Table 5.

Characteristics and therapeutic results of retrospective and prospective studies of R/R PVRL

ReferenceType of studynMedian age (range), ySystemic treatmentResponseCNS relapseSurvival
92  Prospective phase 1 trial 5* 66 (47-79) Lenalidomide (10-30 mg/d for 21 d of 28-d cycle) ± rituximab IV + intraventricular CR: n = 1
PR: n = 3
SD: n = 1 
NA PFS: 1.75, 6, 9, 21, and 48+ mo 
89  Prospective multicenter phase 2 trial 17 69 (46-86) Rituximab IV (375 mg/m2) lenalidomide (20-25 mg/d for 21 d of 28-d cycle) CR = 35% NA
Median follow- up = 19 mo 
Median PFS = 9.2 mo
Median OS not reached 
90  Prospective multicenter phase II trial 14 67 (47-82) Ibrutinib (560 mg/d) CR = 50% N = 1
Median follow- up = 26 mo 
Median PFS = 23 mo 
88  Retrospective multicenter study 21 75 (35-90) Temozolomide (150 mg/m2/d × 5) CR = 71% N = 5
Median follow- up = 42 mo 
PFS = 12 mo 
101  Retrospective study 10 NA Ifosfamide IV or trofosfamide orally CR: n = 9
PR: n = 1 
N = 2 PFS = 18 mo
OS = 32 mo 
83  Retrospective study NA TBC + ASCT CR: n = 5 N = 0 3 patients alive at 14, 14, and 15 mo 
84  Retrospective study 11* 53 (27-64) TBC + ASCT CR: n = 9
PR: n = 2 
N = 0
Median follow- up = 41.5 mo 
7 patients alive in CR at 18, 28, 41, 45, 49, 62, 69, and 70 mo 
85  Retrospective study 11* 52 (23-67) TBC + ASCT NA for the PVRL subgroup of patients 5-y PFS = 50% (no significant difference between patients with PVRL and PCNSL) 
ReferenceType of studynMedian age (range), ySystemic treatmentResponseCNS relapseSurvival
92  Prospective phase 1 trial 5* 66 (47-79) Lenalidomide (10-30 mg/d for 21 d of 28-d cycle) ± rituximab IV + intraventricular CR: n = 1
PR: n = 3
SD: n = 1 
NA PFS: 1.75, 6, 9, 21, and 48+ mo 
89  Prospective multicenter phase 2 trial 17 69 (46-86) Rituximab IV (375 mg/m2) lenalidomide (20-25 mg/d for 21 d of 28-d cycle) CR = 35% NA
Median follow- up = 19 mo 
Median PFS = 9.2 mo
Median OS not reached 
90  Prospective multicenter phase II trial 14 67 (47-82) Ibrutinib (560 mg/d) CR = 50% N = 1
Median follow- up = 26 mo 
Median PFS = 23 mo 
88  Retrospective multicenter study 21 75 (35-90) Temozolomide (150 mg/m2/d × 5) CR = 71% N = 5
Median follow- up = 42 mo 
PFS = 12 mo 
101  Retrospective study 10 NA Ifosfamide IV or trofosfamide orally CR: n = 9
PR: n = 1 
N = 2 PFS = 18 mo
OS = 32 mo 
83  Retrospective study NA TBC + ASCT CR: n = 5 N = 0 3 patients alive at 14, 14, and 15 mo 
84  Retrospective study 11* 53 (27-64) TBC + ASCT CR: n = 9
PR: n = 2 
N = 0
Median follow- up = 41.5 mo 
7 patients alive in CR at 18, 28, 41, 45, 49, 62, 69, and 70 mo 
85  Retrospective study 11* 52 (23-67) TBC + ASCT NA for the PVRL subgroup of patients 5-y PFS = 50% (no significant difference between patients with PVRL and PCNSL) 

NA, not available; PR, partial response; TBC, thiotepa (750 mg/m2), busulfan (8 mg/kg), and cyclophosphamide (120 mg/kg).

*

N of patients with intraocular lymphoma (isolated intraocular relapse of primary CNS lymphoma, n = 3; intraocular and brain relapse of systemic lymphoma, n = 1; intraocular and brain relapse of primary CNS lymphoma, n = 1).

N of patients included in the studies with isolated intraocular disease involvement (R/R PVRL and intraocular relapse of PCNSL).

These 2 studies did not include the same patients.

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