Ocular Lymphoma

This entry looks at ocular lymphoma, one of the subtypes, known of lymphoma. 'Lymphoma' is an umbrella term that loosely refers to several dozen independent categorical types and subtypes of cancers of the lymphatic system. Ocular lymphoma is a form of lymphoma that presents in the eye. It can either be intraocular lymphoma, or orbital and adnexal lymphoma(the adnexa are the appendages of the eye, such as the eyelids, soft tissue and muscles).

What is Ocular Lymphoma?

Ocular lymphoma is an extranodal B-cell non-Hodgkin's lymphoma (meaning it originates in tissue beyond the lymph nodes). It is an extranodal presentation of a marginal zone B-cell lymphoma.

Symptoms of Ocular Lymphoma

The primary symptom of ocular lymphoma is a deterioration of one's vision (blurred vision) or vitreous floaters. The disease commonly begins in one eye and spreads to include both.

Diagnosing Ocular Lymphoma

Ocular lymphoma is often mistaken as a case of either vitritis or uveitis. It is typically diagnosed following a procedure known as a vitrectomy which is a removal of certain cells from the orbital area. Upon pathologic review, a diagnosis is made, although because lymphomas are difficult to diagnose, it is strongly recommended that a certified blood pathologist become involved to perform the clinical staging of the disease, since it is quite common for ocular lymphoma to be diagnosed in later stages, and approximately 80 percent of cases eventually will include involvement of the central nervous system. A lumbar puncture and a bone marrow biopsy may also be performed to further determine staging.

Treatment and Prognosis of Ocular Lymphoma

Treatment of ocular lymphoma sometimes involves methotrexate and/or Rituxan-based chemotherapy, but the most common treatment involves low-dose external beam radiotherapy. However, because the disease is so rare, data on the efficacy of treatments is scarce and no consensus exists on the ideal treatment of this extranodal lymphoma.

Prognosis for those with ocular lymphoma depends largely upon whether the disease has spread to the central nervous system. If so, prognosis is considered poor. If not, treatment can be highly successful.

Sources

• Lymphoma Information Network, Primary ocular lymphoma
• Lymphomation.org: Extranodal lymphomas
• 2002 ASCO Meeting Abstract, Treatment of ocular lymphoma
• Eye Cancer Network, Intraocular lymphoma

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