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Concurrent Primary Vitreoretinal and Spinal Cord Lymphoma A Unique Entity

Isaac C. Ezon, MD1; Giulio Barteselli, MD1; Jay Rosenberg, MD2; William R. Freeman, MD1
[+] Author Affiliations
1Shiley Eye Center, Department of Ophthalmology, University of California, San Diego, La Jolla
2Scripps Memorial Hospital, La Jolla, California
JAMA Ophthalmol. 2014;132(7):902-904. doi:10.1001/jamaophthalmol.2014.419.
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Primary central nervous system lymphoma is a rare non-Hodgkin B-cell lymphoma limited to the central nervous system and distinct from systemic lymphoma with secondary central nervous system involvement. More than 15% of patients with primary central nervous system lymphoma develop vitreoretinal lymphoma involving the retina and/or vitreous, typically misdiagnosed for autoimmune, infectious, demyelinating, vascular, neoplastic, and paraneoplastic conditions.1 Primary spinal cord lymphoma is a subtype that affects the spinal cord to the exclusion of, or prior to, the brain. Only 35 cases have been reported in the literature,1 none associated with vitreoretinal lymphoma. Herein, we describe the first case of primary spinal cord lymphoma concurrent with primary vitreoretinal lymphoma to our knowledge.

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Figure 1.
Multimodal Imaging at Presentation

T1-weighted fast spin echo magnetic resonance image shows an enhancing lesion from C2 to T1 (arrowhead; this slice is C5 to T1) (A), and fluorescein angiography (B) and spectral-domain optical coherence tomography (C) demonstrate normal retina and retinal vasculature without leaking but with shadowing from vitreous debris.

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Figure 2.
Pathology and Follow-up Imaging of Vitreoretinal Lymphoma

A, Histopathologic analysis of the vitreous biopsy specimen reveals multiple atypical lymphocytes (hematoxylin-eosin, original magnification ×40). Lymphocytes stain positive for CD20 (B) and negative for CD3 (C) (original magnification ×40). D, Color fundus photograph of the right eye 1 month after biopsy shows multiple acute retinal whitish lesions in the middle retina (arrowheads). E, Spectral-domain optical coherence tomography 1 month after biopsy shows multiple hyperreflective retinal lesions extending from the outer plexiform layer through the ganglion cell layer (asterisks). N indicates nasal; S, superior; and T, temporal.

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