Isolated intra-ocular relapses of primary central nervous system lymphoma
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Background and aims: Relapses in primary central nervous system lymphoma (PCNSL) are usually cerebral and severe. Isolated intraocular relapses (IIOR) are much rarer and have not been specifically studied so far.Methods: We retrospectively selected patients treated within the French national expert network on PCNSL (LOC network). The inclusion criteria were: histologically proven PCNSL with at least a cerebral localization, immunocompetent status, 1st line treatment based on high-dose methotrexate and isolated IIOR subsequently.Results: 47 patients met the inclusion criteria (median age: 64.5 years (32.8-79.7), median Karnofsky Performance Status (KPS): 70 (40-100)). Initially, 13 patients had an ocular involvement, 16 had no ocular involvement and 18 had unknown status. The IIOR was the first relapse in 80% of cases. Median time from PCNSL diagnosis to IIOR was 14 months (3-51). Median KPS at IIOR was 80 (70-90), the affection was symptomatic in 95%. Decreased visual acuity was the prominent symptom. 76% of the patients received systemic chemotherapy (CT): ifosfamide-based CT (25%), methotrexate-based CT (25%), temozolomide (19%), in association with rituximab in 47%. 32% received a local treatment (intraocular CT or ocular radiotherapy) alone or in association with systemic CT. 31% subsequently received high-dose CT with autologous stem cells rescue). 60% of patients relapsed subsequently (35% in the brain, 62% in the eye) with a median PFS of 10,8 months. 5-year survival rate from relapse was 53.6% (0.28-1).Conclusion: IIOR of PCNSL seem to have a better prognosis than brain relapses. That might be explained by a better KPS allowing intensification chemotherapy for up to the third of patients.
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