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Review
. 2004 Sep-Oct;11(5):285-95.
doi: 10.1177/107327480401100502.

Intraocular lymphoma: update on diagnosis and management

Chi-Chao Chan  1 , Dana J Wallace
Affiliations
Review

Intraocular lymphoma: update on diagnosis and management

Chi-Chao Chan et al. Cancer Control. 2004 Sep-Oct.

Abstract

Background: Primary intraocular lymphoma (PIOL) is a subset of primary central nervous system lymphoma (PCNSL) in which lymphoma cells initially invade the retina, vitreous, or optic nerve head, with or without concomitant CNS involvement. The incidence of this previously rare condition has increased dramatically. Given its nonspecific presentation and aggressive course, PIOL provides a diagnostic and therapeutic challenge.

Methods: We review the current strategies for diagnosis and treatment of PIOL and present our own experience with PIOL.

Results: Recent developments in the diagnosis of PIOL include immunohistochemistry, flow cytometry, cytokine evaluation, and molecular analysis. However, definitive diagnosis still requires harvesting of tissue for histopathology. Optimal treatment for PIOL remains unclear. Initial therapeutic regimens should include methotrexate-based chemotherapy and radiotherapy to the brain and eye. In addition, promising results have been seen with intravitreal methotrexate and autologous stem cell transplantation for recurrent and refractory disease.

Conclusions: Efforts to further determine the immunophenotype and molecular characteristics of PIOL will continue to assist in the diagnosis of PIOL. Future studies are required to determine the role of radiotherapy and optimal local and systemic chemotherapeutic regimens.

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Figures

Fig 1
Fig 1
Fundus photograph of a patient with PIOL showing yellow subretinal infiltrates that appear slightly hazy due to an overlying vitreitis.
Fig 2
Fig 2
Fluorescein angiogram of a patient with PIOL showing blockage that corresponds to tumor infiltrates.
Fig 3
Fig 3
Brain magnetic resonance imaging of a patient with PIOL showing multiple contrast-enhancing lesions (arrows).
Fig 4
Fig 4
Typical cytology of PIOL cells from the vitreous showing several atypical lymphoid cells with basophilic cytoplasm and large prominent irregular nuclei.
Fig 5
Fig 5
Histological examination of the eye showing tumor cells located between Bruch’s membrane and the RPE (arrow). The tumor cells have penetrated the RPE and are infiltrating towards the retina (R, retina; C, choroids; hematoxylin and eosin, × 400).
Fig 6
Fig 6
Algorithm for the diagnosis of PIOL.
See this image and copyright information in PMC

References

    1. Hochberg FH, Miller DC. Primary central nervous system lymphoma. J Neurosurg. 1988;68:835–853. - PubMed
    1. Chan CC, Buggage RR, Nussenblatt RB. Intraocular Lymphoma. Curr Opin Ophthalmol. 2002;13:411–418. - PubMed
    1. Hormigo A, DeAngelis LM. Primary ocular lymphoma: clinical features, diagnosis, and treatment. Clin Lymphoma. 2003;4:22–29. - PubMed
    1. Appen RE. Posterior uveitis and primary cerebral reticulum cell sarcoma. Arch Ophthalmol. 1975;93:123–124. - PubMed
    1. Volcker HE, Naumann GO. “Primary” reticulum cell sarcoma of the retina. Dev Ophthalmol. 1981;2:114–120. - PubMed

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