Intraocular Lymphoma

Description
Most patients diagnosed to have intraocular lymphoma have symptoms of vitreous floaters, a history of systemic lymphoma or have been treated for chronic uveitis. Any patients with vitreous cells, no history of recent intraocular surgery, and a non-painful eye should be suspected to have intraocular lymphoma.

Symptoms
Case Example: A 70 year old woman was noted to experience an acute deterioration of her vision due to vitreous cells, and a vitrectomy was performed. The diagnosis on pathology was large B-cell lymphoma. A complete metastatic survey (imaging studies of the brain, chest, abdomen), a lumbar puncture, and bone marrow biopsy were found to be negative.

Choroidal infiltration by lymphoma

B-scan ultrasound reveals an irregular retinal surface and variable internal reflectivity.

Diagnosis
The diagnosis of intraocular lymphoma is typically made by removal of cells (vitrectomy) from the eye with subsequent cytopathologic evaluation. Once the diagnosis is certain, a hematologist-oncologist should be consulted to perform an evaluation for systemic and central nervous system lymphoma (staging).

Treatments
Though chemotherapy can be used to treat the systemic disease, intraocular penetration can be poor leaving residual lymphoma in the eye. In these cases, external beam radiation therapy to the eyes and orbits will clear the intraocular disease. Fortunately, relatively low doses of radiation are typically required.

The literature consists of a multitude of case-reports and no evidence based comparative studies. In my experience, most patients are eventually treated with ocular irradiation. Others have had prophylactic whole-brain irradiation as well as combinations of local and systemic chemotherapy.

What is common to most of these reports is that, when the lymphoma is found in the brain, the prognosis is poor. Clearly, prospective randomized trials would be helpful to find the best treatment for patients with intraocular lymphoma.

Additional Info
Radiation Therapy

Radiation therapy has been the most common treatment for intraocular lymphoma. Both eyes are usually treated because approximately 80% of cases will either present as or go on to develop bilateral disease (within 8 years).

The prescription dose has been decreasing with less than 3,000 cGy being employed in many centers. The dose to the orbit is adjusted when whole-brain irradiation is required. With this in mind, remember to always obtain a complete neurological work-up to rule out central nervous system (CNS) involvement. Radiation will help acutely, but intraocular and CNS relapse are common.

Chemotherapy

Recent investigations have combined radiation therapy with systemic or intrathecal chemotherapy. Several studies suggest that this approach has prolonged survival. Chemotherapeutic agents have been given intravenously, intrathecally, and into the eye.

Related Links
Search for Scientific Articles on Intraocular Lymphoma

While this WWW site is intended to provide general information as a public service, the content is not intended to represent official policies and practices of the institution or to substitute for health care provided by a clinician. Content and design copyright © Paul T. Finger, MD 1998-2010. ALL RIGHTS RESERVED •115 East 61st Street • New York City, New York 10021 • (212) 832-8170