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04 Eye Lymphoma

Lymphoma may involve the eye or surrounding structures in 3 distinct patterns. Each requires a specific approach to treatment.
1. Orbital or Peri-orbital Lymphoma
Lymphoma of the bony orbit or the soft tissues in and around the orbit but outside of the globe and optic nerve should be managed as indicated in section 3.3.2, as appropriate for the type and stage of the lymphoma and the age of the patient. Approximately 40% of such patients have advanced disease discovered when carefully staged.
2. Conjunctival Lymphoma
Lymphoma involving the conjunctiva but not the structures within the globe or the optic nerve is usually of low grade type and should be treated with irradiation. Doses, fields and shielding specifically modified for treatment of the eye are necessary to minimize long-term complications such as xerophthalmia or cataract formation.
3. Intra-ocular and Optic Nerve Lymphoma
a) 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.
Since methotrexate does not penetrate the vitreous well, primary irradiation should be given to the entire involved globe. The timing of the radiation depends on whether involvement is isolated to the globe (radiation first followed by high dose methotrexate) or concomitant with brain involvement (methotrexate first followed by radiation to the involved globe (a)).
b) Lymphoma involving the uveal structures (usually the choroid). This rare presentation of lymphoma is usually of low grade type and is best managed as such with treatment appropriate for stage and local extent of disease.

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Unofficial document if printed. Please refer to the following web address for up-to-date information: http://www.bccancer.bc.ca/HPI/CancerManagementGuidelines/Lymphoma/HD/04EyeLymphoma.htm