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Vitreoretinal Lymphoma

Lymphoma

Vitreoretinal lymphoma is a form of central nervous system (CNS) lymphoma that often is misdiagnosed as uveitis, but is the most common form of intraocular lymphoma.  A proper diagnosis of vitreoretinal lymphoma requires the histological identification of lymphoma typed cells within the vitreous of the globe or retina which can be a trick due to reactive lymphocytes as well as necrotic regions in the retina.  Contemporary approaches also require immunohistochemistry to reveal the monoclonality.

This rare form of lymphoma commonly has a poor prognosis and is often associated with a CNS lymphoma in aged populations.  That said, historically treatment, like other retinal cancers was commonly enucleation.  These days, chemotherapy combined with radiation therapy is more commonly used, though without good outcomes in many cases due to the difficulty delivering drugs into the eye from systemic administration.  New approaches are being explored through the direct injection of drugs into the globe and those efforts are ongoing.

Fundus photos were made by James Gilman of the Moran Eye Center.

Categories: Grand Rounds.

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