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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.
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.
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’s 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’s 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
Many times, eye cancer does not have symptoms in the early stages. When it does have signs, they vary from person to person.
Common eye cancer symptoms may include:
- Loss of vision
- Blurry vision
- Flashes and floaters (an object in the field of vision)
- Pigmented (dark) area on the conjunctiva
- A small, firm, raised lump with a lesion in the center on the eyelid
- Ulceration of the eyelid
- Loss of eyelashes
- Brown or pigmented lesion on the eyelid
- Sty that does not heal
- Growing dark spot on iris
- Change in pupil shape or size
- Change of eyeball position or the way eye moves
- Bulging of eye
- Double vision
- Orbital or ocular pain
- Excessive tearing, bloody tears
These symptoms do not always mean you have ocular cancer. However, it is important to discuss any eye cancer symptoms with your doctor, since they may signal other health problems.
MD Anderson’s experts use the most advanced and accurate technology and techniques to pinpoint the extent and type of eye cancer. This can make a vital difference in the success of your treatment and recovery.
Our leading-edge diagnostic tools and methods include:
- Sentinel lymph node biopsy to detect early microscopic metastasis
- Ultrasound biomicroscopy to diagnose intraocular tumors
- Confocal biomicroscopy
- Optical coherence tomography (OCT) for more accurate diagnosis of conjunctival cancers
Eye cancer diagnostic tests
Usually an exam by an ophthalmologist or other eye care provider can diagnose ocular cancer. Other tests may include:
- Dilated retinal exam to help diagnose intraocular tumors
- Ultrasound of the eye for intraocular tumors
- Careful inspection of the outside of the eye and eye movements for orbital, eyelid and conjunctival tumors
- Imaging tests, such as:
- CT or CAT (computed axial tomography) scans
- MRI (magnetic resonance imaging) scans
- Surgical biopsy to confirm cancers of the orbit, eyelid or conjunctiva
If you are diagnosed with eye cancer, your doctor will determine the stage of the disease. Staging is a way of classifying how much disease is in the body and where it has spread when it is diagnosed. This information helps your doctor plan the best type of treatment for you.
Eye cancer stages
Once the staging classification is determined, it stays the same even if treatment is successful or the cancer spreads.
The most common staging system used for ocular cancers is the one set up and approved by the American Joint Committee on Cancer (AJCC). The TNM system of the AJCC is based on three key pieces of information:
- T describes the size of the primary tumor and/or whether it has invaded nearby structures
- N describes whether the cancer has spread to nearby (regional) lymph nodes
- M indicates whether the cancer has metastasized (spread) to other parts of the body (The most common site of eye melanoma spread is the liver)
Numbers or letters appear after T, N and M to provide more details about each of these factors:
- The numbers 0 through 4 indicate increasing severity
- The letter X means "cannot be assessed" because the information is not available
Treatment of ocular cancers is highly specialized. At MD Anderson, we personalize your care to include the most-advanced therapies with the least impact on your body. Our goals are to provide successful treatment while preserving your eye and vision when possible and restore your appearance after treatment.
If you are diagnosed with an eye cancer, your doctor will discuss the best options to treat it. This depends on several factors, including:
- The type of cancer
- Location and size of cancer
- If cancer has spread (metastasized)
- Your age and health
At MD Anderson, your eye cancer treatment is customized to your particular needs. One or more of the following therapies may be recommended to treat the cancer or help relieve symptoms.
One of the following surgeries may be used to treat eye cancer.
Eye-preserving surgery: This is often possible for eyelid cancers, conjunctival cancers and orbital cancers. Our highly specialized eye surgeons use the latest surgical techniques to remove the cancer while making every effort to preserve the function and aesthetic appearance of your eye, eyelid and facial area.
Iridectomy: The iris (colored part of the eye) is removed.
Iridotrabeculectomy: The iris and a small part of the outer section of the eyeball are removed.
Eye reconstructive surgery: Our ophthalmic plastic, or oculoplastic, surgeons often can restore function, preserve vision and improve cosmetic appearance after surgical removal of cancer of the eyelid, conjunctiva or orbit.
Enucleation: To treat some types of eye cancer, the entire eye has to be removed. This is done only in advanced cases and in some instances for intraocular tumors such as uveal melanoma or retinoblastoma.
Orbital exenteration: This surgery entails the removal of the eye, eyelids, surrounding skin, orbital muscles, fat and nerves. It is sometimes needed for aggressive cancers of the orbit or sinuses, or advanced or recurrent cancers of the eyelid or conjunctiva.
A laser uses an intense, focused beam of light to destroy eye cancer tissue. This is used sometimes for treatment of uveal melanoma.
MD Anderson provides the most advanced radiation treatments for eye cancer, including:
- Brachytherapy: Tiny radioactive seeds are placed in the body close to the tumor. This technique is commonly used for treatment of uveal melanoma.
- 3D-conformal radiation therapy: Several radiation beams are given in the exact shape of the tumor. This technique can be used for certain types of orbital tumors.
- Intensity-modulated radiotherapy (IMRT): Treatment is tailored to the specific shape of the tumor. This technique is used for treatment of orbital and eyelid cancers.
- Gamma Knife stereotactic radiation surgery: Pinpoint beams of radiation are given from hundreds of angles. This technique is used for certain types of orbital and skull base tumors.
Proton therapy is used for tumors of the orbit such as orbital rhabdomyosarcoma, optic nerve and orbital meningiomas, and certain types of cancers of the lacrimal sac/duct.
Cryotherapy eye cancer treatment
Extreme cold is used to destroy cancer cells. This treatment is used with surgery for some conjunctival cancers such as conjunctival squamous cell carcinoma and conjunctival melanoma.
These special antibodies work with the body’s immune system to help it fight the eye cancer. Our ophthalmologists have conducted clinical trials of monoclonal antibodies for treatment of orbital and conjunctival lymphoma.
Our ophthalmologists have experience with topical chemotherapy in the form of eye drops, which may be used along with surgery for certain types of conjunctival carcinoma and conjunctival melanoma.
Chemotherapy may be given for retinoblastoma and certain types of orbital lymphoma. MD Anderson ophthalmologists have worked with medical oncologists to develop newer forms of oral chemotherapy that are targeted against certain forms of basal cell carcinoma and squamous cell carcinoma as an alternative to surgery.
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.
Some 23 years ago, I was 19 and getting ready to start college at Florida State University. But after experiencing blurry vision that could not be corrected with glasses or contacts, I was diagnosed with ocular melanoma, a very rare cancer in the eye. With my whole life ahead of me, I was determined to beat it. After treatment, which involved radiation and the loss of sight in my right eye, I got a clean bill of health -- just in time to buy my course books and head to college.
I closed that door and never looked back. I became a speech pathologist and an opera singer, got married and had two beautiful children. We live in Jacksonville, Florida. I’ve lived a healthy and happy life, never forgetting how precious life is. I sang for my church for years and performed in many venues around Florida.
I also was running about two or three marathons a year and competed in numerous other competitive races, including an ultra-marathon, two half-IRONMAN triathlons and countless 5K and 10K races -- after singing the national anthem, of course. After finishing the Boston Marathon last April, I was on top of the world. Next up: an IRONMAN triathlon.
My ocular melanoma recurrence
A routine doctor’s visit in May 2015 turned my life upside down. After 23 years of being cancer-free, I learned that the melanoma had spread to my liver. I was classified as stage IV. Whereas I’d felt that I could conquer the world at 19, I now felt different. As a wife and a mother, I had much more to lose. Being in health care, I knew that this was bad. Google searches didn’t help.
I was overwhelmed by the clock ticking on my life. I freaked out. I explored local treatment options, which sounded invasive and scary. My sister, Rebecca, urged me to get a second opinion at MD Anderson in Houston. She and my husband did research for me while I was crippled with fear of the unknown.
I made the trip to Houston. After only 20 minutes with Sapna Patel, M.D., and her team, I knew I had to be treated here. I’ve been on an immunotherapy clinical trial since August, with a combination infusion of ipilimumab and nivolumab every three weeks. I’m now down to just nivolumab once every two weeks.
After six months, here’s what I’ve learned:
- Volume matters. With the number of cases that come through MD Anderson, my doctors have experience treating rare cancers like mine and are able to come up with personalized treatments that worked for me.
- Family and friends matter. They often accompany me on my bimonthly visits to MD Anderson. It helps to have someone beside me asking questions and taking notes. Back home they make meals, run with me when I’m able, and listen when I need to vent. Investing in my friendships throughout the years has definitely paid off.
- Happiness matters. I bought yellow note cards that say “Cancer Sucks,” as well as a yellow purse. (My favorite color is yellow.) These small things made me hopeful.
- Setting goals matters. I made a deal with my husband that when I beat cancer, we’re taking the kids to Paris and I'm running a marathon somewhere fabulous. In the meantime, my husband and children have met my medical team and visited my aunt and uncle’s horse farm, which is about 90 minutes outside Houston. Having a local support system has been amazing.
I’m delighted to report that my tumors are shrinking, and my liver enzymes are back to normal. Dr. Patel calls me a “responder,” which is a much better label than “stage IV.”
My research nurse, Maria Jung, says she's going to cheer me on at my next marathon. I’m still in treatment, but I’m more hopeful than ever that I’ll make it to Paris and have Maria there cheering me on at my next marathon.