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«The Ocular Oncology service at Bascom Palmer Eye Institute is dedicated to helping you and your family cope effectively from the time of the initial ...»

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The Ocular Oncology service at Bascom Palmer Eye Institute is dedicated to

helping you and your family cope effectively from the time of the initial

diagnosis of eye cancer through treatment and the follow-up period.

Because eye tumors are so rare, patients may have to travel long distances to

seek expert opinion and treatment from an ophthalmologist who specializes in

the diagnosis and management of eye cancer. Patients travel from all regions

of the United States and from around the world because of Dr. Timothy Murray's years of experience in treating eye tumors, his international reputation, and his excellence in professional and compassionate patient care.

Bascom Palmer Eye Institute of the University of Miami School of Medicine is recognized throughout the world as a leading center for ophthalmic care, research and education. Its full-time faculty of internationally respected physicians and scientists encompasses every ophthalmic subspecialty and, through the years, has contributed fresh insight and direction to many of today's advanced ophthalmic techniques, diagnoses and treatments.

Consequently, the facility has been rated one of the best eye hospitals in the nation for 12 consecutive years based on a survey of board-certified ophthalmologists conducted by U.S. News & World Report.

This information is intended to help patients and their families better understand a diagnosis of eye cancer and prepare for treatment. The information presented is for educational purposes only and should not be relied upon as medical advice.

This information is also avilable on our website at www.eyecancermd.org.

Table of Contents Introduction

Anatomy of the Eye

Eye Tumors

Diagnosis

Treatment

Frequently Asked Questions..........p, 13-18 Research

Resources

Tips for Low Vision Users...............p. 22 Contact Information

© Copyright 2002-2003 - Timothy G. Murray, MD Information for patients with eye cancer & those who love them Most people have never heard of cancer of the eye. Ocular cancer is very rare; in North America, only about 2500 people will get the most common type of eye cancer, choroidal melanoma, each year.

Cancer is a scary diagnosis, and any threat to our vision is a cause of great anxiety. It is difficult to understand or remember complicated medical explanations. Our aim is to help you and your family learn how to cope effectively at the time of the initial diagnosis, during treatment and throughout the follow-up period.

This site would not be possible without the generous support of the:

–  –  –

The Chatlos Foundation, Inc.

ANATOMY OF THE EYE

In many ways, our eyes resemble an extraordinarily sensitive camera. Much like a camera lens, the lens of the eye can change shape to focus on near or distant objects. The lens projects images on the retina, a layer of light sensitive cells on the back of the eye, functions much like the film in a camera.

The eye is connected to the brain by the optic nerve, a bundle of over one million nerve fibers. The cornea (the clear portion of the front of the eye) bends light rays through the pupil to the lens. The pupil is the black opening in the iris. The iris, the colored ring of tissue, regulates the amount of light entering the eye by adjusting the size of the pupil. The eye also has fluids that bathe the various parts of the eye and help to maintain the correct pressure within the eye. The anterior chamber, in the front of the eye, is filled with aqueous humor, a watery fluid. The back portion of the eye is filled with a clear jelly like substance, called the vitreous humor.

The eye can be divided into three layers (or tissues). The white outer coat of the eye, the sclera, is similar to the shell of an egg. Along with the cornea, the sclera protects the eye from trauma and maintains the delicate structure of the internal eye tissues. The middle tissue layer, the choroids, is the blood supply to the eye wall and outer retinal structures. This tissue is responsible for transporting nutrients and oxygen to the outer sclera and inner retinal tissue.

The innermost layer of the eye is the retina, a thin translucent tissue composed of ten microscopic layers. The retina is responsible for translating light images into electrical impulses that can be recognized and processed by the brain. The macula is the area of the retina that is responsible for central vision. The center of the macula is called the fovea, and it is responsible for very sharp vision. The retina receives images of light and transmits them to the brain via the optic nerve. The brain interprets these messages as sight.

EYE TUMORS Choroidal Hemangioma A hemangioma is a tumor comprised of blood vessels and can grow within the choroid, the blood vessel layer beneath the retina. Choroidal hemangiomas are not cancers and never metastasize. However, if the hemangioma is located in the area of central vision of the eye it can leak fluid that causes a retinal detachment and visual function may be affected.

Many choroidal hemangiomas can be safely monitored by your eye doctor without the need of further treatment. Photographs can be used to document evidence of growth or leakage and the need for treatment. Treatment options may include photodynamic therapy, laser photocoagulation to decrease the amount of fluid leakage, or low doses of external beam radiation therapy.





Choroidal Melanoma Choroidal melanoma is the most common primary intraocular (occurring inside the eye) tumor in adults. It arises from the pigmented cells of the choroid of the eye and is not a tumor that started somewhere else and spread to the eye.

A choroidal melanoma is malignant, meaning that the cancer may metastasize and eventually spread to other parts of the body. Because choroidal melanoma is intraocular and not usually visible, patients with this disease often do not recognize its presence until the tumor grows to a size that impairs vision by obstruction, retinal detachment, hemorrhage, or other complication. Pain is unusual, except with large tumors. Periodic retinal examination through a dilated pupil is the best means of early detection.

Cutting out the tumor and leaving the rest of the eye is not routinely advised for this type of cancer. Opening the eye during surgery would allow the tumor cells to float around into the spaces around the eye, which could spread cancer cells to other parts of the eye. In addition, some studies have shown that up to 50% of choroidal melanomas invade the sclera. Therefore if the tumor is removed from the eye there is a high possibility that cancer cells will remain within the sclera. Lastly, many eyes do not tolerate this procedure and severe complications may occur such as detachments of the retina, hemorrhages, and recurrence of the tumor which may result in having to remove the eye anyway.

Treatment recommendations for choroidal melanoma usually are based on the size of the tumor. Small suspicious melanomas usually are closely watched for evidence of growth before treatment is recommended. Medium-size tumors may be treated with either radioactive plaque therapy or enucleation (removal) of the eye. The Collaborative Ocular Melanoma Study (COMS), supported by the National Eye Institute of the National Institutes of Health, has documented equal success rates for plaque radiation therapy or enucleation for preventing the spread of cancer. Large-size tumors usually are best treated by enucleation.

This is because the amount of radiation required to treat the tumor is too much for the eye to tolerate. The COMS study found no benefit to large-size tumor patients having radiation therapy prior to enucleation.

Choroidal Metastasis Malignant tumors from other parts of the body can spread in and around the eye. Metastatic cancers that appear in the eye usually come from a primary cancer of the breast in women and the lungs in men. Other, less common, sites of origin include the prostate, kidneys, thyroid, and the gastrointestinal tract.

Blood cell tumors (lymphomas and leukemia) also can spread to the eye. The care of patients with metastasis to the eye should be coordinated between the eye cancer specialist, medical oncologist, and radiation oncologist. Treatment options may include chemotherapy, external beam radiation therapy, or, more rarely, enucleation.

Choroidal Nevus Like a raised freckle on the skin, a nevus can occur inside the eye. And, like a skin nevus, a choroidal freckle can become malignant, so should be closely monitored. A choroidal nevus should be examined by an ophthalmologist every four to six months to check if the pigmentation or size of the nevus has changed. In most cases, the only treatment recommended is close observation and monitoring by an ocular oncologist.

Conjunctival Tumors Conjunctival tumors are malignant cancers that grow on the outer surface of the eye. The most common types of conjunctival tumors are squamous cell carcinoma, malignant melanoma, and lymphoma. Squamous cell carcinomas rarely metastasize, but can invade the area around the eye into the orbit and sinuses. Malignant melanomas can start as a nevus (freckle) or can arise as newly formed pigmentation. Lymphoma of the eye can be a sign of systemic lymphoma or be confined to the conjunctiva.

Both squamous cell carcinomas and malignant conjunctival melanomas should be removed. Most small conjunctival tumors can be photographed and followed for evidence of growth prior to treatment. Small tumors can be completely removed surgically. In other instances cryotherapy (freezing therapy) may be necessaryor chemotherapy eye drops may be used to treat the entire surface of the eye.

Eyelid Tumors Tumors of the eyelid may be benign cysts, inflammation, or malignant skin cancers. The most common type of eyelid cancer is basal cell carcinoma. Most basal cell carcinomas can be removed with surgery. If left untreated, these tumors can grow around the eye and into the orbit, sinuses and brain. A simple biopsy can determine if an eyelid tumor is malignant. Malignant tumors are completely removed and the eyelid is repaired using plastic surgery techniques.

Additional cryotherapy (freezing-therapy) and radiation therapy sometimes are required after surgery.

Iris Tumors Tumors can grow within and behind the iris. Though many iris tumors are cysts or a nevus, malignant melanomas can occur in this area. Most pigmented iris tumors do not grow. They are photographed and monitored with a special slit lamp and high frequency ultrasound to establish a baseline for future comparisons. When an iris tumor is documented to grow, treatment is recommended. Most small iris melanomas can be surgically removed. Radiation plaque therapy or enucleation may be considered for larger iris tumors.

Lymphoma/Leukemia Lymphoma tumors can appear in the eyelid tissue, tear ducts and the eye itself.

In most patients with large cell non-Hodgkin's lymphoma, the disease is confined to the eye and central nervous system. In these patients, symptoms appear in the eye an average of two years before they are seen elsewhere. The disease itself as well as treatment, which may include external beam radiation chemotherapy, or both (chemoradiation) to the central nervous system, can affect visual functioning., Melanocytoma This extremely slow-growing tumor usually is found on the surface of the optic disc. Almost all cases of melanocytoma are benign and malignant transformation is rare. It is probably present at birth and typically, there are no symptoms. Under clinical examination and fluorescein angiographic studies, melanocytoma appears as a deeply pigmented area located over the optic disc.

In the majority of cases, close observation is recommended and no treatment is required. If malignant transformation does occur, enucleation, may be considered.

Orbital Tumors Tumors and inflammations can occur behind the eye. These tumors often push the eye forward causing a bulging of the eye called proptosis. The most common causes of proptosis are thyroid eye disease and lymphoid tumors.

Other tumors include hemangiomas (blood vessel tumors), lachrymal (tear) gland tumors, and growths that extend from the sinuses into the orbit. Though CT scans, MRI's and ultrasounds help in determining the probable diagnosis, most orbital tumors are diagnosed by a biopsy.

When possible, orbital tumors are totally removed. If they cannot be removed or if removal will cause too much damage to other important structures around the eye, a piece of tumor may be removed and sent for evaluation. If a tumor cannot be removed during surgery, most orbital tumors can be treated with external beam radiation therapy. Certain rare orbital tumors may require removal of the eye and orbital contents. In certain cases, orbital radiotherapy may be used to treat any residual tumor.

DIAGNOSIS A retinal oncologist (an eye cancer specialist) can determine if you have an eye cancer by performing a complete clinical examination. The examination may include asking questions about your medical history, examining both eyes, looking into the eye at the tumor, doing an ultrasound examination, and obtaining specialized photographs. Biopsy, which is often indicated to diagnose tumors in other parts of the body, is rarely needed with eye cancer. Though occasionally necessary, biopsies are usually avoided because they require opening the eye which risks spreading of the tumor cells.

Eye Examination

Your ophthalmologist may be able to recognize an eye cancer by its appearance, including the degree of pigmentation of the tumor, its shape and location, and by other features. Unlike tumors in other parts of the body, many eye cancers, including choroidal melanoma, may be directly visible through the "window" provided by the pupil.

–  –  –

(Above) The ultrasound examination helps the ophthalmologist diagnose and measure ocular tumors.

Photography There are two types of special photographs ophthalmologists use to assist in diagnosis: fluorescein angiography and fundus photographs.

–  –  –

arm. As the dye passes through the blood vessels in the back of the eye, this allows for a view of the circulation of the retina and the layers beneath the retina, highlighting any abnormalities. Although fluorescein angiography is not diagnostic, it is useful to exclude other possible disorders.

–  –  –



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