Abstract
Objectives To describe and compare clinical findings in different subtypes of biopsy-proven intraocular lymphomas (IOLs). Design Retrospective, observational case series. Methods The clinical and pathologic features in IOLs at the Hospital Clinic of Barcelona from 1995 to 2018 were retrospectively studied. Results Twenty-one patients, 12 men (57%), median age 60 (interquartile range, IQR: 18 years), and a median follow-up of 30 (IQR 60) months were included. Eleven patients had primary vitreo-retinal lymphoma (PVRL, 52%), 4 had primary uveal lymphoma (PUL, 19%), and 6 had systemic metastatic retinal lymphomas (SMRLs, 28%). Diffuse large B-cell lymphoma was the main IOL subset in PVRL (91%) and in SMRL (83%), whereas extranodal marginal zone lymphoma was the only type in PUL (100%). Survival rate was 44% in PVRL and 20% in SMRL at 5 years (p=0.047). One patient had flow cytometry of two different vitreous humour samples. With them, flow cytometry was performed in a total of 11 samples, yielding 7 positive samples. Conclusions and Relevance Even though PVRL is the most frequent IOL subtype, our findings suggest that PUL and SMRL should be considered as potential IOL causes. Overall survival was poor in PVRL and even shorter in SMRL patients.
Highlights
Intraocular lymphomas (IOLs) are lymphoid system neoplasia arising from either within the eye or outside the eye, with subsequent intraocular infiltration
Intraocular lymphomas are rare malignancies. ese are estimated to represent less than 1% of intraocular tumors and 1%-2% of extranodal lymphomas [2, 3]
In PVRL patients, we found a median age at presentation of 60 (IQR 12.5) years with a slight female preponderance (6/11, 55%)
Summary
Intraocular lymphomas (IOLs) are lymphoid system neoplasia arising from either within the eye (primary intraocular lymphoma, PIOL) or outside the eye, with subsequent intraocular infiltration (systemic metastatic retinal lymphoma, SMRL). Recommendations regarding nomenclature are primary or secondary intraocular involvement (systemic metastatic lymphoma) followed by initial intraocular affected tissue (primary vitreo-retinal lymphoma or PVRL and, primary uveal lymphoma or PUL). An increase in primary central nervous system lymphomas and PVRL, as its subset, has been recorded in the last decades [2, 4]. Reasons for PVRL rise are unknown, but a longer life expectancy, increasing use of immunosuppressive therapies, and improvement in diagnostic techniques could be possible explanations. Intraocular lymphomas are a major diagnostic challenge. Diagnosis is crucial in IOLs because they can be life-threatening diseases with a completely different management than uveitis [3, 4].
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