What Is Eye Lymphoma?

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Eye lymphoma, also called ocular lymphoma, is a type of eye cancer. It is the most common type of malignant eye tumor. The condition may cause eye redness or decreased vision, and it can advance to result in eye damage and blindness. While anyone can develop lymphoma of the eye, having an immune deficiency is a risk factor.

Definitive diagnosis relies on a biopsy of the eye, which is a delicate procedure. Treatment to prevent progression includes surgery, chemotherapy, and radiation therapy.

Types of Ocular Lymphoma

Primary intraocular lymphoma (PIOL), also called vitreoretinal lymphoma, is the most common type of ocular lymphoma, followed by ocular adnexal lymphoma and uveal lymphoma.

PIOL/Vitreoretinal Lymphoma

This ocular lymphoma affects the retina (the area in the back of the eye that senses light and converts it to signals for the brain to interpret), the vitreous of the eye (a jellylike substance that fills most of the eyeball), or the optic nerve (the nerve that detects visual input).

PIOL is considered a central nervous system lymphoma because it arises from structures in the eye that are part of the nervous system.

This tumor is usually aggressive and often spreads to the brain.

Uveal lymphoma

This tumor involves the uvea, which is the part of the eye that's directly beneath the sclera (the white of the eye). Uveal lymphoma can affect the choroid (blood vessels of the eye), the iris (the colored part around the pupil), or the ciliary body (muscles and other structures around the iris).

This type of lymphoma is often designated as low grade because it doesn't have a tendency to be very aggressive.

Ocular Adnexal Lymphoma

This type of lymphoma begins in structures that are near the eye but are outside the eye itself. Ocular adnexal lymphoma involves the orbit (eye socket), the conjunctiva (lining of the eye), or the lacrimal gland (a structure that makes tear ducts), or the eyelid.

There are other types of eye cancer that aren't lymphomas, such as retinoblastoma and eye melanoma. Non-lymphoma eye cancers have different symptoms, causes, prognosis, and treatments than ocular lymphoma.

Ocular Lymphoma Symptoms

The early symptoms of ocular lymphoma are fairly non-specific and can be the same as signs of eye infections, degenerative eye diseases, or inflammation of the eye. The most common early symptoms involve subtle vision changes and mild to moderate eye discomfort.

Eye lymphomas can start off in one eye, but they generally eventually affect both eyes.

Symptoms can include:

  • Light sensitivity
  • Diminished or blurry vision
  • Seeing spots or floaters
  • Eye redness
  • Dry eyes
  • Eye irritation
  • Yellowish eye discoloration
  • An appearance of lumpiness in the eye
  • A bump in or around the eye
  • Eyes that appear uneven
  • Swollen eyelid

You might experience the same effects in both eyes, but they can be more noticeable in one eye than the other. It's also possible for each eye to have different effects of the disease.

Advanced Tumors

Ocular lymphomas can enlarge, causing more noticeable effects and complications. Large tumors may cause symptoms due to pressure on the eyeball.

An advanced eye tumor may:

  • Restrict eye movement or cause double vision
  • Push on the eye, making it appear to be enlarged or pushed forward
  • Compress the optic nerve and cause loss of vision
  • Produce inflammation and optic neuropathy
  • Invade structures in the face
  • Spread to the brain and cause weakness, numbness, dizziness, or a variety of other effects

Causes

Lymphoma is a type of cancer that's characterized by an abnormal and harmful proliferation of lymphoid tissue, which is composed of immune cells and proteins. Eye lymphoma is caused by the proliferation of either B cells (most commonly) or T cells, which are two types of immune cells.

Ocular lymphoma can be a primary tumor, originating in the eye and potentially invading nearby structures. It can also be secondary, spreading to the eye from lymphoma that started somewhere else in the body.

Lymphoma, including lymphoma of the eye, occurs when genetic changes in DNA alter the behavior of immune cells. These mutations cause the cells to become dysfunctional in terms of their role in immunity. The alterations also cause the cells to multiply and spread more than usual.

Generally, eye lymphomas form a tumor mass in or around the eye.

Risk Factors

Eye lymphomas can develop without any risk factors, but immune diseases, immune deficiency, or a history of chemotherapy or radiation can increase the chances of developing ocular lymphoma.

These risk factors can lead to the aforementioned mutations. If a cell that's had a mutation survives, it can lead to cancer.

Diagnosis

A tumor in the eye can be diagnosed based on a non-invasive eye examination, imaging studies, and a biopsy of the tumor in the eye. Your medical assessment would also include an evaluation of whether the lymphoma is present elsewhere in your body.

Classification of your tumor involves a determination of the tumor type, grade, and stage.

  • Type: This is a description of the cell type and it identifies the cell of origin. For example, a primary eye lymphoma may be a B-cell lymphoma or T-cell lymphoma. This can be determined by visual microscopic examination of the tumor sample that's obtained with a biopsy. Additionally, cytometry studies and polymerase chain reaction (PCR) studies are laboratory methods that can help identify cell types based on their molecular characteristics.
  • Grade: The grade defines the aggressiveness of the tumor. Generally, cells from a biopsy are visually examined with a microscope to determine their potential for malignancy.
  • Stage: The stage of a tumor is a reflection of how much it has enlarged and the extent to which it has spread. Diagnostic tests, such as a magnetic resonance imaging (MRI) test of the brain or face, are needed to determine the stage of eye lymphoma.

Lymphoma can be either Hodgkin's or non-Hodgkin's type. Most ocular lymphomas are classified as non-Hodgkin’s tumors. This differentiation is based on certain characteristics that are seen on a biopsy evaluation. Generally, non-Hodgkin’s tumors are more aggressive than Hodgkin's tumors.

Biopsy

A biopsy for the diagnosis of an eye tumor is a surgical procedure in which a sample of tissue is taken from the eye. The surgery requires minimal excision of tissue to avoid harming the eye or causing any impairment of vision.

Generally, your sample will be sent to a laboratory right away, but full results might not be available for several days.

Your doctor will give you instructions regarding eye care after your biopsy.

Treatment

Ocular lymphomas don't improve on their own. Because of the nonspecific nature of primary intraocular lymphoma presentation, the condition is a diagnostic challenge. The prognosis for this condition remains poor with a five-year mortality of less than 25%.

Chemotherapy is considered systemic therapy, and it treats the primary tumor, as well as metastatic lesions. If there is central nervous system involvement, systemic treatment is recommended.

External beam radiation therapy (EBRT) is considered a local treatment that can be directed to target the tumor in the eye specifically, or may also be used for treating brain metastasis to make the tumor shrink.

Each tumor is treated based on its classification:

  • PIOL is treated with methotrexate-based chemotherapy and EBRT. Recurrence is fairly common after treatment, but it does improve survival and helps maintain vision.
  • Uveal lymphoma is usually treated with EBRT and Rituximab, a monoclonal antibody therapy, with improved outcomes.
  • Ocular adnexal tumors are treated with EBRT and Rituximab with improved outcomes.

Side effects of treatment with external beam radiation can include:

Many of these side effects can cause a spectrum anywhere from discomfort to total loss of vision. However, the side effects of ocular lymphoma treatment are not as harmful as untreated ocular lymphoma.

A Word From Verywell

Eye lymphomas are very rare. These tumors can cause many of the same symptoms as more common eye diseases. This is why it is important to have regularly scheduled eye examinations and to see your doctor if you develop any eye symptoms or problems related to your vision. Early diagnosis increases the chances of a good outcome.

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