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What Is Eye Lymphoma?

By Lauren R. Rosecan, M.D., Ph.D., F.A.C.S. –

What Is Eye LymphomaIn contrast to other systemic lymphomas, which usually involve the retina and iris (the colored portion of the eye), primary intraocular lymphoma (PIOL) involves the retina, vitreous and optic nerve. Eighty percent of cases involve both eyes; many people with PIOL will develop lymphoma within the part of the brain called the cerebrum.

The two main types of lymphoma are Hodgkin’s disease and non-Hodgkin’s lymphoma. Primary intraocular lymphoma is almost always a non-Hodgkin’s B cell lymphoma. Most people with primary intraocular lymphoma are elderly or have immune system diseases such as AIDS (acquired immunodeficiency syndrome). Primary intraocular lymphoma often occurs with lymphoma of the brain, called primary central nervous system lymphoma (PCNSL).

Eye Lymphoma Causes
People who have a suppressed immune system, whether from birth or acquired over time, are at risk for development of primary intraocular lymphoma (PIOL).

Genetics is one factor that may contribute to certain cells becoming cancerous. DNA is the chemical in each of our cells that makes up our genes, the instructions for how our cells function. Some people with eye cancer may have inherited changes in their DNA that increase their risk for developing eye cancer. Generally, though, these DNA changes happen over a lifetime rather than being inherited before birth.

Eye Lymphoma Symptoms
The most common symptoms for eye lymphoma include blurred vision, decrease or loss of vision, and floaters in vision.

Redness or swelling in the eye and sensitivity to light may also occur. Eye pain is uncommon.

Lymphoma of the eye usually affects both eyes, though symptoms may be more obvious in one eye than the other.

Who Is at Risk for Eye Lymphoma?
This includes people who:

• Have rheumatoid arthritis or other autoimmune diseases;

• Have rare medical conditions that affect their immune system;

• Have AIDS (acquired immunodeficiency syndrome);

• Are taking certain medications to prevent rejection of a transplanted organ.

People without any of these risk factors can still develop an eye lymphoma.

Most people with eye lymphoma are elderly. Eye lymphoma often occurs with lymphoma of the brain, called primary central nervous system lymphoma (PCNSL).

Eye Lymphoma Diagnosis
To diagnose eye cancer, an ophthalmologist (Eye M.D.) will ask about symptoms and check vision and eye movements. During the eye exam, the Eye M.D. will use an ophthalmoscope—an instrument with a light and a small magnifying lens—to get a good look inside the eye.

Certain imaging tests may be done, including:
• Ultrasound: helps the Eye M.D. assess the tumor.
• MRI scan: helps the doctor to see the eye better and look for lymphoma in the brain or spinal cord, where this cancer commonly spreads.
• Positron emission tomography (PET) scan: helps give the Eye M.D. a better idea of whether the cancer has spread to lymph nodes or other parts of the body. In this imaging test, a radiolabeled substance (usually a type of sugar) is injected into the blood. Since the body’s cancer cells often absorb larger amounts of sugar than other cells, a special camera can then take pictures of the areas where the signal shows up. This imaging technique helps identify whether abnormal areas seen on other tests (such as MRIs) may be cancer.

To confirm an eye lymphoma diagnosis, a biopsy is usually needed, where a sample of tissue or cells is removed and examined in a lab under a microscope and tested. The person undergoing a biopsy may be sedated and get local anesthesia (numbing medicine) or may get general anesthesia (to be put in a deep sleep).

For a biopsy, the Eye M.D. usually does a procedure called a vitrectomy. In this procedure, he or she will insert tiny instruments into the eye, cut the vitreous—
the clear, jelly-like substance that fills most of the eye—then suck a sample of it out. This sample is then sent to the lab for examination and testing.

Eye Lymphoma Treatment
Chemotherapy is the use of medication to kill cancer cells. Chemotherapy can be given directly in the eye, through a vein, or directly into the cerebrospinal fluid. If the cancer does not respond to treatment, or if it comes back, the doctor may recommend high-dose chemotherapy followed by a stem cell transplant.

Radiation therapy uses high-energy radiation to shrink tumors and kill cancer cells. For treating lymphoma of the eye, it may be directed only at the affected eye or at both eyes, depending upon where the cancer is. Radiation therapy may also be directed to the brain and spinal cord to help prevent the cancer from spreading there or to help destroy undetected cancer cells that could be there.

Lauren R. Rosecan
M.D., Ph.D., F.A.C.S.
The Retina Institute of Florida with four offices
conveniently located in Palm Beach and Martin Counties.

Toll Free Phone Number:  1-800-445-8898
Or 561-832-4411

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