Orbital lymphoma, also called lymphoma of the eye, is a type of eye cancer. It is a subtype of non-Hodgkin lymphoma (NHL), a blood cancer that affects the lymphocytes (white blood cells) throughout the immune system. According to the American Academy of Ophthalmology, orbital lymphoma is uncommon among people with NHL, making up only 1 percent to 10 percent of total cases. However, orbital lymphoma is considered the second most common form of eye cancer worldwide. The number of cases has tripled in the United States in the past 20 years. As more research is completed, doctors can better understand the disease and find the best treatment plans for people with orbital lymphoma.

What Is Orbital Lymphoma?

Orbital (eye) lymphoma is more commonly found in Asian and European countries. Eye cancer can happen at any age, but the risk increases as people get older. Orbital lymphoma is commonly found among older adults ages 50 to 70 years old. However, it can be seen in people as young as 15 years old.

Orbital lymphoma can be unilateral (affecting one eye) or bilateral (affecting both eyes). The signs and symptoms can vary depending on the site of the lesion (tumor) and the growth rate of the disease, also known as grading. Orbital lymphoma rarely affects the optic nerve and does not typically lead to visual loss or blindness.

Orbital lymphoma can be low grade (slow growth rate), intermediate grade (moderate growth rate), or high grade (fast growth rate).

There are three types of orbital lymphoma depending on the area of the eye that is affected:

• Intraocular lymphoma — Inside the eyeball
• Orbital lymphoma — Eye socket
• Ocular adnexal lymphoma — Eyelid, conjunctiva, and lacrimal (tear) gland

If orbital lymphoma is not detected or treated early in its disease, it can progress into various stages — ranging from stage 1 to stage 4 — and spread throughout the body.

What Causes Orbital Lymphoma?

The main factor that causes orbital lymphoma is immunosuppression, which means the immune system is not as strong as it could be. Immunosuppression can occur from increasing age, immunosuppressive drugs, or autoimmune disorders such as rheumatoid arthritis, lupus, anemia, and HIV. People who are living with HIV or AIDS tend to develop lymphoma more frequently than those living with any other autoimmune disorder.

Recently, clinical studies have found that certain viruses, such as Chlamydia psittaci and Helicobacter pylori, may also contribute to the development of eye lymphoma. These viruses have been found in infected birds and household pets.

Orbital lymphoma tends to be seen more often in women.

What Are the Symptoms of Orbital Lymphoma?

Because orbital lymphoma is a subtype of NHL, some of their symptoms overlap. However, there are key differences in orbital lymphoma symptoms. The most common symptom of orbital lymphoma is the bulging of the eyeball outside the socket, also known as proptosis.

Other symptoms include:

• Blurred vision
• Floaters
• Limited eye movement
• Swelling (edema) of the eyes and eyelids
• Eye redness
• Eye inflammation (chemosis)
• Eye pain
• Double vision (diplopia)
• Enlarged lacrimal gland

Symptoms can last from a few days up to 18 months depending on whether the lymphoma is low or high grade, how fast it grows, what stage the cancer is, and what area of the eye is affected. High-grade orbital lymphoma is more aggressive, and symptoms are typically more noticeable. Talk with your doctor or ophthalmologist right away if you begin to experience any changes in your vision or if new symptoms arise.

Read more about the symptoms of NHL.

How Is Orbital Lymphoma Diagnosed?

Orbital lymphoma is typically diagnosed through various exams and tests. First, an eye exam or dilated fundus exam (dilating the pupil to see inside the eye better) will help the ophthalmologist assess eye movement and overall vision.

Second, a physical exam that includes a full medical history is important, especially if the person is already diagnosed with NHL or an existing autoimmune disorder. If the orbital lymphoma is located in the front of the eye (eyelid, conjunctiva, and lacrimal gland), the doctor may find that the eye appears pink or red, and the tumor may be visible and firm to the touch. However, if the orbital lymphoma is inside the eyeball or socket, an endoscopy procedure through the nasal cavity and sinuses may be needed to locate the tumor.

After a diagnosis of orbital lymphoma, imaging needs to be performed to specify the stage and grade and to rule out whether cancer has spread to other organs of the body. Imaging tests include computed tomography and magnetic resonance imaging of the eye and other areas.

Last, after locating the lymphoma, a biopsy — a small sample of eye tissue, tumor, or bone marrow — is typically collected to confirm an orbital or systemic lymphoma diagnosis and classify its subtype.

Your doctor may also order blood tests, like a complete blood count, to check the number of platelets and white and red blood cells. Liver and kidney (renal) function tests may also be done to check that organs are functioning normally.

How Is Orbital Lymphoma Treated?

There are many treatment options used alone or in combination that have been shown to help treat orbital lymphoma.

Every person’s treatment plan will vary depending on the subtype, grade, and stage of the tumor; the function of the eye; and other factors. Work together with your cancer care team to create a personalized treatment and follow-up plan and to discuss potential side effects.

Radiation Therapy

Radiation therapy is generally considered the main therapy for treating orbital lymphoma. Essentially, radiation therapy uses beams — high doses of X-rays or other forms of energy — to shrink tumors and kill cancer cells. Because orbital lymphoma is usually localized, radiation can easily pinpoint and shrink the tumor, which allows for a low rate of recurrence. However, if the tumor returns, it can sometimes be treated with repeated radiation or surgery.

Surgery

Surgery is typically used during the diagnostic process for collecting a biopsy of the tumor. However, it can also be done to remove the tumor itself. It is important to note that surgery is not the first option due to the risk of injury to the eye’s structures.

Chemotherapy

If a person is diagnosed with stage 2, 3, or 4 disease, chemotherapy will be taken into consideration. Currently, chemotherapy is only used for individuals with diffuse large B-cell lymphoma, a type of NHL. Chemotherapy is typically given after surgery or combined with radiation therapy.

Immunotherapy

Immunotherapy is a form of cancer treatment that allows the body to use its immune system to fight and attack cancer cells. There are many methods for immunotherapy. Monoclonal antibodies, such as Rituxan (rituximab) and Leukeran (chlorambucil), have shown promising outcomes in treating orbital lymphoma. These antibodies target and bind to specific cancer cells, allowing the immune system to identify and destroy those cells over time.

Other Treatments

Antibiotic therapy may be used to treat orbital lymphoma that was manifested by the virus Chlamydia psittaci. In one study, 48 percent of participants given oral doxycycline showed improvements in their orbital lymphoma. However, this clinical study had a small sample size, and further studies are needed on a larger scale.

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