Clinicopathological Findings and Treatment Outcomes of Primary Vitreoretinal Large B-cell Lymphoma: A Case Series.
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Abstract
Objective: To report the clinicopathological findings, treatment modalities, and outcomes of primary vitreoretinal large B-cell lymphoma (PVR-LBCL).
Methods: This retrospective study recruited newly diagnosed PVR-LBCL between January 1, 2013, and December 31, 2022 at Thammasat University Hospital. We reviewed clinical presentations, diagnostic findings, treatment approaches, and outcomes.
Results: Nine PVR-LBCL cases (16 eyes) were elderly (median age 73 years, range 59-78 years), female predominant (n = 6, 67%), and bilateral involvement (n = 7, 78%). All cases presented with blurry vision, while only 3 (33%) reported floaters. Fundus examination revealed vitreous haze (n = 9, 100%), vitreous cells formed sheets (n = 8, 89%) and sub-retinal lesions (n = 5, 56%). The median time from symptom onset to pathologic diagnosis was 8 months (range 1-15 months). One patient transferred after diagnosis and did not have staging. The central nervous system (CNS) involvement was concurrent at diagnosis in 2 cases. The oldest patient did not receive treatment. The initial treatment modalities included ocular radiation (RT), intravitreal (IVT) methotrexate (MTX) or rituximab, whole brain RT, and systemic chemotherapy. The treatment complications were radiation-related cataract and maculopathy (n = 2), and keratopathy (n = 1). The outcomes were refractory disease with CNS progression (n = 1), complete remission (CR) then CNS relapse (n = 3), CR then ocular relapse (n = 1), and CR without event (n = 2).
Conclusion: PVR-LBCL presents a significant challenge in diagnosis and its management. Various modes of treatment were effective, but nearly half relapsed and died.
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