Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network
Sophie Langner-Lemercier
(1)
,
Caroline Houillier
(2)
,
Carole Soussain
(3)
,
Hervé Ghesquières
(4)
,
Olivier Chinot
(5)
,
Luc Taillandier
(6, 7)
,
Pierre Soubeyran
(8)
,
Thierry Lamy
(9, 1)
,
Franck Morschhauser
(10)
,
Alexandra Benouaich-Amiel
,
Guido Ahle
,
Marie-Pierre Moles-Moreau
,
Cécile Moluçon-Chabrot
(11)
,
Pascal Bourquard
,
Ghandi Damaj
(12)
,
Fabrice Jardin
(13)
,
Delphine Larrieu
,
Emmanuel Gyan
(14, 15)
,
Rémy Gressin
(16, 17)
,
Arnaud Jaccard
(18, 19)
,
Sylvain Choquet
(2)
,
Annie Brion
(20)
,
Olivier Casasnovas
(21)
,
Philippe Colin
(22)
,
Oumedaly Reman
,
Adrian Tempescul
(23, 24)
,
Jean-Pierre Marolleau
(25)
,
Michel Fabbro
,
Florian Naudet
(9)
,
Khe Hoang-Xuan
(26, 2, 27)
,
Roch Houot
(28, 29)
1
Centre Hospitalier Universitaire de Rennes [CHU Rennes] = Rennes University Hospital
2 CHU Pitié-Salpêtrière [AP-HP]
3 CIRB - Centre interdisciplinaire de recherche en biologie
4 FNCLCC - Département Hématologie
5 CRO2 - Centre de Recherches en Oncologie biologique et Oncopharmacologie
6 CRAN - Centre de Recherche en Automatique de Nancy
7 Service de Neuro-Oncologie [CHRU Nancy]
8 Plateforme de génétique moléculaire des cancers d'Aquitaine
9 CIC - Centre d'Investigation Clinique [Rennes]
10 Service d'hématologie
11 Service d'hématologie
12 CHU Amiens-Picardie
13 GPL - Groupe d'étude des proliférations lymphoïdes
14 Service d'hématologie
15 CIC - Tours
16 INSERM U823 - Institut d'oncologie/développement Albert Bonniot de Grenoble
17 Service d'hématologie clinique
18 Service d'Hématologie clinique et thérapie cellulaire [CHU Limoges]
19 CRIBL - Contrôle de la Réponse Immune B et des Lymphoproliférations
20 Service d'hématologie
21 Hôpital du Bocage
22 Service de radiothérapie [Reims]
23 CHU-Brest-Hemato - CHRU Brest - Service d'Hématologie
24 EA2216 - Immunologie et Pathologie
25 Service clinique des Maladies du Sang
26 ICM - Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute
27 OncoNeuroTek [ICM Paris]
28 MiCa - Microenvironnement et cancer
29 Service d'hématologie clinique
2 CHU Pitié-Salpêtrière [AP-HP]
3 CIRB - Centre interdisciplinaire de recherche en biologie
4 FNCLCC - Département Hématologie
5 CRO2 - Centre de Recherches en Oncologie biologique et Oncopharmacologie
6 CRAN - Centre de Recherche en Automatique de Nancy
7 Service de Neuro-Oncologie [CHRU Nancy]
8 Plateforme de génétique moléculaire des cancers d'Aquitaine
9 CIC - Centre d'Investigation Clinique [Rennes]
10 Service d'hématologie
11 Service d'hématologie
12 CHU Amiens-Picardie
13 GPL - Groupe d'étude des proliférations lymphoïdes
14 Service d'hématologie
15 CIC - Tours
16 INSERM U823 - Institut d'oncologie/développement Albert Bonniot de Grenoble
17 Service d'hématologie clinique
18 Service d'Hématologie clinique et thérapie cellulaire [CHU Limoges]
19 CRIBL - Contrôle de la Réponse Immune B et des Lymphoproliférations
20 Service d'hématologie
21 Hôpital du Bocage
22 Service de radiothérapie [Reims]
23 CHU-Brest-Hemato - CHRU Brest - Service d'Hématologie
24 EA2216 - Immunologie et Pathologie
25 Service clinique des Maladies du Sang
26 ICM - Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute
27 OncoNeuroTek [ICM Paris]
28 MiCa - Microenvironnement et cancer
29 Service d'hématologie clinique
Olivier Chinot
- Function : Author
- PersonId : 172185
- IdHAL : olivier-chinot
- IdRef : 034791205
Pierre Soubeyran
- Function : Author
- PersonId : 752156
- IdHAL : plsoubhal2022
- ORCID : 0000-0003-3244-7299
- IdRef : 060718668
Franck Morschhauser
- Function : Author
- PersonId : 762201
- ORCID : 0000-0002-3714-9824
Alexandra Benouaich-Amiel
- Function : Author
Guido Ahle
- Function : Author
Marie-Pierre Moles-Moreau
- Function : Author
Pascal Bourquard
- Function : Author
Delphine Larrieu
- Function : Author
Emmanuel Gyan
- Function : Author
- PersonId : 1131487
- ORCID : 0000-0002-7651-9189
Arnaud Jaccard
- Function : Author
- PersonId : 972533
Oumedaly Reman
- Function : Author
Adrian Tempescul
- Function : Author
- PersonId : 908115
Jean-Pierre Marolleau
- Function : Author
- PersonId : 887235
- IdRef : 06981676X
Michel Fabbro
- Function : Author
Florian Naudet
- Function : Author
- PersonId : 14054
- IdHAL : florian-naudet
- ORCID : 0000-0003-3760-3801
- IdRef : 142630837
Roch Houot
- Function : Author
- PersonId : 767105
- ORCID : 0000-0003-1729-8213
Abstract
Background - Treatment of relapsed/refractory (R/R) primary CNS lymphoma (PCNSL) is poorly defined, because randomized trials and large studies are lacking. The aim of this study was to analyze the characteristics, management, and outcome of R/R PCNSL patients after first-line therapy in a nationwide cohort.
Methods - We analyzed R/R PCNSL patients following first-line treatment who had been prospectively registered in the database of the French network for oculocerebral lymphoma (LOC) between 2011 and 2014.
Results - Among 563 PCNSL patients treated with first-line therapy, we identified 256 with relapsed (n = 93, 16.5%) or refractory (n = 163, 29.0%) disease. Patients who were asymptomatic at relapse/progression (25.5%), mostly diagnosed on routine follow-up neuroimaging, tended to have a better outcome. Patients who received salvage therapy followed by consolidation (mostly intensive chemotherapy plus autologous hematopoietic stem cell transplantation [ICT + AHSCT]) experienced prolonged survival compared with those who did not receive salvage or consolidation therapy. Independent prognostic factors at first relapse/progression were: KPS ≥ 70 vs KPS < 70), sensitivity to first-line therapy (relapsed vs refractory disease), duration of first remission (progression-free survival [PFS] ≥1 y vs <1 y), and management at relapse/progression (palliative care vs salvage therapy). Patients who relapsed early after first-line therapy (ie, PFS < 1 y) had a poor outcome, comparable to that of refractory patients. Conversely, patients experiencing late relapses (PFS ≥ 1 y) and/or undergoing consolidation with ICT + AHSCT experienced prolonged survival.
Conclusions - About a third of PCNSL patients are primary refractory to first line treatment. We identified several independent prognostic factors that can guide the management of R/R PCNSL patients.
Methods - We analyzed R/R PCNSL patients following first-line treatment who had been prospectively registered in the database of the French network for oculocerebral lymphoma (LOC) between 2011 and 2014.
Results - Among 563 PCNSL patients treated with first-line therapy, we identified 256 with relapsed (n = 93, 16.5%) or refractory (n = 163, 29.0%) disease. Patients who were asymptomatic at relapse/progression (25.5%), mostly diagnosed on routine follow-up neuroimaging, tended to have a better outcome. Patients who received salvage therapy followed by consolidation (mostly intensive chemotherapy plus autologous hematopoietic stem cell transplantation [ICT + AHSCT]) experienced prolonged survival compared with those who did not receive salvage or consolidation therapy. Independent prognostic factors at first relapse/progression were: KPS ≥ 70 vs KPS < 70), sensitivity to first-line therapy (relapsed vs refractory disease), duration of first remission (progression-free survival [PFS] ≥1 y vs <1 y), and management at relapse/progression (palliative care vs salvage therapy). Patients who relapsed early after first-line therapy (ie, PFS < 1 y) had a poor outcome, comparable to that of refractory patients. Conversely, patients experiencing late relapses (PFS ≥ 1 y) and/or undergoing consolidation with ICT + AHSCT experienced prolonged survival.
Conclusions - About a third of PCNSL patients are primary refractory to first line treatment. We identified several independent prognostic factors that can guide the management of R/R PCNSL patients.