Revised 14 June 2013
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 3 Central Nervous System (CNS) Lymphoma and 5 Paranasal sinus lymphoma)
2. Ocular Adnexal Lymphoma
Lymphoma involving the conjunctiva, lacrimal gland, extraocular muscles and other soft tissues within the orbit but 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 including bilateral involvement, symptomatic) using the currently recommended combination for advanced stage indolent lymphoma 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.
- Patients suspected of having intra-ocular lymphoma should be referred to an ophthalmologist with specific expertise in the assessment and management of intra-ocular lymphoma for diagnostic confirmation and for treatment planning that may include intra-ocular methotrexate and rituximab. The exact number of intra-ocular injections required will be tailored to the patient’s disease status and rate of response, but will typically include a minimum of 4 injections. Patients eligible to receive systemic high dose methotrexate should also be treated with 4 cycles of IV high dose methotrexate prophylactically to minimize the risk of recurrence within the brain. Systemic therapy with IV high dose methotrexate can be integrated between intra-ocular injections, although timing should be discussed with the ophthalmologist with specific expertise in the assessment and management of intra-ocular lymphoma. 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 recurrence after primary radiation, intra-ocular injection of methotrexate plus 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.