Abstract
Purpose :
To analyze a large cohort of individuals with primary central nervous system lymphoma (PCNSL) and vitreoretinal lymphoma (VRL) from a tertiary care center.
Methods :
A retrospective review of all patients with PCNSL and VRL at Massachusetts Eye and Ear from January 01, 2004 to April 1, 2020 was performed. Demographics, affected site at diagnosis (eye, central nervous system (CNS), or both), lymphoma subtype, MYD88 mutational status, local treatment type (intravitreal chemotherapy vs radiation), local recurrence, and progression from the eye to the CNS or vice versa were analyzed.
Results :
49 patients (46.9% female) with a median age at diagnosis of 64 years (range, 35-91 years) were analyzed. At diagnosis, 30 patients (61.2%) had CNS, 13 (26.5%) had eye, and 6 (12.3%) had eye + CNS involvement. Median time to diagnosis was 2.7 months (eye + CNS), 2.8 months (CNS), and 10.1 months (eye). Eye involvement was bilateral at diagnosis in 78.9%. Common diagnoses prior to confirming VRL were: vitritis (10.2%), uveitis (6.1%), and retinitis (2.0%); 6.1% received prior steroid treatment. Cytopathology confirmed diffuse large B-cell lymphoma in 46 (93.9%), T-cell in 2 (4.1%), and other in 1 (2.0%). MYD88 mutational analysis was performed in 9 out of 19 patients (47.4%) with eye involvement and was positive in 8 out of 9 patients (88.9%). A total of 56 eyes had initial or subsequent VRL, of which 32 eyes (57.1%) received local ocular therapy: 26 eyes (81.3%) with an intravitreal methotrexate-based regimen (MTX, 400 mcg/0.1mL), 2 eyes (6.3%) with radiation (30 Gy), 2 eyes (6.3%) with combination (intravitreal MTX + radiation), and 2 eyes (6.3%) by enucleation. At last follow-up (median 63.1 months), local ocular recurrence occurred in 5 eyes (19.2%) treated with intravitreal therapy and 0 eyes treated with radiation. Nine patients (69.2%) with initial eye only disease progressed to involve the CNS (median time to progression: 28.5 months) and 13 patients (43.3%) with initial CNS disease progressed to involve the eye (median time to progression: 14.1 months). At last follow-up, 33 individuals (67.3%) were alive.
Conclusions :
Time to diagnosis was 3.7 times longer for VRL alone, compared to those with CNS involvement. MYD88 testing was helpful for diagnosis (positive in 88.9%). Both intravitreal MTX and radiation achieved excellent ocular control (80.8% and 100.0%, respectively), however 69.2% ultimately progressed to involve the CNS.
This is a 2021 ARVO Annual Meeting abstract.