Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network

Sophie Langner-Lemercier; Caroline Houillier; Carole Soussain; Hervé Ghesquières; Olivier Chinot; Luc Taillandier; Pierre Soubeyran; Thierry Lamy; Franck Morschhauser; Alexandra Benouaich-Amiel; Guido Ahle; Marie-Pierre Moles-Moreau; Cécile Moluçon-Chabrot; Pascal Bourquard; Ghandi Damaj; Fabrice Jardin; Delphine Larrieu; Emmanuel Gyan; Rémy Gressin; Arnaud Jaccard; Sylvain Choquet; Annie Brion; Olivier Casasnovas; Philippe Colin; Oumedaly Reman; Adrian Tempescul; Jean-Pierre Marolleau; Michel Fabbro; Florian Naudet; Khe Hoang-Xuan; Roch Houot

doi:10.1093/neuonc/now033

Journal articles

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 1 [CHU Pitié-Salpétrière]

29 OncoNeuroTek [Paris]

30 MiCa - Microenvironnement et cancer

31 Service d'hématologie clinique

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.

Keywords : progression primary CNS lymphoma relapse Progression

Document type :

Journal articles

Domain :

Life Sciences [q-bio]

Complete list of metadatas

https://hal-univ-rennes1.archives-ouvertes.fr/hal-01290413
Contributor : Laurent Jonchère <>
Submitted on : Friday, March 18, 2016 - 10:29:52 AM
Last modification on : Thursday, January 14, 2021 - 11:28:54 AM

Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network

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Primary CNS lymphoma at first relapse/progression: characteristics, management, and outcome of 256 patients from the French LOC network

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