Choroidal lymphoma: clinical features and association with systemic lymphoma

Purpose: To describe the clinical and histopathologic features of choroidal lymphoma (CL) and its association with systemic lymphoma.

Design: Observational case series.

Participants: Fifty-nine patients (73 eyes) with CL.

Methods: Retrospective chart review.

Main outcome measures: Clinical features, histopathology, and systemic lymphoma.

Results: Of 59 patients with CL, systemic lymphoma was absent in 41 (primary CL; 69%) and present in 18 (secondary CL; 31%). Of 18 patients with systemic lymphoma at presentation, 14 (78%) had known systemic lymphoma and 4 (22%) were diagnosed with systemic lymphoma shortly after presentation. The most common types of systemic lymphoma in patients with secondary CL were diffuse large cell lymphoma (n = 5, 28%), chronic lymphocytic leukemia/lymphoma (n = 4, 22%), multiple myeloma (n = 2, 11%), Waldenstrom's macroglobulinemia (n = 2, 11%), extranodal marginal zone lymphoma (n = 2, 11%), plasmablastic lymphoma (n = 1, 6%), and unspecified non-Hodgkin lymphoma (n = 2, 11%). Compared with patients with primary CL, patients with secondary CL had a shorter mean duration of ocular symptoms (5 vs. 17 months), had less often received steroids before referral (11% vs. 46%), and were more likely to have bilateral ocular involvement (33% vs. 20%). Eyes with secondary CL had a higher rate of poor vision (≤20/200) (46% vs. 12%), iris (20% vs. 4%) or ciliary body (30% vs. 8%) lymphoma, episcleral vascular congestion (40% vs. 16%), anterior chamber reaction (30% vs. 14%), hyphema (15% vs. 0%), vitreous cellular infiltration (30% vs. 4%), and severe media haziness (20% vs. 0%). Secondary CL was morphologically more high grade (50% diffuse large cell lymphoma) compared with primary CL (37% low-grade non-Hodgkin lymphoma, 27% extranodal marginal zone lymphoma). None of the 33 patients with primary CL and subsequent follow-up developed systemic lymphoma during a mean follow-up of 50 months (median, 35 months; range, 2-231 months).

Conclusions: Secondary CL is morphologically high grade and associated with more severe ocular findings. Patients with CL and no known systemic lymphoma at presentation should undergo systemic evaluation to rule out the possibility of undiagnosed concurrent systemic lymphoma. However, none of the patients with primary CL in our study had late development of systemic lymphoma.