye cancer is surgical removal. Radiation therapy or other treatments may be used in addition to surgery if a large area is affected or if the tumor cannot be fully removed.
Melanoma: This type of skin cancer accounts for about 1% of eyelid cancers. It is potentially life-threatening. It can affect the eyelid skin or the conjunctiva.
Melanomas of the eyelid are surgically removed. The eyelid is then reconstructed in a separate procedure, usually done the next day. If the patient has a high-risk tumor, doctors may perform a lymph node biopsy to see if the cancer has spread. A sentinel lymph node biopsy can find microscopic signs of metastasis, which can help doctors diagnose and treat metastatic melanomas in the early stages.
Sebaceous carcinoma (meibomian gland carcinoma): This rare type of eyelid carcinoma is also known as sebaceous gland or sebaceous cell carcinoma. It affects the meibomian glands of the eyelids, as well as of the conjunctiva or other surface structures of the eye. These glands produce the oily layer of liquid that covers the eye.
Sebaceous carcinoma of the eyelid can be mistaken for non-cancerous conditions like a chalazion, a small cyst known as a sty. If a sty does not heal with medical treatment or surgical drainage, doctors should perform a biopsy on the growth.
Sebaceous cancer in the eyelid is surgically removed, and the eyelid is reconstructed. Sometimes patients are given topical chemotherapy in the form of eye drops to use after the surgical area has healed. Sebaceous carcinoma can spread to the regional lymph nodes. For larger sebaceous carcinomas, doctors can perform a sentinel lymph node biopsy to look for microscopic signs of the disease’s spread.
Merkel cell carcinoma: This is a rare but aggressive cancer that starts in the eyelid’s touch receptors. It usually shows up as a fast-growing purplish or flesh-colored mass.
Treatment for this cancer typically starts with surgery to remove the mass and reconstruct the eyelid. Adjuvant radiation therapy can also be used in some cases. Because of toxicity issues, radiation is typically used only on lower eyelid tumors. Doctors may perform a sentinel lymph node biopsy to determine if the cancer has spread to nearby lymph nodes.
In about 30% to 50% of patients, the cancer has spread to the lymph nodes. In these cases, the nodes also need to be treated surgically or with radiation therapy. For tumors larger than 20 mm (about an inch), adjuvant chemotherapy may be used to prevent the cancer’s return.
Cancers of the conjunctiva
Squamous cell carcinoma: This is the most common type of conjunctival eye cancer. It can affect the area around the cornea on the eye surface or the inner conjunctival layer of the eyelids. It has a very low risk of spreading to the lymph nodes.
This eye cancer is most often treated with surgery, and the eye is typically preserved. At the time of surgery, doctors usually perform cryotherapy (freezing treatment) along the edges of surgical area. Sometimes topical chemotherapy in the form of eye drops is used after surgery.
If this cancer returns in an aggressive form, the eye and eye socket may have to be removed.
Melanoma: Melanoma can occur on the conjunctiva on the surface of the eyeball (bulbar conjunctiva) or on the conjunctival covering of the inside of the eyelid (palpebral conjunctiva). Conjunctival melanomas can spread to the lymph nodes and other parts of the body. This risk is higher for thicker melanomas.
Conjunctival melanoma is treated with surgery combined with cryotherapy along the surgery’s edges. At the time of surgery, doctors can perform a sentinel lymph node biopsy to look for signs of early metastasis. Doctors use this procedure to determine the cancer’s stage and plan additional treatments, if needed. Sometimes doctors use chemotherapy eye drops after surgery to reduce the chance of recurrence.
Mucosa-associated lymphoid tissue lymphoma (MALT): This form of lymphoma occurs on the surface covering of the eye and the inside layer of eyelid (conjunctiva). It is a slow-growing form of B-cell lymphoma that usually shows up as a salmon-colored or light orange growth on the surface of the eye.
Treatments for this eye cancer may include chemotherapy, targeted therapy or radiation therapy. Recently, doctors reduced radiation therapy to very low dosages in order to decrease the treatment’s ocular toxicity.
Cancers of the orbit
Orbital lymphoma: This is the most common type of cancer of the orbit in adults. It is usually a form of B-cell non-Hodgkin lymphoma. It may show up as a nodule in the eyelid or around the eye, or in the lacrimal gland and the orbit. It may cause the eye to be pushed out. This type of cancer usually does not cause pain.
There are several types of B-cell lymphomas that occur in the orbit. These include MALT, follicular lymphoma, large B-cell lymphoma and mantle cell lymphoma. Less-common types include natural killer T-cell lymphoma, mycosis fungoidis and Hodgkin lymphoma.
Diagnosing orbital lymphoma often starts with a CT scan followed by a surgical biopsy. Treatment may include radiation therapy, targeted therapy, chemotherapy or a combination of these, depending on type of lymphoma and the stage of the cancer. Recently, doctors have gotten good results and much lower levels of ocular toxicity by using very low-dose radiation therapy.
Orbital sarcoma: Sarcomas arise from muscles or fatty tissue. The most common sarcoma in the orbit is rhabdomyosarcoma, which is found most frequently in children.
The most common type of orbital rhabdomyosarcoma can be treated with a combination of chemotherapy and proton therapy. This combination can prevent the need for removal of the eye and orbital content in the majority of patients.
Orbital and optic nerve meningiomas: These tumors are benign, meaning they are not cancerous and do not spread. They can be associated with the optic nerve. They can also form in the meninges (membranes that line the skull and enclose the brain and top of the spinal cord) in the base of the skull and extend into the orbit. The symptoms of these tumors may include a gradual loss of vision or visual field.
These tumors are usually diagnosed with an imaging exam, such as an MRI of the orbit. Surgical biopsies usually are not necessary and are avoided if possible to minimize risk of visual loss. Treatment usually includes radiation therapy, particularly proton therapy.
Metastatic orbital tumors: Almost all types of cancer can metastasize (spread) to the orbit (the space behind and around the eye). The most common are breast, lung and prostate cancer; melanoma; and carcinoid tumors. Metastatic tumors of the orbit are treated with chemotherapy, radiation and occasionally surgery.
Lacrimal gland tumors
Lymphoma: Lymphomas in the lacrimal gland usually form as lesions in the upper outer part of the eye. Diagnosis starts with a surgical biopsy. A pathologist will then determine the patient’s exact type of lymphoma. Treatment for lacrimal gland lymphoma is similar to other orbital lymphomas and may involve radiation therapy to the orbit, targeted therapy, chemotherapy or a combination of these treatments.
Adenoid cystic carcinoma: This rare cancer has the potential to spread and become life-threatening. For smaller or less aggressive tumors, eye-preserving surgery followed by proton therapy may be used. When the disease is more advanced or aggressive, the eye and surrounding soft tissue may need to be removed.
Pleomorphic adenoma: This is the most common benign (non-cancerous) tumor of the lacrimal gland. It is diagnosis based on clinical signs and imaging studies. The entire tumor should be surgically removed. The eye should always be spared in these procedures.
Other lacrimal gland carcinomas: Other forms of carcinoma such as adenocarcinoma or squamous carcinoma or mucoepidermoid carcinoma of lacrimal gland are managed similarly to adenoid cystic carcinoma.
Lacrimal sac and nasolacrimal duct cancers
Squamous cell carcinoma: As with squamous cell carcinomas in other locations, surgery is the main treatment. Radiation therapy, specifically proton therapy, is used after surgery to decrease the likelihood of recurrence. Usually it is to preserve the eye, though in some cases it may have to be removed, depending on the extent of disease.
Transitional cell carcinoma: This is a cancer of the lacrimal sac. Symptoms may include excessive tearing, bloody tears, frequent nosebleeds or a mass in the inner bottom corner of the orbit. Treatment for transitional cell carcinoma of the lacrimal sac includes surgery, followed by radiation therapy and proton therapy.
Lacrimal sac lymphoma: Treatment of this disease begins with a surgical biopsy to establish exact type of lymphoma. Treatment of lacrimal sac lymphoma is similar to other forms of orbital lymphoma. Depending on the type and stage of lymphoma, very low-dose radiation therapy or chemotherapy can be used
What are eye cancer's risk factors?
Eye cancer risk factors include anything that increases your chance of getting the disease. Certain factors seem to increase the odds you might get cancer in the eye or on the eyelid or orbital area.
These factors include:
- Light skin and/or blue eyes
- Exposure to sun or tanning salons may increase risk of carcinomas or melanomas on your eyelid.
- Exposure to certain viruses such as human papilloma virus (HPV) may increase the risk of squamous cell carcinoma of conjunctiva.
- Atypical mole and melanoma syndrome (AMS): Previously known as dysplastic nevus syndrome, AMS is characterized by large numbers of atypical moles.
Not everyone with these risk factors gets ocular cancers. However, if you have risk factors and eye cancer symptoms, it’s a good idea to discuss them with your health care provider.
Learn more about eye cancer:
Why come to MD Anderson for your eye cancer treatment?
At MD Anderson's Eye Clinic, highly skilled and renowned experts tailor your eye cancer care specifically for you. They work together in teams, collaborating at every step, to be sure you receive the most advanced therapies while making every effort to preserve ocular function and vision.
Most cancers affecting the eye and orbital area are rare and require treatment at centers familiar with the best treatment and diagnosis options. Our ophthalmologists have special training in surgical and medical treatment of all cancers of the eye and eye area. They work closely with radiation oncology, medical oncology, pathology and plastic surgery to deliver the best outcomes.
MD Anderson's ophthalmologists customize your treatment to include the latest technology and methods to treat eye cancer, which may include:
- Radiation therapy including proton therapy
- Targeted therapies
- Sentinel lymph node biopsy, which finds eye cancer that has spread to the lymph nodes
The latest ophthalmic reconstructive procedures to preserve function and appearance in the eye and facial area. If surgery is needed, it is done by some of the nation’s top ophthalmic surgeons and highly specialized eye reconstructive surgeons. And we are constantly researching new ways to diagnose and treat eye cancer. This means we are able to offer clinical trials of new therapies.
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