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 (20) , Annie Brion (21) , Olivier Casasnovas (22) , Philippe Colin (23) , Oumedaly Reman , Adrian Tempescul (24, 25) , Jean-Pierre Marolleau (26) , Michel Fabbro , Florian Naudet (9) , Khe Hoang-Xuan (27, 28, 29) , Roch Houot (30, 31)

1 CHU Pontchaillou [Rennes]
2 Centre de référence sur les démences rares et maladie de Pick
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 clinique [CHU Pitié-Salpêtrière]
21 Service d'hématologie
22 Hôpital du Bocage
23 Service de radiothérapie [Reims]
24 CHU-Brest-Hemato - CHRU Brest - Service d'Hématologie
25 EA2216 - Immunologie et Pathologie
26 Service clinique des Maladies du Sang
27 ICM - Institut du Cerveau et de la Moëlle Epinière = Brain and Spine Institute
28 Service de Neurologie [CHU Pitié-Salpêtrière]
29 OncoNeuroTek [Paris]
30 MiCa - Microenvironnement et cancer
31 Service d'hématologie clinique

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.

Keywords: Progression; relapse; primary CNS lymphoma; progression

Domains: Life Sciences [q-bio]

Sophie Langner-Lemercier, Caroline Houillier, Carole Soussain, Hervé Ghesquières, Olivier Chinot, et al.. Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network. Neuro-Oncology, 2016, 18 (9), pp.1297-1303. ⟨10.1093/neuonc/now033⟩. ⟨hal-01290413⟩