Primary vitreoretinal lymphoma: Single centre experience and review of literature

SUMMARY

BACKGROUND: Primary vitreoretinal lymphoma (PVRL) is a rare and difficult to diagnose lymphoma. The goal of this retrospective monocentric study was to obtain clinical characteristics, to evaluate median time to diagnosis, different treatment modalities and survival outcomes.

METHODS: PVRL cases were selected from the database of the University Hospitals Leuven (Belgium) from 1^st January 2012 until 1^st January 2021. A review of the available literature was performed.

RESULTS: We included eleven cases of PVRL with a median age of 76 years (Interquartile range (IQR): 68–81). Median time to diagnosis was seven months (Range: 3–16). Presenting symptoms were blurred vision (n=11, 100%) and floaters (n=3, 27%). Bilateral eye involvement was seen in 42% (n=5). Diagnosis was made by vitrectomy and immunocytochemistry in all cases. Histopathological diagnosis was diffuse large B-cell lymphoma in all cases. Flow cytometry was used in 55% (n=6) of patients to confirm diagnosis. Initial treatment included local therapy in all patients. A combination of local and systemic therapy was given to three patients (27%). Seven patients (64%) were diagnosed with CNS relapse. No systemic relapse was seen. Median progression-free survival (PFS) and overall survival (OS) were ten (IQR: 6–32) and 26 months (IQR: 12–37). Median PFS of patients treated with local versus combined therapy was 9.7 and 18 months, respectively. However, OS of patients with local versus combination therapy was 29 and 19 months, respectively.

CONCLUSION: We analysed the clinical characteristics of eleven patients with PVRL in our hospital. The majority of these cases will eventually progress to CNS lymphoma. We saw a prolonged PFS for patients treated with combination therapy in first-line, compared to local therapy alone. However, OS was longer in patients treated with local therapy only. Despite the small cohort, these results are comparable to previous literature. Based on larger retrospective studies we conclude that local therapy as first line treatment in PVRL results in similar OS rates with less systemic toxicity compared to combination therapy.

(BELG J HEMATOL 2022;13(6):228–235)