Characteristics and therapeutic results of retrospective and prospective studies of first-line treatment of PVRL
| Reference . | Type of study/time period . | n . | Median age (range), y . | Type of treatment . | Survival . | CNS events . | |
|---|---|---|---|---|---|---|---|
| 9 | International multicenter retrospective study 1977-2005 | 83 | 63 (24-85) | Local (IVT MTX or ORT): n = 23 Systemic + local*: n = 53 | Median PFS = 29.6 mo Median OS = 58 mo Median follow-up of surviving patients = 32 mo No difference among treatment groups | CNS relapse in 29 patients (35%) No difference among treatment groups | |
| 50 | European multicenter retrospective study 1991-2012 | 78 | 58 (39-86) | Local (IVT MTX ± rituximab or ORT): n = 31 Extensive: n = 21 Local and extensive†: n = 23 | PFS: NA 5-y OS = 68% when no CNS relapse occurred 5-y OS = 35% when CNS relapse occurred | Median CNS-free survival = 47 mo No difference between treatment groups | |
| 51 | Retrospective single-center study 2004-2016 | 10 | 59 (48-71) | Local (IVT MTX): n = 3 Local + IV MTX-based: n = 7 | Median PFS not reached in the local + IV treatment group NA in the local treatment group P = .032 | NA | |
| 52 | Retrospective single-center study 1990-2018 | 32‡ | 67 (36-84) | Local (IVT MTX ± rituximab or ORT): n = 17 IV§: n = 7 Local + IV: n = 8 | PFS = NA FFS: Local = 1.8 y IV = 3.2 y Local + IV = NR P = .002 | Median CNS-free survival = NR CNS relapse = 37% with a median follow-up = 2.8 y | |
| 53 | Retrospective single-center study 2007-NA | 11 | 66 (48-72) | Local + IV RMPV + 36 Gy binocular + HD cytarabine | Median PFS = 3.8 y | 4-y cumulative incidence of CNS progression = 58% | |
| 54 | Retrospective single-center study 2007-2009 | 5 | 65 (43-72) | Local (IVT MTX or ORT) + IV RMPV + rdWBRT | 5 patients alive in CR with a median follow-up = 32 mo | NA | |
| 55 | Retrospective single-center study 2001-2011 | 26 | 67 (36-85) | Local IVT MTX or IVT rituximab: n = 15 Local + systemic IV HD MTX or IT MTX: n = 11 | NA | Median follow-up = 44 mo CNS relapse: Local treatment = 73% Local + IV or IT = 40% (ns) Median time to CNS relapse: Local treatment = 10.2 ± 2.0 mo Local + IV or IT = 42.8 ± 13.8 mo) P = .0005 | |
| 82 | Retrospective single-center study 2005-2018 | 12 | 64 (38-81) | Local (bilateral ORT) + MPV ± rituximab: n = 10 Bilateral ORT: n = 1 IVT MTX: n = 1 | 5-y PFS = 62% 5-y OS = 71% | 5-y cumulative incidence of CNS progression = 37.5% | |
| 56 | Prospective single-center cohort study 2007-2013 | 10 | 68 (46–78) | Local (IVT MTX) followed by IV HD MTX | NA | 2-y CNS-free survival = 58% | |
| 57 | Prospective single-center study 2008-2015 | 11 | 63 (43-72) | Local (IVT MTX) + systemic IV RMPV + rdWBRT‖ + IV HD cytarabine | 4-y PFS = 73% 4-y OS = 89% | Median follow-up = 40 mo 4-y cumulative incidence of CNS progression = 10% | |
| 8 | Retrospective multicenter study 2011-2018 | 59 | 70 (39-88) | Systemic IV HD MTX–based chemotherapy: n = 51 Local (IVT MTX or ORT) + systemic IV HD MTX: n = 8 | Median PFS = 18 mo Median OS = 75 mo | Median follow-up = 61 mo Median CNS-free survival = 73 mo | |
| Reference . | Type of study/time period . | n . | Median age (range), y . | Type of treatment . | Survival . | CNS events . | |
|---|---|---|---|---|---|---|---|
| 9 | International multicenter retrospective study 1977-2005 | 83 | 63 (24-85) | Local (IVT MTX or ORT): n = 23 Systemic + local*: n = 53 | Median PFS = 29.6 mo Median OS = 58 mo Median follow-up of surviving patients = 32 mo No difference among treatment groups | CNS relapse in 29 patients (35%) No difference among treatment groups | |
| 50 | European multicenter retrospective study 1991-2012 | 78 | 58 (39-86) | Local (IVT MTX ± rituximab or ORT): n = 31 Extensive: n = 21 Local and extensive†: n = 23 | PFS: NA 5-y OS = 68% when no CNS relapse occurred 5-y OS = 35% when CNS relapse occurred | Median CNS-free survival = 47 mo No difference between treatment groups | |
| 51 | Retrospective single-center study 2004-2016 | 10 | 59 (48-71) | Local (IVT MTX): n = 3 Local + IV MTX-based: n = 7 | Median PFS not reached in the local + IV treatment group NA in the local treatment group P = .032 | NA | |
| 52 | Retrospective single-center study 1990-2018 | 32‡ | 67 (36-84) | Local (IVT MTX ± rituximab or ORT): n = 17 IV§: n = 7 Local + IV: n = 8 | PFS = NA FFS: Local = 1.8 y IV = 3.2 y Local + IV = NR P = .002 | Median CNS-free survival = NR CNS relapse = 37% with a median follow-up = 2.8 y | |
| 53 | Retrospective single-center study 2007-NA | 11 | 66 (48-72) | Local + IV RMPV + 36 Gy binocular + HD cytarabine | Median PFS = 3.8 y | 4-y cumulative incidence of CNS progression = 58% | |
| 54 | Retrospective single-center study 2007-2009 | 5 | 65 (43-72) | Local (IVT MTX or ORT) + IV RMPV + rdWBRT | 5 patients alive in CR with a median follow-up = 32 mo | NA | |
| 55 | Retrospective single-center study 2001-2011 | 26 | 67 (36-85) | Local IVT MTX or IVT rituximab: n = 15 Local + systemic IV HD MTX or IT MTX: n = 11 | NA | Median follow-up = 44 mo CNS relapse: Local treatment = 73% Local + IV or IT = 40% (ns) Median time to CNS relapse: Local treatment = 10.2 ± 2.0 mo Local + IV or IT = 42.8 ± 13.8 mo) P = .0005 | |
| 82 | Retrospective single-center study 2005-2018 | 12 | 64 (38-81) | Local (bilateral ORT) + MPV ± rituximab: n = 10 Bilateral ORT: n = 1 IVT MTX: n = 1 | 5-y PFS = 62% 5-y OS = 71% | 5-y cumulative incidence of CNS progression = 37.5% | |
| 56 | Prospective single-center cohort study 2007-2013 | 10 | 68 (46–78) | Local (IVT MTX) followed by IV HD MTX | NA | 2-y CNS-free survival = 58% | |
| 57 | Prospective single-center study 2008-2015 | 11 | 63 (43-72) | Local (IVT MTX) + systemic IV RMPV + rdWBRT‖ + IV HD cytarabine | 4-y PFS = 73% 4-y OS = 89% | Median follow-up = 40 mo 4-y cumulative incidence of CNS progression = 10% | |
| 8 | Retrospective multicenter study 2011-2018 | 59 | 70 (39-88) | Systemic IV HD MTX–based chemotherapy: n = 51 Local (IVT MTX or ORT) + systemic IV HD MTX: n = 8 | Median PFS = 18 mo Median OS = 75 mo | Median follow-up = 61 mo Median CNS-free survival = 73 mo | |
IT, intrathecal; MPV, HD MTX, procarbazine, and vincristine; NA, not available; NR, not reached; ns, not significant; rdWBRT, reduced-dose whole-brain radiotherapy (23.4 Gy); RMPV, rituximab, HD MTX, procarbazine, and vincristine.
Systemic + local treatment included various combinations of systemic chemotherapy, ocular chemotherapy, whole-brain radiotherapy, and ocular radiotherapy. Systemic chemotherapy was MTX-based therapy in 37 patients (70%).
Local treatment included local radiotherapy and IVT MTX and/or rituximab. Extensive treatment regimens were heterogeneous and included HD MTX or aracytine (n = 40), whole-brain radiotherapy (n = 6), and peripheral blood stem cell transplantation (n = 4).
In this report, 33 patients had PVRL, 18 with concurrent intraocular and CNS or systemic disease and 18 with secondary VRL. One patient with PVRL did not receive any treatment. Only patients with PVRL who received treatment were considered in this review.
Detailed descriptions of the systemic treatments administered to the subgroup of patients with PVRL are not available.
IV chemotherapy and rdWBRT were administered to patients who achieved CR.