Revised 8 Nov. 2011

Lymphoma may involve the eye or surrounding structures in 4 distinct patterns. Each requires a specific approach to treatment.

1. Bony Orbit and Sinus Lymphoma

Lymphoma of the bony orbit or paranasal sinuses is usually diffuse large B cell lymphoma and should be managed as appropriate for the presenting stage (see 03 Central Nervous System (CNS) Lymphomas and 05 Paranasal sinus lymphoma.)

2. Ocular Adnexal Lymphoma

Lymphoma involving the conjunctiva, lacrimal gland, extraocular muscles and other soft tissues outside the globe but not the structures within the globe or the optic nerve is usually of indolent type and such lymphoma is highly responsive to radiation. Approximately 30% of such patients have advanced disease. Radio-immunotherapy offers the advantage of delivering potentially curative radiation while minimizing eye toxicity (reference Ann Oncology 2009;20:709-14). Patients should be offered radio-immunotheray (I¹³¹ tositumomab or Y⁹⁰ ibritumomab tiuxetan). If this is declined or there is a contraindication, unilateral external beam radiation (disease localized to one eye) or systemic chemotherapy (advanced stage, symptomatic) using the currently recommended combination for advanced stage indolent lymphoma (see 03 Central Nervous System (CNS) Lymphomas) should be offered.

3. Intra-ocular and Optic Nerve Lymphoma

Lymphoma involving the vitreous, retina or other structures within the optic globe or the optic nerve.

Lymphoma of the vitreous or retina or the optic nerve itself is usually of large cell type and is equivalent to CNS lymphoma, although it may pursue a more indolent course. Bilateral involvement is common. Evaluation and management should be the same as for primary CNS lymphoma with the following exceptions/additions:

• All patients should also be followed by an ophthalmologist experienced in the evaluation of lymphoma of the eye.
• For unilateral disease primary irradiation should be given to the entire involved globe. If concomitant brain involvement is present high dose methotrexate should be used, followed by radiation to the involved globe if response is less than complete. For bilateral involvement or recurrence after primary radiation, intra-vitreal injection of methotrexate plus intra-vitreal rituximab should be considered, in consultation with an ophthalmologist familiar with this technique.

4. Uveal lymphoma

Lymphoma involving the uveal structures (iris, ciliary body, lens, and choroid (vascular layer between the sclera and the retina)) is almost always marginal zone lymphoma and is typically unilateral. Radio-immunotherapy (see section 2 above) or external beam radiation is appropriate.